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Romanelli P, Tuniz F, Fabbro S, Beltramo G, Conti A. Image-guided LINAC radiosurgery in hypothalamic hamartomas. Front Neurol 2022; 13:909829. [PMID: 36119668 PMCID: PMC9475216 DOI: 10.3389/fneur.2022.909829] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2022] [Accepted: 08/01/2022] [Indexed: 11/23/2022] Open
Abstract
Introduction Hypothalamic hamartomas (HH) are developmental malformations that are associated with mild to severe drug-refractory epilepsy. Stereotactic radiosurgery (SRS) is an emerging non-invasive option for the treatment of small and medium-sized HH, providing good seizure outcomes without neurological complications. Here, we report our experience treating HH with frameless LINAC SRS. Materials and methods We retrospectively collected clinical and neuroradiological data of ten subjects with HH-related epilepsy that underwent frameless image-guided SRS. Results All patients underwent single-fraction SRS using a mean prescribed dose of 16.27 Gy (range 16–18 Gy). The median prescription isodose was 79% (range 65–81 Gy). The mean target volume was 0.64 cc (range 0.26–1.16 cc). Eight patients experienced complete or near complete seizure freedom (Engel class I and II). Five patients achieved complete seizure control within 4 to 18 months after the treatment. Four patients achieved Engel class II outcome, with stable results. One patient had a reduction of seizure burden superior to 50% (Engel class III). One patient had no benefit at all (Engel class IV) and refused further treatments. Overall, at the last follow-up, three patients experience class I, five class II, one class III and one class IV outcome. No neurological complications were reported. Conclusions Frameless LINAC SRS provides good seizure and long-term neuropsychosocial outcome, without the risks of neurological complications inherently associated with microsurgical resection.
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Affiliation(s)
- Pantaleo Romanelli
- Cyberknife Center, Italian Diagnostic Center (CDI), Milan, Italy
- *Correspondence: Pantaleo Romanelli
| | - Francesco Tuniz
- Department of Neurosurgery, ASUFC “Santa Maria della Misericordia”, Udine, Italy
| | - Sara Fabbro
- Department of Neurosurgery, ASUFC “Santa Maria della Misericordia”, Udine, Italy
| | | | - Alfredo Conti
- Dipartimento di Scienze Biomediche e Neuromotorie (DIBINEM), IRCCS Istituto delle Scienze Neurologiche di Bologna, Alma Mater Studiorum-Università di Bologna, Bologna, Italy
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Savateev AN, Golanov AV, Saushev DA, Osinov IK, Kostyuchenko VV, Dalechina AV, Melikian AG, Vlasov PA, Mazerkina NA, Makashova ES. [Stereotactic radiosurgery for epilepsy related to hypothalamic hamartoma]. ZHURNAL VOPROSY NEIROKHIRURGII IMENI N. N. BURDENKO 2022; 86:14-24. [PMID: 35942833 DOI: 10.17116/neiro20228604114] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/15/2023]
Abstract
UNLABELLED Hypothalamic hamartoma (HH) is a dysplastic lesion fused with hypothalamus and followed by epilepsy, precocious puberty and behavioral disorders. Up to 50% of patients become free of seizures after surgery, but various complications occur in 1/4 of cases. Radiofrequency thermocoagulation, laser interstitial thermal therapy and stereotactic radiosurgery (SRS) are alternative treatment options. OBJECTIVE To define the indications for SRS in patients with HH and to clarify the irradiation parameters. MATERIAL AND METHODS Twenty-two patients with HH and epilepsy underwent SRS at the Moscow Gamma-knife Center. A retrospective analysis included 19 patients with sufficient follow-up data. Median age of patients was 11.5 years (range 1.3-25.8). The diameter of irradiated HHs ranged between 5.5 and 40.9 mm. In 8 (36%) cases, the volume of hamartoma exceeded 3 cm3. Mean prescribed dose was 18±2.0 Gy, mean prescribed isodose - 48±4.2%. Median follow-up period was 14.8 months (range 3.4 - 96.1). RESULTS Three (15.8%) patients were free of seizures. One patient (5.3%) improved dramatically after treatment with compete resolution of generalized seizures and experienced only rare emotional seizures (Engel IB). Eleven (57.8%) patients reported lower incidence of seizures. Severity and incidence of seizures were the same in 4 patients (21.1%). The best results were achieved in mean target dose over 20-22 Gy, minimal target dose over 7-10 Gy, covering by the prescribed dose of at least 70-80% of hamartoma volume, as well as in patients with the prescribed dose of 12 Gy delivered to almost entire volume of tumor. None patient had any complications after SRS. CONCLUSION SRS is safe regarding neurological, endocrine or visual disturbances. Careful patient selection for SRS makes it an effective option for HH-related epilepsy. The best candidates for SRS are children with seizures aged over 1 year, hamartoma <3 cm3 and area of fusion with hypothalamus <150 mm2.
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Affiliation(s)
| | - A V Golanov
- Burdenko Neurosurgical Center, Moscow, Russia
| | | | - I K Osinov
- Moscow Gamma-knife Center, Moscow, Russia
| | | | | | | | - P A Vlasov
- Burdenko Neurosurgical Center, Moscow, Russia
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3
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Kondajji AM, Evans A, Lum M, Kulinich D, Unterberger A, Ding K, Duong C, Patel K, Yang I. A systematic review of stereotactic radiofrequency ablation for hypothalamic hamartomas. J Neurol Sci 2021; 424:117428. [PMID: 33813160 DOI: 10.1016/j.jns.2021.117428] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2021] [Accepted: 03/25/2021] [Indexed: 11/30/2022]
Abstract
BACKGROUND The seizure activity associated with hypothalamic hamartomas (HHs) is refractory to medical management and surgical intervention is often required. Stereotactic Radiofrequency Ablation (SRFA) is a minimally invasive technique offering targeted lesion ablation with a reduced risk of complications. OBJECTIVE Here, we review the current literature on the use of SRFA for HHs. METHODS This study was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for systematic literature review. A database search of PubMed, Cochrane, Embase, and Web of Science was conducted in July 2020. RESULTS A total of 12 articles were included in this review, showing outcomes for 185 unique patients. Of these patients, 142 (76.8%) experienced some extent of therapeutic benefit in alleviating seizures following SRFA, with 128 (69.2%) patients achieving complete freedom from seizures. Common transient complications included hyponatremia (52 patients, 28.1%), hyperphagia (48 patients, 25.9%), hyperthermia (47 patients, 25.4%), Horner's syndrome (103 patients, 55.7%), and weight gain (75 patients, 40.5%). CONCLUSIONS SRFA is a potential therapy for patients with HHs, especially when resection confers significant risk to surrounding structures. Multiple rounds of SRFA treatment may be needed in approximately a quarter of patients to improve gelastic seizure outcomes but may not affect non-gelastic seizures. There is limited data on the use of SRFA for HHs and further case series and clinical trials are needed to establish the use of SRFA for HHs.
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Affiliation(s)
- Aditya M Kondajji
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Audree Evans
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Meachelle Lum
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Daniel Kulinich
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Ansley Unterberger
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Kevin Ding
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Courtney Duong
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Kunal Patel
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States
| | - Isaac Yang
- Departments of Neurosurgery, Los Angeles (UCLA), Los Angeles, CA, United States; Departments of Radiation Oncology, Los Angeles (UCLA), Los Angeles, CA, United States; Departments of Head and Neck Surgery, Los Angeles (UCLA), Los Angeles, CA, United States; Jonsson Comprehensive Cancer Center, Los Angeles (UCLA), Los Angeles, CA, United States; Los Angeles Biomedical Research Institute, Los Angeles (UCLA), Los Angeles, CA, United States; Harbor-UCLA Medical Center, Los Angeles (UCLA), Los Angeles, CA, United States; David Geffen School of Medicine, Los Angeles (UCLA), Los Angeles, CA, United States.
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4
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Alomari SO, El Houshiemy MN, Bsat S, Moussalem CK, Allouh M, Omeis IA. Hypothalamic Hamartomas: A Comprehensive Review of Literature - Part 3: Updates on Radiotherapy Management. Clin Neurol Neurosurg 2020; 197:106077. [PMID: 32717560 DOI: 10.1016/j.clineuro.2020.106077] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2020] [Revised: 05/25/2020] [Accepted: 07/06/2020] [Indexed: 10/23/2022]
Abstract
Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated incidence ranges from 1 in 50,000 to 1 in 1,000,000. Hypothalamic hamartomas are associated with different clinical presentations including various types of seizures, most characteristically; the gelastic seizures, precocious puberty, cognitive impairment and behavioral changes. In this review, the authors discuss the recent advancements in different modalities of radiotherapy and their application in hypothalamic hamartomas management.
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Affiliation(s)
- Safwan O Alomari
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Mohammed N El Houshiemy
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Shadi Bsat
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Charbel K Moussalem
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Mohammed Allouh
- Anatomy Department, College of Medicine and Health Sciences, United Arab Emirates University, United Arab Emirates
| | - Ibrahim A Omeis
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon.
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5
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Alomari SO, Houshiemy MNE, Bsat S, Moussalem CK, Allouh M, Omeis IA. Hypothalamic hamartomas: A comprehensive review of the literature - Part 1: Neurobiological features, clinical presentations and advancements in diagnostic tools. Clin Neurol Neurosurg 2020; 197:106076. [PMID: 32717559 DOI: 10.1016/j.clineuro.2020.106076] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2020] [Revised: 05/25/2020] [Accepted: 07/06/2020] [Indexed: 10/23/2022]
Abstract
Hypothalamic hamartomas (HH) are rare, non-neoplastic heterotopic tissues which contains normal neurons and glia including oligodendrocytes and fibrillary astrocytes but in an abnormal distribution. They arise from the floor of the third ventricle, tuber cinereum, or mammillary bodies. Estimated incidence ranges from 1 in 50,000-1 in 1,000,000. Hypothalamic hamartomas are associated with different clinical presentations including various types of seizures, most characteristically; the gelastic seizures, precocious puberty, cognitive impairment, and behavioral changes. In this review, the authors discuss advancements in different diagnostic elements of hypothalamic hamartoma; including clinical features, EEG findings, and neuroimaging techniques. Moreover, different classifications described in the literature will be discussed.
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Affiliation(s)
- Safwan O Alomari
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Mohammed N El Houshiemy
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Shadi Bsat
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Charbel K Moussalem
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon
| | - Mohammed Allouh
- Anatomy Department, College of Medicine and Health Sciences, United Arab Emirates University, United Arab Emirates
| | - Ibrahim A Omeis
- Division of Neurosurgery, Department of Surgery, American University of Beirut Medical Center, Lebanon.
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6
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Bourdillon P, Ferrand-Sorbet S, Apra C, Chipaux M, Raffo E, Rosenberg S, Bulteau C, Dorison N, Bekaert O, Dinkelacker V, Le Guérinel C, Fohlen M, Dorfmüller G. Surgical treatment of hypothalamic hamartomas. Neurosurg Rev 2020; 44:753-762. [PMID: 32318922 DOI: 10.1007/s10143-020-01298-z] [Citation(s) in RCA: 33] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2020] [Revised: 03/29/2020] [Accepted: 03/31/2020] [Indexed: 12/15/2022]
Abstract
Hypothalamic hamartomas are aberrant masses, composed of abnormally distributed neurons and glia. Along endocrine and cognitive symptoms, they may cause epileptic seizures, including the specific gelastic and dacrystic seizures. Surgery is the treatment of drug-resistant hamartoma epilepsy, with associated positive results on endocrine, psychiatric, and cognitive symptoms. Recently, alternatives to open microsurgical treatment have been proposed. We review these techniques and compare their efficacy and safety. Open resection or disconnection of the hamartoma, either through pterional, transcallosal, or transventricular approach, leads to good epileptological control, but its high complication rate, up to 30%, limits its indications. The purely cisternal peduncular forms remain the only indication of open, pterional approach, while other strategies have been developed to overcome the neurological, endocrine, behavioral, or cognitive complications. Laser and radiofrequency thermocoagulation-based disconnection through robot-guided stereo-endoscopy has been proposed as an alternative to open microsurgical resection and stereotactic destruction. The goal is to allow safe and complete disconnection of a possibly complex attachment zone, through a single intraparenchymal trajectory which allows multiple laser or radiofrequency probe trajectory inside the ventricle. The efficacy was high, with 78% of favorable outcome, and the overall complication rate was 8%. It was especially effective in patients with isolated gelastic seizures and pure intraventricular hamartomas. Stereotactic radiosurgery has proved as efficacious and safer than open microsurgery, with around 60% of seizure control and a very low complication rate. Multiple stereotactic thermocoagulation showed very interesting results with 71% of seizure freedom and 2% of permanent complications. Stereotactic laser interstitial thermotherapy (LiTT) seems as effective as open microsurgery (from 76 to 81% of seizure freedom) but causes up to 20% of permanent complications. This technique has however been highly improved by targeting only the epileptogenic onset zone in the hamartoma, as shown on preoperative functional MRI, leading to an improvement of epilepsy control by 45% (92% of seizure freedom) with no postoperative morbidity. All these results suggest that the impact of the surgical procedure does not depend on purely technical matters (laser vs radiofrequency thermocoagulation or stereotactic vs robot-guided stereo-endoscopy) but relies on the understanding of the epileptic network, including inside the hamartoma, the aim being to plan an effective disconnection or lesion of the epileptogenic part while sparing the adjacent functional structures.
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Affiliation(s)
- Pierre Bourdillon
- Department of Neurosurgery, Rothschild Foundation Hospital, 29 Rue Manin, 75019, Paris, France. .,Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France. .,Sorbonne Université, Paris, France. .,INSERM U1127, CNRS, UMR7225, Brain and Spine Institute, Paris, France.
| | - S Ferrand-Sorbet
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - C Apra
- Sorbonne Université, Paris, France.,INSERM U1127, CNRS, UMR7225, Brain and Spine Institute, Paris, France.,Department of Neurosurgery, Pitié-Salpêtrière Hospital, Paris, France
| | - M Chipaux
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - E Raffo
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France.,Université de Lorraine, Nancy, France
| | - S Rosenberg
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - C Bulteau
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France.,Université de Paris, Paris, France
| | - N Dorison
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - O Bekaert
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - V Dinkelacker
- Department of Neurology, Rothschild Foundation Hospital, Paris, France
| | - C Le Guérinel
- Department of Neurosurgery, Rothschild Foundation Hospital, 29 Rue Manin, 75019, Paris, France
| | - M Fohlen
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
| | - G Dorfmüller
- Department of Pediatric Neurosurgery, Rothschild Foundation Hospital, Paris, France
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7
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Wright JM, Staudt MD, Alonso A, Miller JP, Sloan AE. A novel use of the NeuroBlate SideFire probe for minimally invasive disconnection of a hypothalamic hamartoma in a child with gelastic seizures. J Neurosurg Pediatr 2018; 21:302-307. [PMID: 29328005 DOI: 10.3171/2017.9.peds1747] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
The authors describe the case of a 22-month-old boy who presented with gelastic seizures and developmental delay. Magnetic resonance imaging and video-electroencephalography monitoring revealed a primarily intraventricular hypothalamic hamartoma and gelastic seizures occurring 20-30 times daily. The patient was treated with various regimens of antiepileptic medications for 16 months, but the seizures remained medically intractable. At 3 years of age, he underwent stereotactic laser ablation with an aim of disconnection of the lesion. The procedure was performed with the NeuroBlate SideFire probe. To the authors' knowledge, this is the first reported use of this technology for this procedure and serves as proof of concept. There were no perioperative complications, and 2 years postprocedure, the patient remains seizure free with marked behavioral and cognitive improvements.
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Affiliation(s)
- James M Wright
- 1Department of Neurological Surgery, University Hospitals, Case Western Reserve University.,3Case Western Reserve University School of Medicine, Cleveland, Ohio; and
| | - Michael D Staudt
- 1Department of Neurological Surgery, University Hospitals, Case Western Reserve University.,4Department of Clinical Neurological Sciences, London Health Science Centre, Western University, London, Ontario, Canada
| | - Andrea Alonso
- 3Case Western Reserve University School of Medicine, Cleveland, Ohio; and
| | - Jonathan P Miller
- 1Department of Neurological Surgery, University Hospitals, Case Western Reserve University.,3Case Western Reserve University School of Medicine, Cleveland, Ohio; and
| | - Andrew E Sloan
- 1Department of Neurological Surgery, University Hospitals, Case Western Reserve University.,3Case Western Reserve University School of Medicine, Cleveland, Ohio; and
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8
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Khawaja AM, Pati S, Ng YT. Management of Epilepsy Due to Hypothalamic Hamartomas. Pediatr Neurol 2017; 75:29-42. [PMID: 28886982 DOI: 10.1016/j.pediatrneurol.2017.07.001] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2015] [Revised: 06/28/2017] [Accepted: 07/01/2017] [Indexed: 11/18/2022]
Abstract
A hypothalamic hamartoma consists of hyperplastic heterotopic tissue growing in a disorganized fashion. These lesions occur in about one per 50,000 to 100,000 people. Hypothalamic hamartomas can cause intrinsic epileptogenesis leading to gelastic seizures. Surrounding cortical structures may also develop secondary epileptogenesis. Persistent seizures caused by hypothalamic hamartomas can be debilitating and result in significant cognitive and behavioral impairment. Early recognition and treatment is important in controlling seizures and in preventing further cognitive deterioration. Some patients experience improved cognition and behavior following early treatment, suggesting that hypothalamic hamartomas represent a reversible epileptic encephalopathy. The outcome of epilepsy associated with these lesions has significantly evolved with the availability of new treatment techniques and an improved understanding of its pathogenesis. Increasing evidence supporting the role of hypothalamic hamartomas as a cause of gelastic seizures and secondary epileptogenesis has led to more frequent use of surgery as the definitive treatment. Several minimally invasive procedures have been devised, including neuroendoscopic approaches and different stereotactic radio and laser ablation techniques. Each of these techniques can lead to unique adverse events. We review the various classification schemes used to characterize hypothalamic hamartomas and the recommended surgical approaches for each subtype. We also review the literature for currently available treatment modalities and compare their efficacy in controlling seizures and their safety profiles.
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Affiliation(s)
- Ayaz M Khawaja
- Department of Neurology, University of Alabama at Birmingham Hospital, Birmingham, Alabama
| | - Sandipan Pati
- Department of Neurology, University of Alabama at Birmingham Hospital, Birmingham, Alabama.
| | - Yu-Tze Ng
- Department of Pediatrics, Baylor College of Medicine, The Children's Hospital of San Antonio, San Antonio, Texas
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9
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Pure endoscopic management of epileptogenic hypothalamic hamartomas. Neurosurg Rev 2017; 40:647-653. [PMID: 28168619 DOI: 10.1007/s10143-017-0822-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2016] [Revised: 01/09/2017] [Accepted: 01/22/2017] [Indexed: 10/20/2022]
Abstract
Hypothalamic hamartomas (HH) are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Their usual clinical presentation is characterized by gelastic/dacrystic seizures which often become pharmaco-resistant and progress to secondary focal/generalized intractable epilepsy causing mostly in children cognitive and behavioral problems (particularly in cases of progressive epileptic encephalopathy) and precocious puberty. Whereas gelastic seizures can be surgically controlled either by resection of the lesion or disconnection (tissue-destructive) procedures, aimed at functionally prevent the spreading of the epileptic burst; generalized seizures tend to respond better to HH excision rather than isolated neocortical resections, which generally fail to control them. Prospective analysis of 14 consecutive patients harboring HH treated in an 8-year period; 12 patients had unilateral and two bilateral HH. All patients were managed by pure endoscopic excision of the HH. The mean operative time was 48 min and mean hospital stay was 2 days; perioperative blood loss was negligible in all cases. Two patients showed a transient diabetes insipidus (DI); no transient or permanent postoperative neurological deficit or memory impairment was recorded. Complete HH excision was achieved in 10/14 patients. At a mean follow-up of 48 months, no wound infection, meningitis, postoperative hydrocephalus, and/or mortality were recorded in this series of patients. Eight patients became seizure free (Engel class I), 2 other experienced worthwhile improvement of disabling seizures (Engel class II); 2 patients were cured from gelastic attacks while still experiencing focal dyscognitive seizures; and 2, having bilateral HH (both undergoing unilateral HH excision), did not experience significant improvement and required later on a temporal lobectomy coupled to amygdalohyppocampectomy. Overall, the followings resulted to be predictive factors for better outcomes in terms of seizure control: (1) cases of unilateral, Delalande class B, HH, (2) shorter history of epilepsy. Endoscopic resection of HH proved, in our series, to be effective in achieving complete control or in reducing the frequency of seizures. Furthermore, this approach has confirmed its minimally invasive nature with a very low morbidity rate: of note, it allowed to better preserve short-term memory and hypothalamic function.
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10
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Butragueño Laiseca L, Oikonomopoulou N, Miranda Herrero MC, Barredo Valderrama E, Vázquez López M, Jiménez de Domingo A, Aguado Del Hoyo A, García-Leal R, Meiriño RM. Neurological complications after gamma-knife radiosurgery for hypothalamic hamartoma. Eur J Paediatr Neurol 2016; 20:745-9. [PMID: 27262616 DOI: 10.1016/j.ejpn.2016.05.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/30/2015] [Revised: 04/10/2016] [Accepted: 05/17/2016] [Indexed: 10/21/2022]
Abstract
BACKGROUND The Gamma-knife technique is a safe and effective option for the treatment of hypothalamic hamartomas that produce epileptic seizures refractory to medical treatment and/or serious behavioral disorders. After this type of radiosurgery, an adequate symptomatic control is normally achieved, with notable decrease or even disappearance of the seizures. Radiological changes, such as a decrease in the size of the tumor or adjacent edema secondary to non-necrotizing radioinduced inflammatory reaction are unusual consequences. Side effects and neurological complications are also rare events. CASE PRESENTATION This report describes an unusual case of complete radiological resolution of a hypothalamic hamartoma as well as neurological complications after Gamma-knife surgery (receiving 13 Gy to the 85% isodose line, 1 cm(3) of tumor volume) in a 8-year-old boy who suffered from severe refractory seizures. After radiosurgery, the patient experienced a notable improvement in his symptoms, achieving seizure cessation within 3 months. However, 4 months after the procedure he presented drowsiness, fever and decreased level of consciousness due to a direct effect on the hypothalamus with local and regional edema secondary to the radiosurgery that was performed. He was successfully treated with corticosteroids (with a total duration of 11 months), and twelve months after the surgery, complete disappearance of both the nodular lesion and the secondary edema was observed. The patient remains seizure-free in the last 16 months, with remarkable changes in his behavior. CONCLUSIONS The present case shows that complete radiological resolution of a hypothalamic hamartoma after Gamma-knife technique is unusual but possible, without long-term neurological consequences. Nevertheless, despite its low incidence, if a patient presents neurological symptoms, primarily during the first year after intervention, possible complications of this type of surgery must be taken into account.
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Affiliation(s)
| | - Niki Oikonomopoulou
- Department of Pediatrics, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
| | | | | | - María Vázquez López
- Department of Neuropediatrics, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
| | - Ana Jiménez de Domingo
- Department of Neuropediatrics, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
| | | | - Roberto García-Leal
- Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain.
| | - Rosa M Meiriño
- Department of Radiation Oncology, Clínica La Luz, Madrid, Spain.
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11
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Shim KW, Park EK, Kim JS, Kim DS. How would we deal with hypothalamic hamartomas? World J Surg Proced 2015; 5:65-74. [DOI: 10.5412/wjsp.v5.i1.65] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2014] [Revised: 12/26/2014] [Accepted: 01/19/2015] [Indexed: 02/06/2023] Open
Abstract
Hypothalamic hamartoma (HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neurosurgical approaches. Treatment options for intractable gelastic seizure in HH patients include direct open surgery with craniotomy, endoscopic surgery, radiosurgery with gamma knife and stereotactic radiofrequency thermocoagulation. Selection of treatment modalities depends on type and size of the HH and the surgeon’s preference. Two surgical techniques, resection and disconnection, had been described with favorable outcomes. Pretreatment evaluation, patient selection, surgical techniques, complications, and possible selection of treatment are discussed.
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12
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Zuleta JLB, Mezo RC, Ortega EP, Barrón BL, Espinosa RC, Marín Muentes DP, Cortázar JS, de Guadalupe Gómez Pérez M, Zuleta JAB, Zuleta JAB. Child with temporal lobe hamartoma: A to Z images and a case report. Ecancermedicalscience 2014; 8:436. [PMID: 24966888 PMCID: PMC4060958 DOI: 10.3332/ecancer.2014.436] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2014] [Indexed: 12/04/2022] Open
Abstract
Gelastic seizure was first described by Trousseau in 1877 and comes from the Greek word gelos (laughs), as laughter is the main feature [1]. Normal laughter is a reactive emotional behaviour and motor action that involves the limbic system, hypothalamus, temporal cortex, and several regions of the brainstem. A female patient, six years old, left-handed, with gelastic seizures, uncontrolled despite being treated with two antiepileptic drugs at high doses, was treated. A simple axial tomography was done, where a hypodense lesion that shapes the inner table of the skull temporal level was observed; later, magnetic resonance imaging was requested, better characterising an intraxial lesion in the right second temporal gyrus cystic appearance.
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Affiliation(s)
- José Liders Burgos Zuleta
- Magnetic Resonance Department, Advanced Medical Image Centre, La Paz, Bolivia ; Magnetic Resonance Department, Hospital Ángeles del Pedregal, México, DF 10700, México
| | - Roger Carillo Mezo
- Magnetic Resonance Department, Hospital Ángeles del Pedregal, México, DF 10700, México
| | | | - Beatriz Luna Barrón
- Department of Medical Genetics, Universidad Mayor de San Andrés, La Paz, Bolivia
| | - Rubén Conde Espinosa
- Magnetic Resonance Department, Hospital Ángeles del Pedregal, México, DF 10700, México
| | - Diana P Marín Muentes
- Magnetic Resonance Department, Hospital Ángeles del Pedregal, México, DF 10700, México
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Wilfong AA, Curry DJ. Hypothalamic hamartomas: optimal approach to clinical evaluation and diagnosis. Epilepsia 2014; 54 Suppl 9:109-14. [PMID: 24328883 DOI: 10.1111/epi.12454] [Citation(s) in RCA: 107] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Hypothalamic hamartomas (HHs) present a difficult medical problem, manifested by gelastic seizures, which are often medically intractable. Although existing techniques offer modest surgical outcomes with the potential for significant morbidity, the relatively novel technique of magnetic resonance imaging (MRI)-guided stereotactic laser ablation (SLA) offers a potentially safer, minimally invasive method with high efficacy for the HH treatment. We report here on 14 patients with medically refractory gelastic epilepsy who underwent stereotactic frame-based placement of an MR-compatible laser catheter (1.6 mm diameter) through a 3.2-mm twist drill hole. A U.S. Food and Drug Administration (FDA)-cleared laser surgery system (Visualase, Inc.) was utilized to ablate the HH, using real-time MRI thermometry. Seizure freedom was obtained in 12 (86%) of 14 cases, with mean follow-up of 9 months. There were no permanent surgical complications, neurologic deficits, or neuroendocrine disturbances. One patient had a minor subarachnoid hemorrhage that was asymptomatic. Most patients were discharged home within 1 day. SLA was demonstrated to be a safe and effective minimally invasive tool in the ablation of epileptogenic HH. Because use of SLA for HH is being adopted by other medical centers, further data will be acquired to help treat this difficult disorder.
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Affiliation(s)
- Angus A Wilfong
- Division of Pediatric Neurology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, U.S.A
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Mittal S, Mittal M, Montes JL, Farmer JP, Andermann F. Hypothalamic hamartomas. Part 2. Surgical considerations and outcome. Neurosurg Focus 2014; 34:E7. [PMID: 23724841 DOI: 10.3171/2013.3.focus1356] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Hypothalamic hamartomas are uncommon developmental heterotopic masses composed of an intermixed array of neurons, glia, and myelinated fibers closely associated with the mammillary bodies. Gelastic seizures, the hallmark feature of hypothalamic hamartomas, commonly present in early childhood. However, patients usually also display a disabling clinical syndrome, which may include various other types of refractory seizures with secondary generalization together with progressive cognitive, behavioral, and psychiatric dysfunction. The hamartoma itself has been unequivocally shown to be intrinsically epileptogenic. Over the past 2 decades there has been considerable effort to develop neurosurgical techniques to treat the epileptic syndrome effectively as well as to improve the neurocognitive and behavioral outcome.
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Affiliation(s)
- Sandeep Mittal
- Department of Neurosurgery, Comprehensive Epilepsy Center, Wayne State University, Detroit Medical Center, Detroit, MI 48201, USA.
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15
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Susheela SP, Revannasiddaiah S, Mallarajapatna GJ, Basavalingaiah A. Robotic-arm stereotactic radiosurgery as a definitive treatment for gelastic epilepsy associated with hypothalamic hamartoma. BMJ Case Rep 2013; 2013:bcr-2013-200538. [PMID: 24027254 DOI: 10.1136/bcr-2013-200538] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Gelastic seizures, characterised by paroxysms of pathological laughter, are most often associated with an underlying hypothalamic hamartoma. This report describes the definitive treatment using stereotactic-radiosurgery for a teenaged child whose gelastic epilepsy was found refractory to various antiepileptic drugs. Since surgery was not consented to, the child was referred to us for stereotactic radiosurgery (SRS), which was delivered with robotic-arm -SRS to a dose of 30 Gy in five fractions in five consecutive days. A decrease in the frequency of seizures was noticeable as early as within a week, and at 12 months after the procedure, there has been a total cessation of seizures.
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Affiliation(s)
- Sridhar Papaiah Susheela
- Department of Radiation Oncology, HealthCare Global-Bangalore Institute of Oncology, Bengaluru, Karnataka, India
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16
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O'Connor L, Curl-Roper T, Reeves N, Kemeny AA, Josan VA. Image-defined resolution following radiosurgery for hypothalamic hamartoma. J Neurosurg Pediatr 2013; 11:464-8. [PMID: 23373624 DOI: 10.3171/2013.1.peds12290] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
The authors present the rare case of complete image-defined resolution of a hypothalamic hamartoma (HH) following Gamma Knife surgery (GKS). A 9-month-old girl presented with an episode of generalized tonic-clonic seizures. Magnetic resonance imaging revealed a left-sided HH, which remained radiologically stable. By 3 years of age the patient had a development delay of 12 months, and experienced 8 gelastic seizures per day while on 2 antiepileptic medications. Thirty-one months after presentation, the patient underwent elective GKS to treat the HH. She has since been seizure free for 22 months, while receiving 3 antiepileptic medications. Twelve months after radiosurgery, MRI revealed complete radiological resolution of the lesion. The authors discuss alternative management options for HH, including microsurgical resection, endoscopic disconnection, stereotactic radiofrequency thermocoagulation, and interstitial radiosurgery. Gamma Knife surgery is a minimally invasive procedure associated with a lower morbidity rate than that of published surgical results. The present case demonstrates the potential for complete image-defined resolution of an HH post-GKS, without long-term neurological sequelae, emphasizing the safety and efficacy of this therapeutic option for the control of epileptic seizures produced by small-volume, surgically inaccessible HHs.
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Affiliation(s)
- Lucy O'Connor
- Manchester Medical School, University of Manchester, UK
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Abstract
Radiosurgery is commonly considered to be effective through a destructive physical mechanism acting on neural tissue. However, the results of modern neurophysiological, radiological, and histological studies are providing a basis on which to question this assumption. There are now multiple pieces of evidence pointing to a nonlesional mechanism of the radiosurgical action. It appears that tissue destruction is absent or minimal and in almost all cases insufficient to explain the clinical effects produced. There is a real possibility that radiosurgery induces changes in the functioning of neural tissue by differential effects on various neuronal populations and remodeling the glial environment, leading to modulation of function while preserving basic processing. Hence, the majority of radiosurgical procedures induce the desired biological effect without histological destruction of tissue. These findings may result in a major paradigm shift in the treatment of functional brain disorders.
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Affiliation(s)
- Jean Régis
- Department of Stereotactic and Functional Neurosurgery, Aix Marseille University, Timone University Hospital, and INSERM U751, 264 rue Saint Pierre, Marseille, 13385, Cedex 05, France.
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18
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Innovative treatments for epilepsy: radiosurgery and local delivery. HANDBOOK OF CLINICAL NEUROLOGY 2012. [PMID: 22939079 DOI: 10.1016/b978-0-444-52899-5.00041-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register]
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Romanelli P, Striano P, Barbarisi M, Coppola G, Anschel DJ. Non-resective surgery and radiosurgery for treatment of drug-resistant epilepsy. Epilepsy Res 2012; 99:193-201. [PMID: 22245137 DOI: 10.1016/j.eplepsyres.2011.12.016] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2011] [Revised: 12/13/2011] [Accepted: 12/26/2011] [Indexed: 10/14/2022]
Abstract
Epilepsy surgery is an effective treatment for properly selected patients with intractable seizures. However, many patients with medically intractable epilepsy are not excellent candidates for surgical resection of the epileptogenic zone. Due to recent advances in computer technology and bioengineering, several novel techniques are receiving increasing interest for their role in the care of people with epilepsy. Neuromodulation is an emerging surgical option to be used when conventional resective surgery is not indicated. We review the indications and expected outcomes of neuromodulatory treatments currently available for the treatment of refractory epilepsy, i.e., vagus nerve stimulation, deep brain stimulation, stereotactic radiosurgery, and multiple subpial transections.
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Affiliation(s)
- Pantaleo Romanelli
- Cyberknife Radiosurgery Center, Centro Diagnostico Italiano, Milan, Italy.
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20
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Abstract
Hypothalamic hamartoma (HH) is usually associated with refractory epilepsy, cognitive impairment, and behavioral disturbance. There is now increasing evidence that HH can be treated effectively with a variety of neurosurgical approaches. Treatment options for intractable gelastic seizure in HH patients include direct open surgery with craniotomy, endoscopic surgery, radiosurgery with gamma knife (GKS) and stereotactic radiofrequency thermocoagulation. Selection of treatment modalities depends on type and size of the HH and the surgeon's preference. Two surgical techniques, resection and disconnection, had been described with favorable outcomes. Pretreatment evaluation, patient selection, surgical techniques, complications, and possible selection of treatment are discussed in this chapter.
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Marras CE, Rizzi M, Villani F, Messina G, Deleo F, Cordella R, Franzini A. Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma. Case report. Neurosurg Focus 2011; 30:E4. [PMID: 21284450 DOI: 10.3171/2010.11.focus10241] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Abstract
Hypothalamic hamartomas (HHs) are developmental malformations associated with a range of neurological problems, including intractable seizures. There is increasing evidence of the epileptogenicity of the hamartoma and of the inhomogeneous distribution of the epileptic abnormalities within the malformation. The management strategy for treatment and results differ according to the insertion plane and the extension of the malformation into the hypothalamus. Cases characterized by extensive involvement of the hypothalamus are particularly challenging. The authors describe the case of a patient with drug-resistant epilepsy and a large hypothalamic hamartoma with an extensive area of attachment. The patient underwent implantation of 2 deep brain electrodes. The intraoperative recording showed a synchronous interictal epileptic discharge in the left temporal lobe and on the left side of the lesion. The patient was treated with chronic high-frequency stimulation. No side effects due to the stimulation were reported. At 18 months' follow-up, a reduction in complex partial seizure frequency was reported, but no significant reduction in overall seizure frequency was noticed (p = 0.14, t-test). The authors report on neurophysiological studies of the relationship between HH and epilepsy, and also discuss the literature on chronic high-frequency stimulation, including its rationale and the results of chronic stimulation of various targets for the treatment of drug-resistant epilepsy due to HH.
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Affiliation(s)
- Carlo Efisio Marras
- Department of Neurosurgery, Fondazione Istituto Neurologico Carlo Besta, Milan, Italy
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22
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Majchrzak K, Bierzyñska-Macyszyn G, Bobek-Billewicz B, Majchrzak H, Ładziński P. Rare primary tumours of the hypothalamus in adults: clinical course and surgical treatment. Neurol Neurochir Pol 2010; 44:546-53. [DOI: 10.1016/s0028-3843(14)60151-1] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
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Addas B, Sherman EMS, Hader WJ. Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy. Neurosurg Focus 2009; 25:E8. [PMID: 18759632 DOI: 10.3171/foc/2008/25/9/e8] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.
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Affiliation(s)
- Bassam Addas
- Calgary Epilepsy Programme, Department of Clinical Neurosciences, University of Calgary, Alberta, Canada
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24
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Frazier JL, Goodwin CR, Ahn ES, Jallo GI. A review on the management of epilepsy associated with hypothalamic hamartomas. Childs Nerv Syst 2009; 25:423-32. [PMID: 19153751 DOI: 10.1007/s00381-008-0798-y] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/09/2008] [Indexed: 11/30/2022]
Abstract
INTRODUCTION Hypothalamic hamartomas are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Patients may be asymptomatic, but the usual presentation is gelastic seizures, precocious puberty, and/or developmental delay. CLINICAL PRESENTATION Without surgical intervention, the gelastic seizures, which are typically present in childhood, may progress to other seizure types, including generalized epilepsy, and are generally refractory to antiepileptic drugs. SUMMARY This review will discuss the clinical and electrophysiologic aspects of these lesions, as well as treatment options, including surgery, endoscopy, and radiosurgery.
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Affiliation(s)
- James L Frazier
- Department of Neurosurgery, Meyer 8-161, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287, USA.
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Téllez-Zenteno JF, Serrano-Almeida C, Moien-Afshari F. Gelastic seizures associated with hypothalamic hamartomas. An update in the clinical presentation, diagnosis and treatment. Neuropsychiatr Dis Treat 2008; 4:1021-31. [PMID: 19337448 PMCID: PMC2646637 DOI: 10.2147/ndt.s2173] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022] Open
Abstract
Gelastic seizures are epileptic events characterized by bouts of laughter. Laughter-like vocalization is usually combined with facial contraction in the form of a smile. Autonomic features such as flushing, tachycardia, and altered respiration are widely recognized. Conscious state may not be impaired, although this is often difficult to asses particularly in young children. Gelastic seizures have been associated classically to hypothalamic hamartomas, although different extrahypothalamic localizations have been described. Hypothalamic hamartomas are rare congenital lesions presenting with the classic triad of gelastic epilepsy, precocious puberty and developmental delay. The clinical course of patients with gelastic seizures associated with hypothalamic hamartomas is progressive, commencing with gelastic seizures in infancy, deteriorating into more complex seizure disorder resulting in intractable epilepsy. Electrophysiological, radiological, and pathophysiological studies have confirmed the intrinsic epileptogenicity of the hypothalamic hamartoma. Currently the most effective surgical approach is the trancallosal anterior interforniceal approach, however newer approaches including the endoscopic and other treatment such as radiosurgery and gamma knife have been used with success. This review focuses on the syndrome of gelastic seizures associated with hypothalamic hamartomas, but it also reviews other concepts such as status gelasticus and some aspects of gelastic seizures in other locations.
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Affiliation(s)
- José F Téllez-Zenteno
- Division of Neurology, University of Saskatchewan, Department of Medicine, Royal University Hospital 103, Hospital Drive. BOX 26, Room 1622, Saskatoon SK S7N OW8, Canada.
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Shim KW, Chang JH, Park YG, Kim HD, Choi JU, Kim DS. Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Neurosurgery 2008; 62:847-56; discussion 856. [PMID: 18496191 DOI: 10.1227/01.neu.0000318170.82719.7c] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
OBJECTIVE Hypothalamic hamartomas (HHs) are often associated with early-onset gelastic seizures, thus configuring a well recognized and usually severe case of childhood epilepsy syndrome. We present a treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. METHODS This study presents 14 patients with medically refractory seizure associated with HHs treated between 1995 and 2005. The HHs were diagnosed on the basis of magnetic resonance imaging, except in the case of one patient in whom hamartoma was confirmed histologically. There were seven boys and seven girls in this study. The most frequent clinical presentations were seizures. To identify the epileptic focus, we performed comprehensive epilepsy investigations, including electroencephalographic recording using a depth electrode into the hamartoma. RESULTS To control the seizure, we performed surgical resection in one patient, gamma knife radiosurgery in four patients, and endoscopic disconnection in 11 patients. Seizure outcome was scored according to Engel's classification throughout a mean follow-up period of 27.4 months (range, 3-54 mo). Of the 11 patients who underwent endoscopic disconnection, six were seizure-free immediately after surgery. Two patients were already diagnosed as having an HH and underwent gamma knife radiosurgery, but seizure control was not achieved. Their gelastic seizure disappeared after endoscopic disconnection. CONCLUSION We confirmed that HHs are intrinsically epileptogenic. Therefore, we suggest that HH-related seizures may be controlled by blocking the seizure propagation from epileptogenic HHs through simple disconnection, regardless of the treatment modality, and the endoscopic disconnection of HHs is safer and more effective than other modalities.
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Affiliation(s)
- Kyu-Won Shim
- Department of Neurosurgery, Pediatric Epilepsy Clinic, Severance Children's Hospital, Brain Korea 21 Project for Medical Science, Yonsei University College of Medicine, Seoul, Korea
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Abstract
Radiosurgery plays an important role in the treatment of refractory seizures induced by hypothalamic hamartomas (HHs). These lesions, deeply located and surrounded by a delicate vascular and neuronal network, are often associated with catastrophic epilepsy leading to progressive cognitive and behavioral deterioration. Surgical approaches include microsurgical resection, endoscopic resection or disconnection, radiofrequency lesioning, and interstitial brachytherapy. Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date.
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Friehs GM, Park MC, Goldman MA, Zerris VA, Norén G, Sampath P. Stereotactic radiosurgery for functional disorders. Neurosurg Focus 2007; 23:E3. [PMID: 18081480 DOI: 10.3171/foc-07/12/e3] [Citation(s) in RCA: 40] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
✓ Stereotactic radiosurgery (SRS) with the Gamma Knife and linear accelerator has revolutionized neurosurgery over the past 20 years. The most common indications for radiosurgery today are tumors and arteriovenous malformations of the brain. Functional indications such as treatment of movement disorders or intractable pain only contribute a small percentage of treated patients. Although SRS is the only noninvasive form of treatment for functional disorders, it also has some limitations: neurophysiological confirmation of the target structure is not possible, and one therefore must rely exclusively on anatomical targeting. Furthermore, lesion sizes may vary, and shielding adjacent radiosensitive neural structures may be difficult or impossible.
The most common indication for functional SRS is the treatment of trigeminal neuralgia. Radiosurgical treatment for epilepsy and certain psychiatric illnesses is performed in several centers as part of strict research protocols, and radiosurgical pallidotomy or medial thalamotomy is no longer recommended due to the high risk of complications. Radiosurgical ventrolateral thalamotomy for the treatment of tremor in patients with Parkinson disease or multiple sclerosis, as well as in the treatment of essential tremor, may be indicated for a select group of patients with advanced age, significant medical conditions that preclude treatment with open surgery, or patients who must receive anticoagulation therapy. A promising new application of SRS is high-dose radiosurgery delivered to the pituitary stalk. This treatment has already been successfully performed in several centers around the world to treat severe pain in patients with end-stage cancer.
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Affiliation(s)
- Gerhard M. Friehs
- 1Department of Clinical Neurosciences Program in Neurosurgery and New England Gamma Knife Center, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island; and
- 2Department of Neurosurgery, Boston University Medical School, Boston, Massachusetts
| | - Michael C. Park
- 1Department of Clinical Neurosciences Program in Neurosurgery and New England Gamma Knife Center, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island; and
| | - Marc A. Goldman
- 1Department of Clinical Neurosciences Program in Neurosurgery and New England Gamma Knife Center, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island; and
| | - Vasilios A. Zerris
- 1Department of Clinical Neurosciences Program in Neurosurgery and New England Gamma Knife Center, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island; and
| | - Georg Norén
- 1Department of Clinical Neurosciences Program in Neurosurgery and New England Gamma Knife Center, Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island; and
| | - Prakash Sampath
- 2Department of Neurosurgery, Boston University Medical School, Boston, Massachusetts
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Homma J, Kameyama S, Masuda H, Ueno T, Fujimoto A, Oishi M, Fukuda M. Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures. Epilepsy Res 2007; 76:15-21. [PMID: 17643965 DOI: 10.1016/j.eplepsyres.2007.06.007] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2006] [Revised: 05/19/2007] [Accepted: 06/03/2007] [Indexed: 02/06/2023]
Abstract
Management of hypothalamic hamartoma with intractable gelastic epilepsy remains controversial. We have used stereotactic thermocoagulation for treatment of hypothalamic hamartoma with intractable gelastic epilepsy since 1997. Herein, we review our experience in five cases to clarify the usefulness of this treatment. A total of five patients with hypothalamic hamartoma were treated by stereotactic thermocoagulation at our hospital during the period October 1997 through February 2004. In all patients, the hamartoma was less than 10mm in diameter and was located on the floor of the third ventricle with sessile attachment to the wall. To identify ictal onset, chronic intracranial electroencephalography was performed in three patients with the use of a depth electrode implanted in the hamartoma. Attempts were made to induce gelastic seizure by electrical stimulation of the hamartoma in three patients. After magnetic resonance imaging-guided targeting, radiofrequency thermocoagulation of the boundary between the hamartoma and normal hypothalamus was performed to achieve disconnection effects. Marked reductions in seizure frequency were obtained in all cases, with three patients becoming seizure-free after the procedure. No intraoperative complications occurred except in one patient who experienced acute and transient panidrosis with hot flushes during coagulation. Our results suggest that stereotactic thermocoagulation of hypothalamic hamartoma is an acceptable treatment option for patients with intractable gelastic seizures.
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Affiliation(s)
- Junpei Homma
- Department of Neurosurgery, Epilepsy Center, Nishi-Niigata Chuo National Hospital, 1-14-1 Masago, Niigata 950-2085, Japan
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30
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Régis J, Scavarda D, Tamura M, Villeneuve N, Bartolomei F, Brue T, Morange I, Dafonseca D, Chauvel P. Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol 2007; 14:73-9. [PMID: 17544950 DOI: 10.1016/j.spen.2007.03.005] [Citation(s) in RCA: 61] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
Numerous neurosurgical approaches are available for children presenting with hypothalamic hamartomas (HHs) associated with severe epilepsy. A concern regarding the impairment of short-term memory after resective surgery is promoting the exploration of less invasive alternatives like radiosurgery. Gamma knife radiosurgery (GKS) can lead to a real reversal of the epileptic encephalopathy. Three years after radiosurgery, 60% of the children have an excellent result with complete seizure cessation in 40% and rare nondisabling seizures in 20%, often in association with dramatic behavioral and cognitive improvement. No permanent neurologic complications have thus far been reported. Rare transient cases of poikilothermia have been observed. GKS is clearly the safer approach for these difficult patients. Young patients with severe epilepsy and neurocognitive comorbidity must be treated by using a curative approach as early as possible. Topological type (according to our original classification) is the major feature for selection of the best treatment strategy. Type I HH deeply embedded in the hypothalamus is treated safely and efficiently by GKS. Type II HH can be resected by either endoscopic or transcallosal approaches or treated by GKS depending on the parent's choice and severity of epilepsy. In small type III HH, GKS is the safer procedure because of the very close relationship to the fornix and mammillary bodies. Types V (rarely epileptic) and IV are frequently operable by disconnection. Very large type VI (or mixed type) with a large component above the floor of the third ventricle must be disconnected, and then the upper remnant is best treated by GKS using a staged technique. Overall, when the lesion is sufficiently small, GKS offers a rate of seizure control comparable to microsurgery but with much lower risk. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a reliable evaluation of the role of GKS.
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Affiliation(s)
- Jean Régis
- Department of Functional Neurosurgery, INSERM 751, Timone Hospital, Marseilles, France.
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31
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Procaccini E, Dorfmüller G, Fohlen M, Bulteau C, Delalande O. Surgical management of hypothalamic hamartomas with epilepsy: the stereoendoscopic approach. Neurosurgery 2007; 59:ONS336-44; discussion ONS344-6. [PMID: 17041502 DOI: 10.1227/01.neu.0000233900.06146.72] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022] Open
Abstract
OBJECTIVE Hypothalamic hamartomas (HHs) require surgical treatment in patients presenting with refractory epilepsy. METHODS The authors report on a single-center series of 33 patients (24 males, 9 females) who underwent surgery between January 1997 and April 2004. They experienced several types of seizure (gelastic, tonic, partial, atonic, generalized tonic-clonic, dacrystic, infantile spasm, mental retardation, and behavioral and endocrinological abnormalities). Forty-nine interventions were carried out. Every patient, with the exception of the first, underwent hamartoma disconnection (pterional approach, six patients; endoscopy, 15 patients; both, 11 patients). The endoscopic approach was carried out with a frameless stereotactic system to enhance feasibility and efficacy of the disconnecting procedure. RESULTS Surgery-related neurological complications occurred in two patients, both after a pterional microsurgical approach. Furthermore, two patients experienced panhypopituitarism and one patient experienced transitory central insipid diabetes. All patients but one showed recovery or considerable improvement of their epilepsy (Engel Class 1, 48.5%; Engel Class 2, 3%; Engel Class 3, 45.5%; mean follow-up duration, 1 yr 7 mo). CONCLUSION According to the proposed classification of sessile HH into four types, the best candidates for endoscopic disconnection are Type 2 and Type 3 HHs. In the present series, 90% of patients affected by Type 2 HH became seizure free and the remaining 10% improved; of those with Type 3 HH at presentation, 35.3% recovered and 60% improved. Neuropsychological and endocrinological test results showed improvement in many patients. Data from our series demonstrate that frameless stereotactic endoscopic disconnection should be considered as the treatment of choice in the presence of favorable anatomic conditions.
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Affiliation(s)
- Emidio Procaccini
- Division of Pediatric Neurosurgery, Fondation Adolphe de Rothschild, Paris, France
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Kato K, Yamane F, Hayashi M, Kasuya H, Okada Y, Kubo O, Oguni H, Oosawa M, Hori T. Hypothalamic hamartoma associated with anterior paraclinoid aneurysm of the internal carotid artery. Neurol Med Chir (Tokyo) 2006; 46:491-4. [PMID: 17062988 DOI: 10.2176/nmc.46.491] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
A 15-year-old boy presented with a history of medically refractory gelastic seizures and cognitive impairments. Neuroimaging demonstrated a sessile type hypothalamic hamartoma, which was treated by gamma knife surgery. However, the gelastic seizures only partially decreased and the frequency of seizures remained unchanged. One year later, angiography before surgery detected anterior paraclinoid aneurysm of the left internal carotid artery. Blood pressure and endocrinological examinations showed no abnormality. Direct surgery was performed to treat the aneurysm and hamartoma. No sclerotic changes were noted in the arterial wall. The aneurysm was treated with clipping, and the hamartoma was partially removed. Postoperative course was uneventful and the gelastic seizures disappeared. No evidence for a causal relationship between the hamartoma and aneurysm was found.
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Affiliation(s)
- Koichi Kato
- Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan.
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Régis J, Scavarda D, Tamura M, Nagayi M, Villeneuve N, Bartolomei F, Brue T, Dafonseca D, Chauvel P. Epilepsy related to hypothalamic hamartomas: surgical management with special reference to gamma knife surgery. Childs Nerv Syst 2006; 22:881-95. [PMID: 16807727 DOI: 10.1007/s00381-006-0139-y] [Citation(s) in RCA: 98] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2006] [Indexed: 12/12/2022]
Abstract
OBJECTIVE A large spectrum of surgical techniques can be proposed to young patients presenting with hypothalamic hamartomas (HH) associated with severe epilepsy. The aim of this report is to point on some clinical and anatomical parameters supposed to influence the choice of the surgical approach and to emphasize the specific role of radiosurgery. MATERIALS AND METHODS We reviewed both our experience and the recent literature based on a Pubmed search. Lateral pterional, midline frontal through the lamina terminalis, transcallosal interforniceal approaches, endoscopic treatment through the foramen of Monro, disconnecting surgery, radiofrequency ablation, brachytherapy and gamma knife surgery (GKS) were all considered. Mortality, morbidity, and efficacy of each of these techniques were compared. Specific limits, difficulties, and constraints were taken into account. Our experience of radiosurgery is based on a prospective trial which enrolled 60 patients with HH and associated severe epilepsy between October 1999 and December 2005. RESULTS Several surgical techniques can lead to a real reversal of the epileptic encephalopathy. The main factors for the decision-making process are the age, the size of the lesion and its anatomical type (according to our original classification), the severity of the epilepsy, and the severity of the cognitive/psychiatric comorbidity. In our prospective trial (GKS), 27 patients have a follow-up superior to 3 years. Among those, 59.2% have an excellent result with a dramatic behavioral and cognitive improvement and are completely seizure-free (37%) or have only rare non-disabling seizures (22.2%). No permanent neurological complication has been observed so far; three patients have presented a transient poïkilothermia. GKS is clearly the safer approach for these difficult patients. Young patients with severe epilepsy and comorbidity must be operated on using a curative approach as early as possible. Very large type VI or mixed type with a large component above the floor of the third ventricle must be disconnected and then the upper remnant can be ideally treated by GKS (staged surgery). Type V (rarely epileptic) and IV are frequently operable by disconnection. Type I HH deeply embedded in the hypothalamus are operated on by GKS efficiently and safely. Type II HH can be operated on either endoscopically or transcallosally or by GKS depending on the parents' choice and severity of epilepsy. In small type III HH, GKS is a safer procedure, due to the very close relationship to the fornix and mammillary bodies. In very large type III HH, transcallosal interforniceal approach is proposed but with significant risks especially concerning short-term memory. When the lesion is sufficiently small, GKS is globally offering the patient a rate of seizure cessation comparable to microsurgery with, however, a much lower risk (no neurological deficit reported till now). CONCLUSION Our first results indicate that GKS is as effective as microsurgical resection and very much safer. GKS also allows avoiding the vascular risk related to radiofrequency lesioning or stimulation. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a reliable evaluation of the role of GKS. The early effect on subclinical discharges turns out to play a major role in the dramatic improvement of sleep quality, behavior, and developmental learning acceleration at school.
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Affiliation(s)
- Jean Régis
- Functional Neurosurgery Department, INSERM 751, Timone Hospital, Marseille, France.
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Mathieu D, Kondziolka D, Niranjan A, Flickinger J, Lunsford LD. Gamma knife radiosurgery for refractory epilepsy caused by hypothalamic hamartomas. Stereotact Funct Neurosurg 2006; 84:82-7. [PMID: 16790990 DOI: 10.1159/000094036] [Citation(s) in RCA: 56] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
BACKGROUND Hypothalamic hamartomas are associated with precocious puberty and chronic epilepsy characterized by gelastic seizures. The seizure disorder is usually refractory to most antiepileptic drugs. Gamma knife surgery has emerged as an alternative to microsurgical removal or radiofrequency ablation to improve seizure control. We present our experience with radiosurgery in 4 patients afflicted by this disorder. METHODS Using gamma knife radiosurgery, 4 patients with intractable gelastic seizures and complex epilepsy were managed. Patient age varied from 5 to 29 years. The duration of symptoms was 4-28 years. A conformal radiosurgery plan was designed with a mean of 4.25 isocenters to cover the hamartoma at the 50% isodose line. A mean margin dose of 17.5 Gy was used. The clinical outcome was evaluated with the Engel scale. RESULTS No complication occurred. After a median follow-up of 22 months, 3 patients had shown some improvement, with 2 attaining Engel class II status. CONCLUSION Gamma knife surgery is a promising alternative to microsurgical removal for patients with refractory epilepsy caused by hypothalamic hamartomas.
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Affiliation(s)
- David Mathieu
- Department of Neurological Surgery, University of Pittsburgh, and Center for Image-Guided Neurosurgery, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
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Striano S, Striano P, Sarappa C, Boccella P. The clinical spectrum and natural history of gelastic epilepsy-hypothalamic hamartoma syndrome. Seizure 2005; 14:232-9. [PMID: 15911357 DOI: 10.1016/j.seizure.2005.01.004] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2004] [Indexed: 10/25/2022] Open
Abstract
PURPOSE To delineate the clinical spectrum and patterns of evolution of epilepsy with gelastic seizures related to hypothalamic hamartoma (HH). PATIENTS AND METHODS We evaluated patients with HH, observed between 1986 and 2002 for whom at least one ictal video-EEG or EEG recording of gelastic seizures was available. RESULTS Six subjects (four male, two female) with sessile HH between 0.8 and 1.7 cm in diameter were identified. The onset of gelastic seizures was between 2 months and 20 years. It evolved to secondary generalized epilepsy in one case, and to drug-resistant partial epilepsy in the other five from 2 to 13 years after onset. No patient showed precocious puberty. Severe cognitive impairment developed in the patient with secondary generalized epilepsy, and a mild cognitive defect in two others. Patients with an HH below 1cm did not show neuropsychological or behavioural disturbances. Drug resistance occurred in all cases. Surgical removal of HH markedly improved the clinical evolution in two patients. CONCLUSIONS Gelastic epilepsy-HH syndrome can differ in severity and evolution. A catastrophic evolution and drug resistance can be reversed by surgical or by gamma-knife ablation of HH.
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Affiliation(s)
- Salvatore Striano
- Epilepsy Center, Department of Neurological Sciences, Federico II University, Via Pansini 5, 80131 Naples, Italy.
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Kerrigan JF, Ng YT, Chung S, Rekate HL. The hypothalamic hamartoma: a model of subcortical epileptogenesis and encephalopathy. Semin Pediatr Neurol 2005; 12:119-31. [PMID: 16114178 DOI: 10.1016/j.spen.2005.04.002] [Citation(s) in RCA: 123] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
Although uncommon, the hypothalamic hamartoma (HH) is often associated with a devastating clinical syndrome, which may include refractory epilepsy, progressive cognitive decline, and deterioration in behavioral and psychiatric functioning. Contrary to conventional thinking which attributed seizure origin to cortical structures, the hamartoma itself has now been firmly established as the site of intrinsic epileptogenesis for the gelastic seizures (i.e., characterized by unusual mirth) peculiar to this disorder. It also appears that the HH contributes to a process of secondary epileptogenesis, with eventual cortical seizure onset of multiple types in some patients. Anticonvulsant medications are known to be poorly effective in this disorder. Treatment, including some innovative approaches to surgical resection, is now targeted directly at the HH itself, with impressive results. Younger patients, in particular, may avoid the deteriorating course described earlier. Access to tissue from larger numbers of patients at single or collaborating centers specializing in HH surgery will allow for research into the fundamental mechanisms producing this little understood disorder. Refractory epilepsy associated with HH is the premier human model for subcortical epilepsy and an excellent model for secondary epileptogenesis and epileptic encephalopathy.
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Affiliation(s)
- John F Kerrigan
- Division of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ 85013, USA.
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Régis J, Hayashi M, Eupierre LP, Villeneuve N, Bartolomei F, Brue T, Chauvel P. Gamma knife surgery for epilepsy related to hypothalamic hamartomas. ACTA NEUROCHIRURGICA. SUPPLEMENT 2005; 91:33-50. [PMID: 15707024 DOI: 10.1007/978-3-7091-0583-2_4] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
Abstract
OBJECTIVE Drug resistant epilepsy associated with hypothalamic hamartoma (HH) can be cured by microsurgical resection of the lesion. Morbidity and mortality risks of microsurgery in this area are significant. Gamma Knife Surgery's (GKS) reduced invasivity seems to be well adapted. In view of the severity of the disease and risks of surgical resection it is crucial to evaluate GKS for this indication. A first retrospective study has shown a very good safety and efficacy level but for a more reliable evaluation a prospective study would be required. METHODS Between Oct 1999 and July 2002, 30 patients with HH and associated severe epilepsy were included. Seizure semiology (video EEG) and frequency, behavioural disturbances, neuropsychological performance, endocrinological status, sleep electroclinical abnormalities, MR imaging, and visual function were systematically evaluated before and after GKS (6, 12, 18, 24, 36 months). Twenty patients had experienced precocious puberty at a median age of 3,7 (0-9). Range of maximum diameter was from 7,5 to 23 mm with only 3 larger than 18 mm. The median marginal dose was 17 gy (14-20). RESULTS Sufficient follow up for final evaluation is not yet available. Only 6 patients have a follow-up of more than 12 months and 19 more than 6 months. However a lot of very dramatic changes did occur during that period in this group. Among the 19 patients with more than 6 months of follow-up, a lot had already experienced an increase of gelastic seizures around 3 months (3), an improvement in their seizure rate (18), behaviour (9), sleep (3), and EEG background activity (3), a cessation of partial complex seizures (7). No complications have occurred till now except one patient experiencing at 5 months a hyperthermia without infection and concomitant increase of gelastic seizures both ceasing suddenly and spontaneously after 15 days. CONCLUSION Our first results indicate that GKS is as effective as microsurgical resection and very much safer. GKS also allows to avoid the vascular risk related to radiofrequency lesioning or stimulation. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a serious evaluation of the role of GKS. Results are faster and more complete in patients with smaller lesions inside the 3rd ventricle (grade II). The early effect on subclinical discharges turns out to play a major role in the dramatic improvement of sleep quality, behaviour, developmental acceleration at school.
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Affiliation(s)
- J Régis
- Stereotactic and Functional Neurosurgery Department, Timone Hospital, Marseilles, France.
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Abstract
The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have yielded important data. Categorizing HHs by the degree of hypothalamic involvement has contributed to the accurate prediction of their prognosis and to improved treatment strategies. Rather than undergoing corticectomy, HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, total eradication or disconnection of the lesion leads to cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life. This review of hypothalamic hamartomas presents current classifications, pathophysiologies, and treatment modalities.
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Affiliation(s)
- Kazunori Arita
- Department of Neurosurgery, Graduate School of Biomedical Science, Hiroshima University, Japan.
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Homberg V, Schulze-Bonhage A, Quiske A, Trippel M, Ostertag C. Gelastische Anfälle, Pubertas praecox und Verhaltensstörung bei hypothalamischem Hamartom. Monatsschr Kinderheilkd 2004. [DOI: 10.1007/s00112-003-0725-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Schulze-Bonhage A, Quiske A, Homberg V, Trippel M, Wagner K, Frings L, Bast T, Huppertz HJ, Warnke C, Ostertag C. Effect of interstitial stereotactic radiosurgery on behavior and subjective handicap of epilepsy in patients with gelastic epilepsy. Epilepsy Behav 2004; 5:94-101. [PMID: 14751213 DOI: 10.1016/j.yebeh.2003.11.021] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Patients with symptomatic epilepsy due to hypothalamic hamartomas often are compromised not only by pharmacoresistant epileptic seizures but also by behavioral disturbances and cognitive dysfunction. We report the effect of successful treatment with stereotactic interstitial radiosurgery by intrahypothalamic implantation of 125I seeds on behavior and subjective handicap. In all patients rendered seizure-free or suffering only from auras, improvement of behavior was reported by parents and colleagues or schoolteachers. Parents' ratings according to the Child Behavior Checklist showed improvements with respect to social problems and attention. Self-ratings of quality of life by adult patients showed improvements in activities, working situation, and self-perception. These improvements were not observed in patients in whom clinically manifest seizures and interictal EEG discharges persisted after radiosurgery.
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Arán E, Pereira J, Castro L, Vaz R. Voluminoso hamartoma hipotalámico en un niño de 5 meses: Epilepsia y cirugía. Neurocirugia (Astur) 2004; 15:294-7. [PMID: 15239017 DOI: 10.1016/s1130-1473(04)70487-x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
The authors report the case of 5-month-old boy with a hypothalamic hamartoma and persistent gelastic seizures in spite of a wide combination of different antiepileptic drugs. It was decided to carry out only partial removal of the tumor for decompression and to decreasing the activity of the epileptogenic focus. Surgical therapy revealed as a valid option in the treatment of the uncontrollable gelastic seizures.
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Affiliation(s)
- E Arán
- Servicio de Neurocirugía. Hospital São João. Oporto. Portugal
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Brandberg G, Raininko R, Eeg-Olofsson O. Hypothalamic hamartoma with gelastic seizures in Swedish children and adolescents. Eur J Paediatr Neurol 2004; 8:35-44. [PMID: 15023373 DOI: 10.1016/j.ejpn.2003.10.003] [Citation(s) in RCA: 62] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/22/2003] [Accepted: 10/07/2003] [Indexed: 12/21/2022]
Abstract
BACKGROUND Hypothalamic hamartoma with gelastic seizures (HHGS) is an uncommon, often unrecognized, epileptic syndrome with onset of symptoms during childhood. AIM In order to study the occurrence, clinical symptoms and different investigations of HHGS in Swedish children and adolescents, a nationwide survey was undertaken. Methods. Twelve patients, three females, aged 5 to 19 years were identified and their hospital records reviewed. MRI examinations were reinvestigated. RESULTS Gelastic seizures were noted before the age of six months in seven patients in at least three as early as the neonatal period. During the course of disease one or more other seizure types developed in 11 patients. Behaviour disorder became subsequently obvious in ten patients, and mental retardation was diagnosed in seven. Precocious puberty was diagnosed in five patients. A total of 46 MRI examinations were performed in 11 patients, revealing hypothalamic tumors, eight of which were drooping with a broad base. Interictal and ictal EEG examinations were pathological in 10 patients with nonspecific results. Nonspecific results were also found on SPECT and PET performed in six and two patients, respectively. Available antiepileptic drugs had little or no effect on gelastic seizures, but some effect on other seizure types. Precocious puberty was treated with a GnRH-agonist. Neurosurgical treatment of the hypothalamic hamartoma, performed in three patients, had a rather good outcome concerning gelastic seizures and behaviour. Vagal nerve stimulation in five patients had no effect. CONCLUSIONS Review of the literature and experience from this group's own cases confirms that early diagnosis of HHGS is important. Hypothalamic hamartoma should be considered in any child with laughing attacks. MRI investigation is compulsory, and neurosurgery the most important treatment.
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MESH Headings
- Adolescent
- Child
- Child, Preschool
- Comorbidity
- Cross-Sectional Studies
- Diagnosis, Differential
- Diagnostic Imaging
- Epilepsies, Partial/diagnosis
- Epilepsies, Partial/epidemiology
- Epilepsies, Partial/etiology
- Epilepsies, Partial/therapy
- Female
- Hamartoma/complications
- Hamartoma/diagnosis
- Hamartoma/epidemiology
- Hamartoma/therapy
- Health Surveys
- Hospitals, University
- Humans
- Hypothalamic Diseases/complications
- Hypothalamic Diseases/diagnosis
- Hypothalamic Diseases/epidemiology
- Hypothalamic Diseases/therapy
- Hypothalamus/pathology
- Male
- Puberty, Precocious/diagnosis
- Puberty, Precocious/epidemiology
- Puberty, Precocious/etiology
- Puberty, Precocious/therapy
- Sweden/epidemiology
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Unger F, Schröttner O, Feichtinger M, Bone G, Haselsberger K, Sutter B. Stereotactic radiosurgery for hypothalamic hamartomas. ACTA NEUROCHIRURGICA. SUPPLEMENT 2003; 84:57-63. [PMID: 12379005 DOI: 10.1007/978-3-7091-6117-3_6] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/26/2023]
Abstract
Hypothalamic hamartomas are nonneoplastic lesions often characterized by central precocious puberty and gelastic epilepsy. Due to the delicate location surgery is often unsuccessful and associated with considerable risks. In the presented series, Gamma Knife radiosurgery was applied. Four cases (aged between 5-13 years) who presented with medically intractable gelastic epilepsy and increasing secondary generalization, abnormal behaviour and precocious puberty (3 cases) are reported. Hypothalamic hamartomas sized 11-17 mm had been diagnosed by MR imaging. Radiosurgical treatment was performed in general anaesthesia with margin doses of 12-14 Gy to the 50-90% isodoses covering volumes of 600-2300 mm3. After follow-up periods of 12 to 68 months, a continuing decrease both in seizure frequency and intensity was noted (outcome according to Engel: II a (3 cases) and III a (1 case)). All patients are socially reintegrated. MR imaging did not reveal significant changes concerning the size of the lesions. Gamma Knife radiosurgery can be an effective and safe alternative treatment modality for HH capable of achieving good seizure control and improving behavioural disorders in selected cases.
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Affiliation(s)
- F Unger
- Department of Neurosurgery, Karl-Franzens University, Graz, Austria
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Régis J, Bartolomei F, Hayashi M, Chauvel P. Gamma Knife surgery, a neuromodulation therapy in epilepsy surgery! ACTA NEUROCHIRURGICA. SUPPLEMENT 2003; 84:37-47. [PMID: 12379003 DOI: 10.1007/978-3-7091-6117-3_4] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
INTRODUCTION The more classical approach for Epilepsy surgery is the removal of the epileptogenic zone (ZE). We present a critical review of information in favor of a possible non-destructive effect of radiosurgery in epilepsy surgery. MATERIAL Clinical material of patients with epilepsies related to a lesion in highly functional areas subjected to radiosurgery with relief of the seizures and no functional worsening is available. We applied direct treatment of the EZ with good efficacy in the absence of destructive aspects on the MR and no functional deterioration (e.g. hypothalamic hamartomas). Experimental studies have shown biochemical differential effect of radiosurgery on the striatum, glial cell elimination, stem cell migration toward the target area, sprouting,... Plasticity phenomenon are induced by radiosurgery when using non necrotizing dosemetry. DISCUSSION There is clinical and experimental evidence of Gamma Knife capability to induce modulation in the neural system. Detailed mechanism of this modulation and dosemetric parameters enabling to induce such plasticity with no necrosis are still unknown. Subpial transection turning out actually to be quite disappointing, there is a specific rationale to test radiosurgery capability to treat EZ cortex while preserving the underlying function of this cortex when the functional risk for cortectomy is too high.
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Affiliation(s)
- J Régis
- Stereotactic and Functional Neurosurgery Department, Neurophysiology/Neuropsychology INSERM 9926, Timone Hospital, Marseilles, France
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Carvalho GA, Matthies C, Osorio E, Samii M. Hamartomas of the internal auditory canal: report of two cases. Neurosurgery 2003; 52:944-8; discussion 948-9. [PMID: 12657191 DOI: 10.1227/01.neu.0000053100.29308.f3] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2002] [Accepted: 12/05/2002] [Indexed: 11/19/2022] Open
Abstract
OBJECTIVE AND IMPORTANCE To highlight the clinical, radiological, and surgical findings and therapeutic options for this rare entity, which may mimic a purely intrameatal vestibular schwannoma, and to define the particular aspects of preoperative differential diagnosis and surgical management. CLINICAL PRESENTATION Two patients presented with clinical findings typical of vestibular schwannomas, i.e., tinnitus, hearing loss of 30 dB, and an intrameatal contrast-enhancing lesion on magnetic resonance imaging studies. TECHNIQUE The lesions were exposed via a suboccipital transmeatal approach, and tumor infiltration of the cochlear and/or facial cranial nerves was identified. In view of the unclear intraoperative histology, surgical management was based on criteria of cranial nerve function. In Patient 1, after nerve decompression by subtotal tumor removal, preserved auditory brainstem responses and facial nerve electromyography indicated functional nerve preservation and facilitated the decision for partial resection. In Patient 2, minimal tumor dissection resulted in complete loss of auditory brainstem response without reversibility. Therefore, a radical tumor removal was performed that sacrificed the cochlear but preserved the facial nerve. CONCLUSION Symptoms and signs of internal auditory canal hamartomas are congruent with other typical pathological lesions of the internal auditory canal and cerebellopontine angle. Accurate preoperative diagnosis by radiological means is not possible, but careful evaluation of the different signal intensities on magnetic resonance imaging studies may indicate this rare pathological condition. Intraoperative surgical findings of tumor infiltration of the faciocochlear cranial nerve complex may support simple observation. In view of the nonneoplastic characteristic of these lesions, a more conservative approach is justified. The decision should be based on the functional status of the cranial nerves, for which reliable electrophysiological monitoring is indispensable.
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Affiliation(s)
- Gustavo A Carvalho
- Department of Neurosurgery, State University of Rio de Janeiro, and Clinica Bambina, Rio de Janeiro, Brazil.
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Delalande O, Fohlen M. Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. Neurol Med Chir (Tokyo) 2003; 43:61-8. [PMID: 12627881 DOI: 10.2176/nmc.43.61] [Citation(s) in RCA: 188] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
A series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 53% of patients and dramatic improvement in the others. Surgical observations led us to propose a new anatomical classification according to the anatomical relationship between the hamartoma and the adjacent hypothalamus and third ventricle. Endoscopic disconnection seems to be a very safe way to treat hamartomas in intraventricular locations.
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Affiliation(s)
- Olivier Delalande
- Unité de neurochirurgie pédiatrique, Fondation Ophtalmologique A. de Rothschild, Paris, France.
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Baldwin K, Miller L, Scott JB. Proactive identification of seizure risk improves terminal care. Am J Hosp Palliat Care 2002; 19:251-8. [PMID: 12141789 DOI: 10.1177/104990910201900409] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Seizures are a common neurologic consequence of various etiologies in the end-stage cancer patient. Because dying cancer patients and their families are often coping with new challenges daily, any intervention that can prevent further patient or family discomfort should be implemented. The purpose of this study was to evaluate four pharmacy interventions aimed at improving nursing care to cancer patients at a facility for the terminally ill. The four interventions were development of a seizure risk factor assessment toll, assembly or availability of a diazepam seizure kit, nursing education via in-service training, and development of a manual for nursing stations. Successful implementation of this project indicated that nurses perceived an empowerment in improving quality of life of their patients.
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Abstract
Like normal cerebral function, epileptic seizures involve widespread network interactions between cortical and subcortical structures. Although the cortex is often emphasized as the site of seizure origin, accumulating evidence points to a crucial role for subcortical structures in behavioral manifestations, propagation, and, in some cases, initiation of epileptic seizures. Extensive previous studies have shown the importance of subcortical structures in animal seizure models, but corresponding human studies have been relatively few. We review the existing evidence supporting the importance of the thalamus, basal ganglia, hypothalamus, cerebellum, and brain stem in human epilepsy. We also propose a "network inhibition hypothesis" through which focal cortical seizures disrupt function in subcortical structures (such as the medial diencephalon and pontomesencephalic reticular formation), leading secondarily to widespread inhibition of nonseizing cortical regions, which may in turn be responsible for behavioral manifestations such as loss of consciousness during complex partial seizures.
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Affiliation(s)
- Andrew D. Norden
- Departments of Neurology and Neurobiology, Yale University School of Medicine, 333 Cedar Street, New Haven, 06520-8018, CT, USA
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Nishio S, Morioka T, Mihara F, Fukui M. Neuroimaging and neuropathology in epilepsy: With special reference to focal epileptogenic abnormalities. Neuropathology 2002. [DOI: 10.1046/j.1440-1789.1999.00229.x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Affiliation(s)
- Shunji Nishio
- Department of Neurosurgery, Neurological Institute and,
| | | | - Futoshi Mihara
- Department of Radiology, Faculty of Medicine, Kyushu University, Fukuoka, Japan
| | - Masashi Fukui
- Department of Neurosurgery, Neurological Institute and,
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Dunoyer C, Ragheb J, Resnick T, Alvarez L, Jayakar P, Altman N, Wolf A, Duchowny M. The use of stereotactic radiosurgery to treat intractable childhood partial epilepsy. Epilepsia 2002; 43:292-300. [PMID: 11906515 DOI: 10.1046/j.1528-1157.2002.06501.x] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
PURPOSE Although conventional surgery is presently used to treat seizures of temporolimbic and neocortical origin, deep-seated lesions are often associated with morbidity. Stereotactic radiosurgery is a noninvasive procedure that effectively treats patients with vascular malformations and brain tumors, but its efficacy for epileptogenic foci is limited, especially in children. METHODS Between 1995 and 1999, four candidates who had medically uncontrolled seizures and localized seizure foci were selected for stereotactic radiosurgery, with a mean age of 9.75 years at the time of surgery (range, 4-17 years). Seizure foci were identified on the basis of ictal and interictal video-EEG. Magnetic resonance (MR) images were obtained before and after surgery. Ictal single-photon emission computed tomography (SPECT) was performed by using stabilized hexamethyl-propyleneamine oxime (HMPAO; 300 microcuries/kg) with early injection after electrographic ictal onset. The clinical features of the patients are given. All radiosurgical procedures were performed with the gamma knife unit with the Leksell stereotactic frame, stereotactic MRI imaging, and the Gamma Plan workstation. Seizure outcome was scored according to Engel's classification. RESULTS Two patients had hypothalamic hamartoma (HH), and two had neocortical epilepsy. At mean follow-up of 39.2 months (range, 26-69 months), two patients were seizure free, one with a HH and one with a suggestive developmental tumor in the insular cortex by MRI findings. The other patient with HH had 90% reduction of seizures. One patient with a widespread seizure focus that involved the motor strip was unimproved. The two patients with HH also exhibited markedly improved neurobehavioral status after surgery. There were no significant complications of radiosurgical therapy. CONCLUSIONS Our findings suggest that gamma knife surgery is a potentially valuable treatment modality for children with medically intractable epilepsy due to a well-localized seizure focus that is difficult to excise by conventional techniques or for whom they are deemed unsuitable. More widespread application in childhood epilepsy should be investigated in larger series.
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Affiliation(s)
- Catalina Dunoyer
- Department of Neurology, Miami Children's Hospital, Miami, Florida 33155, USA
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