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Mahjoubi YS, Dahmani I, Zgolli F, Kaabi W, Kastalli S, Aouintin I, El Aidli S. Adalimumab-induced myoclonus: A potential adverse drug reaction? Therapie 2025; 80:356-358. [PMID: 39757086 DOI: 10.1016/j.therap.2024.12.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2024] [Revised: 11/19/2024] [Accepted: 12/12/2024] [Indexed: 01/07/2025]
Affiliation(s)
- Yasmine Salem Mahjoubi
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia.
| | - Israa Dahmani
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
| | - Fatma Zgolli
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
| | - Widd Kaabi
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
| | - Sarrah Kastalli
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
| | - Imen Aouintin
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
| | - Sihem El Aidli
- National Center Chalbi Belkahia of Pharmacovigilance, 1006 Tunis, Tunisia; University of Tunis El Manar, Faculty of Medicine of Tunis, Research Unit UR17ES12, 1006 Tunis, Tunisia
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Canafoglia L, Meletti S, Bisulli F, Alvisi L, Assenza G, d’Orsi G, Dubbioso R, Ferlazzo E, Ferri L, Franceschetti S, Gambardella A, Granvillano A, Licchetta L, Nucera B, Panzica F, Perulli M, Provini F, Rubboli G, Strigaro G, Suppa A, Tartara E, Cantalupo G. A Reappraisal on cortical myoclonus and brief Remarks on myoclonus of different Origins. Clin Neurophysiol Pract 2024; 9:266-278. [PMID: 39559741 PMCID: PMC11570231 DOI: 10.1016/j.cnp.2024.10.001] [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: 07/11/2024] [Revised: 10/08/2024] [Accepted: 10/13/2024] [Indexed: 11/20/2024] Open
Abstract
Myoclonus has multiple clinical manifestations and heterogeneous generators and etiologies, encompassing a spectrum of disorders and even physiological events. This paper, developed from a teaching course conducted by the Neurophysiology Commission of the Italian League against Epilepsy, aims to delineate the main types of myoclonus, identify potential underlying neurological disorders, outline diagnostic procedures, elucidate pathophysiological mechanisms, and discuss appropriate treatments. Neurophysiological techniques play a crucial role in accurately classifying myoclonic phenomena, by means of simple methods such as EEG plus polymyography (EEG + Polymyography), evoked potentials, examination of long-loop reflexes, and often more complex protocols to study intra-cortical inhibition-facilitation. In clinical practice, EEG + Polymyography often represents the first step to identify myoclonus, acquire signals for off-line studies and plan the diagnostic work-up.
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Affiliation(s)
- Laura Canafoglia
- Department of Diagnostic and Technology, full member of the European Reference Network EpiCARE, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
| | - Stefano Meletti
- Department of Biomedical, Metabolic and Neural Sciences University of Modena and Reggio Emilia, Director of Neurophysiology Unit & Epilepsy Centre, AOU Modena
| | - Francesca Bisulli
- Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
- IRCCS Istituto delle Scienze Neurologiche di Bologna, Full member of the ERN EpiCARE, Bologna, Italy
| | - Lara Alvisi
- Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
- IRCCS Istituto delle Scienze Neurologiche di Bologna, Full member of the ERN EpiCARE, Bologna, Italy
| | - Giovanni Assenza
- Unit of Neurology, Neurophysiology, Neurobiology, Department of Medicine, University Campus Bio-Medico of Rome, Via Álvaro del Portillo, 21, 00128, Rome, Italy
| | - Giuseppe d’Orsi
- Neurology Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, S. Giovanni Rotondo, Foggia, Italy
| | - Raffaele Dubbioso
- Neurophysiology Unit, Department of Neurosciences, Reproductive Sciences and Odontostomatology, University of Naples “Federico II”, Napoli, Italy
| | - Edoardo Ferlazzo
- Regional Epilepsy Centre, Great Metropolitan “Bianchi-Melacrino-Morelli” Hospital, Reggio Calabria, Italy
- Department of Medical and Surgical Sciences, Magna Graecia University of Catanzaro, Italy
| | - Lorenzo Ferri
- Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
- IRCCS Istituto delle Scienze Neurologiche di Bologna, Full member of the ERN EpiCARE, Bologna, Italy
| | - Silvana Franceschetti
- Neurophysiopathology, full member of the European Reference Network EpiCARE, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy
| | - Antonio Gambardella
- Department of Medical and Surgical Sciences, Institute of Neurology, University Magna Græcia, Catanzaro, Italy
| | - Alice Granvillano
- Neurophysiopathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy
| | - Laura Licchetta
- IRCCS Istituto delle Scienze Neurologiche di Bologna, Full member of the ERN EpiCARE, Bologna, Italy
| | - Bruna Nucera
- Department of Neurology, Hospital of Merano (SABES-ASDAA), Franz Tappeiner Hospital, Via Rossini, 5-39012, Merano, Italy. 2 Paracelsus Medical University, 5020 Salzburg, Austria
| | - Ferruccio Panzica
- Clinical Engineering Service, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
| | - Marco Perulli
- Neuropsichiatria Infantile, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy
| | - Federica Provini
- Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
- IRCCS Istituto delle Scienze Neurologiche di Bologna, Full member of the ERN EpiCARE, Bologna, Italy
| | - Guido Rubboli
- Danish Epilepsy Center, Dianalund, University of Copenhagen, Denmark
| | - Gionata Strigaro
- Epilepsy Center, Neurology Unit, Department of Translational Medicine, University of Piemonte Orientale, and Azienda Ospedaliero-Universitaria “Maggiore Della Carità”, Novara, Italy
| | - Antonio Suppa
- Department of Human Neurosciences, Sapienza University of Rome, Viale dell’Università, 30, 00185 Rome, Italy
- IRCCS Neuromed Institute, Via Atinense, 18, 86077 Pozzilli (IS), Italy
| | - Elena Tartara
- Epilepsy Center, IRCCS Mondino Foundation, Pavia, Italy
| | - Gaetano Cantalupo
- Department of Engineering for Innovation Medicine, University of Verona, Italy
- Child Neuropsychiatry Unit, Verona University Hospital (AOUI Verona) - full member of the European Reference Network EpiCARE, Italy
- Center for Research on Epilepsy in Pediatric age (CREP), AOUI Verona, Verona, Italy
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Peraio S, Mantovani G, Araceli T, Mongardi L, Noris A, Fino E, Formica F, Piccinini L, Melani F, Lenge M, Scalise R, Battini R, Di Rita A, D'Incerti L, Appleton T, Cavallo MA, Guerrini R, Giordano F. Unilateral deep brain stimulation (DBS) of nucleus ventralis intermedius thalami (Vim) for the treatment of post-traumatic tremor in children: a multicentre experience. Childs Nerv Syst 2024; 40:2457-2464. [PMID: 38573550 DOI: 10.1007/s00381-024-06380-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2024] [Accepted: 03/26/2024] [Indexed: 04/05/2024]
Abstract
PURPOSE Deep brain stimulation (DBS) of nucleus ventralis intermedius thalami (Vim) is a validated technique for the treatment of essential tremor (ET) in adults. Conversely, its use for post traumatic tremor (PTT) and in paediatric patients is still debated. We evaluated the efficacy of Vim-DBS for lesional tremor in three paediatric patients with drug-resistant post-traumatic unilateral tremor. METHODS We retrospectively collected data regarding three patients with unilateral tremor due to severe head injury, with no MRI evidence of basal ganglia lesions. The three patients underwent stereotactic frame-based robot-assisted DBS of Vim contralateral to the tremor side. RESULTS Mean follow-up was 48 months (range: 36-60 months). Tremor was reduced in all patients with a better control of voluntary movements and improvement of functional status (mean FIM scale improvement + 7 points). No surgical complications occurred. CONCLUSION Unilateral contralateral DBS of Vim could be efficacious in post-traumatic tremor, even in paediatric patients and should be offered in PTT drug-resistant patients.
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Affiliation(s)
- Simone Peraio
- Department of Neurosurgery, Meyer Children's Hospital IRCCS, Florence, Italy
| | - Giorgio Mantovani
- Department of Translational Medicine and for Romagna, University of Ferrara, Ferrara, Italy
| | - Tommaso Araceli
- Department of Neurosurgery, Meyer Children's Hospital IRCCS, Florence, Italy
- Department of Neurosurgery, University Hospital Regensburg, Regensburg, Germany
| | - Lorenzo Mongardi
- Department of Translational Medicine and for Romagna, University of Ferrara, Ferrara, Italy
| | - Alice Noris
- Department of Neurosurgery, Meyer Children's Hospital IRCCS, Florence, Italy
| | - Edoardo Fino
- Pediatric Neurology Clinic - Meyer Children's Hospital IRCCS, Florence, Italy
- University of Florence, Florence, Italy
| | - Francesca Formica
- Istituto Medea "La Nostra Famiglia" IRCCS, Bosisio Parini, LC, Italy
| | - Luigi Piccinini
- Istituto Medea "La Nostra Famiglia" IRCCS, Bosisio Parini, LC, Italy
| | - Federico Melani
- Pediatric Neurology Clinic - Meyer Children's Hospital IRCCS, Florence, Italy
| | - Matteo Lenge
- Pediatric Neurology Clinic - Meyer Children's Hospital IRCCS, Florence, Italy.
| | - Roberta Scalise
- Istituto Stella Maris - IRCCS - University of Pisa, Pisa, Italy
| | - Roberta Battini
- Istituto Stella Maris - IRCCS - University of Pisa, Pisa, Italy
| | - Andrea Di Rita
- Department of Neurosurgery, Meyer Children's Hospital IRCCS, Florence, Italy
| | - Ludovico D'Incerti
- Department of Radiology, Meyer Children's Hospital IRCCS, Florence, Italy
| | | | | | - Renzo Guerrini
- Pediatric Neurology Clinic - Meyer Children's Hospital IRCCS, Florence, Italy
- University of Florence, Florence, Italy
| | - Flavio Giordano
- Department of Neurosurgery, Meyer Children's Hospital IRCCS, Florence, Italy
- University of Florence, Florence, Italy
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Adamaszek M, Langner S, Mehrholz J, Heiinrich A. Opsoclonus-Myoclonus-Ataxia Syndrome Due to Covid-19. CEREBELLUM (LONDON, ENGLAND) 2024; 23:1245-1248. [PMID: 37814146 DOI: 10.1007/s12311-023-01610-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 09/26/2023] [Indexed: 10/11/2023]
Abstract
Opsoclonus-myoclonus syndrome (OMS) as a rare neurological encephalopathic entity associated with non-specific infections or cancer processes has been repeatedly described in the setting of SARS-CoV-2 infection. We report a case of a 53-year-old man with SARS-CoV-2 infection, who developed clinical features of opsoclonus-myoclonus ataxia syndrome including cognitive impairments with a prolonged course of disease. Of particular note, cerebrospinal fluid (CSF) analysis revealed the production of myelin oligodendrocyte glycoprotein (MOG) antibodies, suggesting an underlying neuroimmunological mechanism associated with infection with the novel SARS-CoV-2 virus.
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Affiliation(s)
- Michael Adamaszek
- Department of Neurological and Neurocognitive Rehabilitation, Klinik Bavaria Kreischa, An der Wolfsschlucht, 1-2 01731, Kreischa, Germany.
| | - Soenke Langner
- Department of Radiology, Rostock University Medical Center, Schillingallee 35, 18057, Rostock, Germany
| | - Jan Mehrholz
- Department of Public Health, Dresden Medical School, Technical University Dresden, Dresden, Germany
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Petriceks A, Vyas CM, Paudel S, Donovan AL, Van Alphen MU, Stern TA. Assessment and Treatment of Abnormal Involuntary Movements: A Clinically Focused Narrative Review. Harv Rev Psychiatry 2024; 32:47-57. [PMID: 38452284 DOI: 10.1097/hrp.0000000000000390] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 03/09/2024]
Abstract
LEARNING OBJECTIVES After participating in this CME activity, the psychiatrist should be better able to:• Categorize and describe different types of abnormal involuntary movements (AIMs).• Identify assessment tools and treatment options for AIMs. ABSTRACT Abnormal involuntary movements (AIMs) comprise a diverse group of movement disorders characterized by uncontrolled and unintended movements (e.g., tremors, tics, dystonia). AIMs can occur at any stage of life and pose significant challenges for clinicians. It is difficult to determine their underlying causes due to the complex neurobiological mechanisms involved. Therefore, it is crucial to quantify the severity and progression of AIMs using well-validated measurement scales, such as the Abnormal Involuntary Movement Scale (AIMS). By employing reliable assessment approaches, clinicians can objectively evaluate the motoric manifestations of AIMs and track them over time. Treatment of AIMs varies depending on their nature and etiology. While AIMs often respond to treatment, serious side effects can undermine treatment efficacy. In this clinically focused narrative review, we categorize different types of AIMs and discuss their neurobiological aspects. Further, we emphasize the importance of using well-validated measurement scales for accurate assessment and discuss available treatment modalities that target the specific AIMs manifestations. Additionally, we cover the need for comprehensive care to address the multifaceted nature of AIMs, accounting for their physical manifestations as well as their psychological, social, and functional toll on patients. By embracing a multidisciplinary approach, health care professionals can provide patient-centered care that promotes overall well-being and enhances the lives of patients coping with AIMs. Regular follow-up assessments are necessary to monitor treatment response, adjust medications when needed, and provide ongoing support for individuals affected by AIMs.
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Affiliation(s)
- Aldis Petriceks
- From Harvard Medical School, Boston, MA (Mr. Petriceks, Drs. Vyas, Paudel, Donovan, Van Alphen, and Stern); Department of Psychiatry, Massachusetts General Hospital, Boston, MA (Drs. Vyas, Paudel, Donovan, Van Alphen, and Stern); Atrius Health, Boston, MA (Dr. Van Alphen)
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Grippe T, Chen R. Utility of Neurophysiological Evaluation in Movement Disorders Clinical Practice. Mov Disord Clin Pract 2023; 10:1599-1610. [PMID: 38026509 PMCID: PMC10654828 DOI: 10.1002/mdc3.13856] [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: 11/25/2022] [Revised: 07/13/2023] [Accepted: 07/19/2023] [Indexed: 12/01/2023] Open
Abstract
Background Quantitative and objective neurophysiological assessment can help to define the predominant phenomenology and provide diagnoses that have prognostic and therapeutic implications for movement disorders. Objectives Evaluate the agreement between initial indications and final diagnoses after neurophysiological evaluations in a specialized movement disorders center. Methods Electrophysiological studies conducted for movement disorders from 2003 to 2021 were reviewed. The indications were classified according to predominant phenomenology and the diagnoses categorized in subgroups of phenomenology. Results A total of 509 studies were analyzed. 51% (259) of patients were female, with a mean age of 51 years (ranges 5 to 89 years). The most common reasons for referral were evaluation of functional movement disorders (FMD), followed by jerky movements, tremor and postural instability. Regarding FMD referrals, there was a diagnostic change in 13% of the patients after electrophysiological assessment. The patients with jerky movements as indication had a diagnosis other than myoclonus in 27% of them, and tremor was not confirmed in 20% of the cases. In patients with an electrophysiological diagnosis of FMD, it was not suspected in 30% of the referrals. Similarly, tremor was not mentioned in the referral of 17% of the patients with this electrophysiological diagnosis and myoclonus was not suspected in 13% of the cases. Conclusions Electrophysiological assessment has utility in the evaluation of movement disorders, even in patients evaluated by movement disorders neurologists. More studies are needed to standardize the protocols between centers and to promote the availability and use of these techniques among movement disorders clinics.
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Affiliation(s)
- Talyta Grippe
- Edmond J. Safra Program in Parkinson's Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, UHNTorontoOntarioCanada
- Division of NeurologyUniversity of TorontoTorontoOntarioCanada
- Neuroscience Graduate ProgramFederal University of Minas GeraisBelo HorizonteBrazil
| | - Robert Chen
- Edmond J. Safra Program in Parkinson's Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, UHNTorontoOntarioCanada
- Neuroscience Graduate ProgramFederal University of Minas GeraisBelo HorizonteBrazil
- Krembil Brain InstituteTorontoOntarioCanada
- Center for Advancing Neurotechnological Innovation to Application (CRANIA)TorontoOntarioCanada
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Sauerbier A, Gronostay A, Dafsari HS. SOP: acute hyperkinetic movement disorders. Neurol Res Pract 2023; 5:35. [PMID: 37496100 PMCID: PMC10373268 DOI: 10.1186/s42466-023-00260-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2023] [Accepted: 06/26/2023] [Indexed: 07/28/2023] Open
Abstract
INTRODUCTION Movement disorders emergencies describe acute-onset neurological conditions in which a delay of recognition and treatment may cause severe morbidity and mortality of patients. Hyperkinetic movement disorders include tremor, chorea/ballism, dystonia, myoclonus, and tics. Here we present a standard operating procedure (SOP) for the diagnostic work-up and different treatment options depending on the phenomenology as well as the aetiology of underlying diseases. COMMENTS The recognition of the phenomenology is essential for the symptomatic therapy of the acute movement disorder and forms the basis for the choice of ancillary investigations to confirm the suspected underlying causes. Furthermore, we summarise diagnostic techniques, including blood and cerebrospinal fluid tests and neuroimaging, which provide rapid results and are useful for the indication of causal treatments of specific acute movement disorders. CONCLUSIONS Despite their acute nature, most of these conditions can result in good clinical outcomes, if recognised early.
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Affiliation(s)
- Anna Sauerbier
- Faculty of Medicine, Department of Neurology, University of Cologne, University Hospital Cologne, Cologne, Germany
| | - Alexandra Gronostay
- Faculty of Medicine, Department of Neurology, University of Cologne, University Hospital Cologne, Cologne, Germany
| | - Haidar S Dafsari
- Faculty of Medicine, Department of Neurology, University of Cologne, University Hospital Cologne, Cologne, Germany.
- Department of Neurology, University Hospital Cologne, Kerpener Str. 62, 50937, Cologne, Germany.
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Hayase T, Saiga H, Yamaguchi T. Haloperidol-induced myoclonus in a patient with delirium. Geriatr Gerontol Int 2023; 23:243-244. [PMID: 36709514 DOI: 10.1111/ggi.14550] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2022] [Revised: 01/03/2023] [Accepted: 01/17/2023] [Indexed: 01/30/2023]
Affiliation(s)
- Tomomi Hayase
- Department of Palliative Medicine, Kobe University Hospital, Kobe, Japan
| | - Hiroshi Saiga
- Division of Gastrointestinal Surgery, Department of Surgery, Graduate School of Medicine, Kobe University, Kobe, Japan
| | - Takashi Yamaguchi
- Department of Palliative Medicine, Kobe University Hospital, Kobe, Japan
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Zhu XY, Tang XH, Yu H. Amiodarone-induced muscle tremor in an elderly patient: A case report. World J Clin Cases 2022; 10:12726-12733. [PMID: 36579101 PMCID: PMC9791518 DOI: 10.12998/wjcc.v10.i34.12726] [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: 09/07/2022] [Revised: 10/09/2022] [Accepted: 11/04/2022] [Indexed: 12/02/2022] Open
Abstract
BACKGROUND Amiodarone is a Class III antiarrhythmic drug, which has been adopted for the treatment of atrial fibrillation and ventricular arrhythmia. However, the use of amiodarone can cause lower limb muscle tremors, which is recognized as a rare side effect of this medication.
CASE SUMMARY An 84-year-old female was administrated with amiodarone for paroxysmal supraventricular tachycardia and frequent ventricular tachycardia. The patient developed a bilateral gastrocnemius tremor in the course of medication, and the strength of the patient’s bilateral knee flexor and extensor reached 4/5 and 3/5, respectively. After the use of amiodarone was stopped, and the patient was given a small dose of levetiracetam, the lower limb tremor symptoms were significantly mitigated, along with activity and function.
CONCLUSION Attention should be paid to the significance of the side effects of drugs in the elderly, which may be atypical in the elderly. The relevant side effects of drugs may not be as rare as reported due to individual differences and different pharmacokinetics. If the side effects are generated, the medication should be adjusted in time, and the progress of the side effects should be intervened.
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Affiliation(s)
- Xiao-Yong Zhu
- Department of Cardiology, Jiujiang University Affiliated Hospital, Jiujiang 332000, Jiangxi Province, China
| | - Xin-Hu Tang
- Department of Cardiology, Jiujiang University Affiliated Hospital, Jiujiang 332000, Jiangxi Province, China
| | - Hua Yu
- Department of Cardiology, Jiujiang University Affiliated Hospital, Jiujiang 332000, Jiangxi Province, China
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Müller MKR, Christ M, Naumann M, Bayas A. Prolonged-release fampridine for the treatment of myoclonus after cervical myelitis: a case report. Ther Adv Neurol Disord 2022; 15:17562864221083608. [PMID: 35371294 PMCID: PMC8968993 DOI: 10.1177/17562864221083608] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/15/2021] [Accepted: 02/09/2022] [Indexed: 11/29/2022] Open
Abstract
Prolonged-release fampridine (PR-FAM), a potassium channel blocker, is approved for improving walking ability in patients with multiple sclerosis (MS). Beyond this, positive effects on other MS symptoms like fatigue, cognition, and tremor have been described. To our knowledge, a positive effect of PR-FAM on spinal myoclonus has not been described so far. Here, we report a 32-year-old female with myoclonus after cervical myelitis affecting both hands which markedly improved after administration of PR-FAM. Treatments used before such as carbamazepine or levetiracetam had to be withdrawn because of intolerable side effects or lack of efficacy. The positive effect of PR-FAM could be confirmed by transient suspension. PR-FAM may be considered as a treatment option in refractory spinal myoclonus after myelitis in selected cases.
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Affiliation(s)
- Mona Klara Ros Müller
- Department of Neurology and Clinical Neurophysiology, University Hospital Augsburg, Augsburg, Germany
| | - Monika Christ
- Department of Neurology and Clinical Neurophysiology, University Hospital Augsburg, Augsburg, Germany
| | - Markus Naumann
- Department of Neurology and Clinical Neurophysiology, University Hospital Augsburg, Augsburg, Germany
| | - Antonios Bayas
- Department of Neurology and Clinical Neurophysiology, University Hospital Augsburg, Stenglinstrasse 2, 86156 Augsburg, Germany
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Elston MS, Elajnaf T, Hannan FM, Thakker RV. Autosomal dominant hypocalcemia type 1 (ADH1) associated with myoclonus and intracerebral calcifications. J Endocr Soc 2022; 6:bvac042. [PMID: 35402765 PMCID: PMC8989155 DOI: 10.1210/jendso/bvac042] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/08/2021] [Indexed: 11/19/2022] Open
Abstract
Abstract
Autosomal dominant hypocalcemia type 1 (ADH1) is a disorder of extracellular calcium homeostasis caused by germline gain-of-function mutations of the calcium-sensing receptor (CaSR). Over 35% of ADH1 patients have intracerebral calcifications predominantly affecting the basal ganglia. The clinical consequences of such calcifications remain to be fully characterized, although the majority of patients with these calcifications are considered to be asymptomatic. We report a 20-year-old female proband with a severe form of ADH1 associated with recurrent hypocalcemic and hypercalcemic episodes, persistent childhood hyperphosphatemia, and a low calcium/phosphate ratio. From the age of 18 years, she had experienced recurrent myoclonic jerks affecting the upper limbs that were not associated with epileptic seizures, extra-pyramidal features, cognitive impairment, or alterations in serum calcium concentrations. Computerised tomography (CT) scans revealed calcifications of the globus pallidus regions of the basal ganglia bilaterally, and also the frontal lobes at the grey-white matter junction, and posterior horn choroid plexuses. The patient’s myoclonus resolved following treatment with levetiracetam. CASR mutational analysis identified a reported germline gain-of-function heterozygous missense mutation, c.2363T>G; p.(Phe788Cys), which affects an evolutionarily conserved phenylalanine residue located in transmembrane domain helix 5 of the CaSR protein. Analysis of the cryo-electron microscopy CaSR structure predicted the wild-type Phe788 residue to form interactions with neighbouring phenylalanine residues, which likely maintain the CaSR in an inactive state. The p.(Phe788Cys) mutation was predicted to disrupt these interactions, thereby leading to CaSR activation. These findings reveal myoclonus as a novel finding in an ADH1 patient with intracerebral calcifications.
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Affiliation(s)
- Marianne S Elston
- Waikato Clinical Campus, University of Auckland, Hamilton, New Zealand
| | - Taha Elajnaf
- Nuffield Department of Women’s & Reproductive Health, University of Oxford, Oxford, UK
| | - Fadil M Hannan
- Nuffield Department of Women’s & Reproductive Health, University of Oxford, Oxford, UK
| | - Rajesh V Thakker
- Academic Endocrine Unit, Radcliffe Department of Medicine, University of Oxford, Oxford, UK
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Guo Y, Xiao Y, Chen LF, Yin DH, Wang RD. Lance Adams syndrome: two cases report and literature review. J Int Med Res 2022; 50:3000605211059933. [PMID: 35220777 PMCID: PMC8894979 DOI: 10.1177/03000605211059933] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Hypoxic myoclonus, also known as Lance Adams syndrome, is a rare syndrome that results from the serious brain damage caused by cerebral hypoxia that often follows cardiopulmonary resuscitation. This current case report describes two patients with post-hypoxic myoclonus, both of whom received cardiopulmonary resuscitation. The neurological symptoms of these two patients were significantly improved by the administration of clonazepam and sodium valproate sustained-release tablets. The report presents a literature review detailing the pathogenesis, diagnosis and treatment of Lance Adams syndrome. The timely diagnosis and treatment of Lance Adams syndrome can significantly improve the quality of life of patients. Valproic acid, clonazepam and other antiepileptic drugs can be used. Whether levetiracetam is effective for cortical myoclonus requires further clinical study.
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Affiliation(s)
- Yu Guo
- Department of Neurology, Second Affiliated Hospital of Army Medical University, Chongqing, China
| | - Yan Xiao
- Department of Neurology, Second Affiliated Hospital of Army Medical University, Chongqing, China
| | - Li-Fa Chen
- Department of Neurology, Second Affiliated Hospital of Army Medical University, Chongqing, China
| | - De-Hui Yin
- Department of Neurology, Second Affiliated Hospital of Army Medical University, Chongqing, China
| | - Ruo-Dan Wang
- Department of Neurology, Second Affiliated Hospital of Army Medical University, Chongqing, China
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Canine Lafora Disease: An Unstable Repeat Expansion Disorder. Life (Basel) 2021; 11:life11070689. [PMID: 34357061 PMCID: PMC8304204 DOI: 10.3390/life11070689] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2021] [Revised: 07/11/2021] [Accepted: 07/12/2021] [Indexed: 11/17/2022] Open
Abstract
Canine Lafora disease is a recessively inherited, rapidly progressing neurodegenerative disease caused by the accumulation of abnormally constructed insoluble glycogen Lafora bodies in the brain and other tissues due to the loss of NHL repeat containing E3 ubiquitin protein ligase 1 (NHLRC1). Dogs have a dodecamer repeat sequence within the NHLRC1 gene, which is prone to unstable (dynamic) expansion and loss of function. Progressive signs of Lafora disease include hypnic jerks, reflex and spontaneous myoclonus, seizures, vision loss, ataxia and decreased cognitive function. We studied five dogs (one Chihuahua, two French Bulldogs, one Griffon Bruxellois, one mixed breed) with clinical signs associated with canine Lafora disease. Identification of polyglucosan bodies (Lafora bodies) in myocytes supported diagnosis in the French Bulldogs; muscle areas close to the myotendinous junction and the myofascial union segment had the highest yield of inclusions. Postmortem examination of one of the French Bulldogs revealed brain Lafora bodies. Genetic testing for the known canine NHLRC1 mutation confirmed the presence of a homozygous mutation associated with canine Lafora disease. Our results show that Lafora disease extends beyond previous known breeds to the French Bulldog, Griffon Bruxellois and even mixed-breed dogs, emphasizing the likely species-wide nature of this genetic problem. It also establishes these breeds as animal models for the devastating human disease. Genetic testing should be used when designing breeding strategies to determine the frequency of the NHLRC1 mutation in affected breeds. Lafora diseases should be suspected in any older dog presenting with myoclonus, hypnic jerks or photoconvulsions.
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14
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Sakusic A, Rabinstein AA. Acute Coma. Neurol Clin 2021; 39:257-272. [PMID: 33896518 DOI: 10.1016/j.ncl.2021.01.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
An acutely comatose patient constitutes a medical emergency until proved otherwise. Managing these emergencies requires organized teamwork to recognize and treat life-threatening situations and reversible causes of coma. Once vital functions have been stabilized, information from the history and physical examination should be used to rationally guide subsequent testing. Identifying causes of coma for which emergency treatment is possible should be the priority. The treatment and prognosis depend on the cause.
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Affiliation(s)
- Amra Sakusic
- Department of Neurology, Mayo Clinic, 4500 San Pablo Road South (Attention: Cannaday Building 3W CIM), Jacksonville, FL 32224, USA
| | - Alejandro A Rabinstein
- Department of Neurology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA.
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15
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Tater P, Pandey S. Post-stroke Movement Disorders: Clinical Spectrum, Pathogenesis, and Management. Neurol India 2021; 69:272-283. [PMID: 33904435 DOI: 10.4103/0028-3886.314574] [Citation(s) in RCA: 49] [Impact Index Per Article: 12.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
Involuntary movements develop after 1-4% of strokes and they have been reported in patients with ischemic and hemorrhagic strokes affecting the basal ganglia, thalamus, and/or their connections. Hemichorea-hemiballism is the most common movement disorder following a stroke in adults while dystonia is most common in children. Tremor, myoclonus, asterixis, stereotypies, and vascular parkinsonism are other movement disorders seen following stroke. Some of them occur immediately after acute stroke, some can develop later, and others may have delayed onset progressive course. Proposed pathophysiological mechanisms include neuronal plasticity, functional diaschisis, and age-related differences in brain metabolism. There are no guidelines regarding the management of post-stroke movement disorders, mainly because of their heterogeneity.
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Affiliation(s)
- Priyanka Tater
- Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, New Delhi, India
| | - Sanjay Pandey
- Department of Neurology, Govind Ballabh Pant Postgraduate Institute of Medical Education and Research, New Delhi, India
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16
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Mure H, Toyoda N, Morigaki R, Fujita K, Takagi Y. Clinical Outcome and Intraoperative Neurophysiology of the Lance-Adams Syndrome Treated with Bilateral Deep Brain Stimulation of the Globus Pallidus Internus: A Case Report and Review of the Literature. Stereotact Funct Neurosurg 2020; 98:399-403. [PMID: 32894852 DOI: 10.1159/000509318] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2020] [Accepted: 06/10/2020] [Indexed: 11/19/2022]
Abstract
BACKGROUND The Lance-Adams syndrome (LAS) is a myoclonus syndrome caused by hypoxic-ischemic encephalopathy. LAS cases could be refractory to first-line medications, and the neuronal mechanism underlying LAS pathology remains unknown. OBJECTIVES To describe a patient with LAS who underwent bilateral globus pallidus internus (GPi) stimulation and discuss the pathophysiology of LAS with intraoperative electrophysiological findings. PATIENTS A 79-year-old woman presented with a history of cardiopulmonary arrest due to internal carotid artery rupture following carotid endarterectomy after successful cardiopulmonary resuscitation. However, within 1 month, the patient developed sensory stimulation-induced myoclonus in her face and extremities. Because her myoclonic symptoms were refractory to pharmacotherapy, deep brain stimulation of the GPi was performed 1 year after the hypoxic attack. RESULTS Continuous bilateral GPi stimulation with optimal parameter settings remarkably improved the patient's myoclonic symptoms. At the 2-year follow-up, her Unified Myoclonus Rating Scale score decreased from 90 to 24. In addition, we observed burst firing and interburst pause patterns on intraoperative microelectrode recordings of the bilateral GPi and stimulated this area as the therapeutic target. CONCLUSION Our results show that impairment in the basal ganglion circuitry might be involved in the pathogenesis of myoclonus in patients with LAS.
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Affiliation(s)
- Hideo Mure
- Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan, .,Parkinson's Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima, Japan,
| | - Naoto Toyoda
- Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan
| | - Ryoma Morigaki
- Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan.,Parkinson's Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima, Japan.,Department of Advanced Brain Research, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan
| | - Koji Fujita
- Parkinson's Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima, Japan.,Department of Clinical Neuroscience, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan
| | - Yasushi Takagi
- Department of Neurosurgery, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan.,Department of Advanced Brain Research, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Japan
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Celli D, Marquez A, Byer M, Colombo R. Levetiracetam for the treatment of myoclonic neurotoxicity induced by amiodarone. HeartRhythm Case Rep 2020; 6:488-490. [PMID: 32817824 PMCID: PMC7424226 DOI: 10.1016/j.hrcr.2020.04.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/31/2022] Open
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18
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Park JM, Kim WJ, Han JS, Park SY, Park SN. Management of palatal myoclonic tinnitus based on clinical characteristics: a large case series study. Acta Otolaryngol 2020; 140:553-557. [PMID: 32406274 DOI: 10.1080/00016489.2020.1749724] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
Background: Palatal myoclonic tinnitus (PMT) is a rare otological condition caused by rhythmic contractions of soft palate muscles. Due to its rarity, only a few case series studies have been reported in the literature at the present time.Aims: This large case series study reviews treatment outcomes of PMT patients over the past 15 years based on clinical characteristics.Materials and methods: Between the year 2003 and 2018, 54 patients with a diagnosis of PMT were assessed. Clinical characteristics, audiological findings, psychological status, and other relevant medical history were thoroughly reviewed. Response to various treatment modalities were retrospectively analyzed.Results: The mean age of enrolled patients was 29.0 ± 16.4 years, with female gender predominance. All of the patients complained of 'clicky' sounding tinnitus. Twenty-nine patients had comorbid tinnitus of other type. Reassurance and behavior therapy was sufficient for young patients. Medication was effective in 44.4% of the patients. Botulinum toxin injection in the palate led to complete resolution of symptoms in a majority of intractable PMT patients.Conclusions and significance: Management of PMT should be customized according to the individual clinical characteristics of the patients. This study may provide insightful information to establish optimal treatment modalities for PMT.
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Affiliation(s)
- Jung Mee Park
- Department of Otolaryngology-Head and Neck Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea and Department of Otorhinolaryngology-Head and Neck Surgery, Gangneung Asan Hospital, College of Medicine University of Ulsan, Gangneung, Republic of Korea
| | - Woo Jin Kim
- Department of Otolaryngology-Head and Neck Surgery, Busan Paik Hospital, College of Medicine, Inje University, Busan, Republic of Korea
| | - Jae Sang Han
- Department of Otolaryngology-Head and Neck Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea and Department of Otorhinolaryngology-Head and Neck Surgery, Gangneung Asan Hospital, College of Medicine University of Ulsan, Gangneung, Republic of Korea
| | - So Young Park
- Department of Otolaryngology-Head and Neck Surgery, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
| | - Shi Nae Park
- Department of Otolaryngology-Head and Neck Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea and Department of Otorhinolaryngology-Head and Neck Surgery, Gangneung Asan Hospital, College of Medicine University of Ulsan, Gangneung, Republic of Korea
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Fearon C, Peall KJ, Vidailhet M, Fasano A. Medical management of myoclonus-dystonia and implications for underlying pathophysiology. Parkinsonism Relat Disord 2020; 77:48-56. [PMID: 32622300 DOI: 10.1016/j.parkreldis.2020.06.016] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/08/2020] [Revised: 05/19/2020] [Accepted: 06/16/2020] [Indexed: 12/14/2022]
Abstract
Myoclonus-dystonia is an early onset genetic disorder characterised by subcortical myoclonus and less prominent dystonia. Its primary causative gene is the epsilon-sarcoglycan gene but the syndrome of "myoclonic dystonia" has been shown to be a heterogeneous group of genetic disorders. The underlying pathophysiology of myoclonus-dystonia is incompletely understood, although it may relate to dysfunction of striatal monoamine neurotransmission or disruption of cerebellothalamic networks (possibly via a GABAergic deficit of Purkinje cells). A broad range of oral medical therapies have been used in the treatment of myoclonus-dystonia with a varying response, and limited data relating to efficacy and tolerability, yet this condition responds dramatically to alcohol. Few well conducted randomized controlled trials have been undertaken leading to an empirical ad hoc approach for many patients. We review the current evidence for pharmacological therapies in myoclonus-dystonia, discuss implications for underlying pathogenesis of the condition and propose a treatment algorithm for these patients.
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Affiliation(s)
- Conor Fearon
- Dublin Neurological Institute at the Mater Misericordiae University Hospital, Dublin, Ireland
| | - Kathryn J Peall
- Neurosciences and Mental Health Research Institute, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, CF24 4HQ, UK
| | - Marie Vidailhet
- AP-HP, Hôpital Salpetriere, Department of Neurology, F-75013, Paris, France; Institut du Cerveau et de la Moelle, ICM, F-75013, Paris, France; INSERM U1127, CNRS UMR 7225, Sorbonne Unversité, F-75013, Paris, France
| | - Alfonso Fasano
- Edmond J. Safra Program in Parkinson's Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital - UHN, Division of Neurology, University of Toronto, Toronto, Ontario, Canada; Krembil Research Institute, Toronto, Ontario, Canada; Center for Advancing Neurotechnological Innovation to Application (CRANIA), Toronto, ON, Canada.
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20
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Perampanel in refractory post-hypoxic myoclonus: see the difference! Acta Neurol Belg 2020; 120:741-742. [PMID: 31848939 DOI: 10.1007/s13760-019-01259-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2019] [Accepted: 11/26/2019] [Indexed: 10/25/2022]
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21
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Besa Lehmann V, Rosenbaum M, Bulman DE, Read T, Verhagen Metman L. A Case Report of Myoclonus-Dystonia with Isolated Myoclonus Phenotype and Novel Mutation Successfully Treated with Deep Brain Stimulation. Neurol Ther 2020; 9:187-191. [PMID: 32274660 PMCID: PMC7229070 DOI: 10.1007/s40120-020-00186-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2019] [Indexed: 12/27/2022] Open
Abstract
INTRODUCTION Myoclonus-dystonia is an inherited disorder characterized by a combination of myoclonic jerks and dystonia. Mutations in the epsilon-sarcoglycan gene (SGCE) represent the main known genetic cause. In the last few years, deep brain stimulation (DBS) has shown significant promise in treating these patients. There is only one report in the literature of a patient with positive SGCE mutation and isolated myoclonus phenotype who has been successfully treated with DBS. CASE PRESENTATION We present a case of a 16-year-old young man with a history of quick jerks since childhood. They progressed gradually over the years involving the entire body and interfering with most of his daily activities. He had no dystonia. Genetic testing identified a single base deletion in exon 3 of the SGCE gene, considered very likely pathogenic. After unsuccessfully trying several oral medications, he underwent DBS of the globus pallidus internus (GPi). His Unified Myoclonus Rating Scale score during rest and with action improved by 92.8% and 82.6%, respectively. DISCUSSION The striking effect of DBS on myoclonic jerks confirms the superior benefit of DBS over oral medications. Further study is needed to determine the role of mutation status in predicting DBS response, especially considering that myoclonus-dystonia is genetically heterogeneous. CONCLUSION Our case confirms the poor response to oral medications and supports the use of GPi DBS for patients with genetically confirmed myoclonus-dystonia and isolated-myoclonus phenotype. In addition, our case represents familial myoclonus-dystonia due to a novel SGCE mutation.
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Affiliation(s)
| | - Marc Rosenbaum
- Department of Neurological Sciences, Rush University Medical Center, Chicago, IL, USA
| | - Dennis E Bulman
- Department of Pediatrics, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada
| | - Tara Read
- Department of Biochemistry, Microbiology and Immunology, University of Ottawa, Ottawa, ON, Canada
| | - Leo Verhagen Metman
- Department of Neurological Sciences, Rush University Medical Center, Chicago, IL, USA
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22
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Der-Nigoghossian C, Rubinos C, Alkhachroum A, Claassen J. Status epilepticus - time is brain and treatment considerations. Curr Opin Crit Care 2020; 25:638-646. [PMID: 31524720 DOI: 10.1097/mcc.0000000000000661] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
PURPOSE OF REVIEW Status epilepticus is a neurological emergency associated with high morbidity and mortality. There is a lack of robust data to guide the management of this neurological emergency beyond the initial treatment. This review examines recent literature on treatment considerations including the choice of continuous anesthetics or adjunctive anticonvulsant, the cause of the status epilepticus, and use of nonpharmacologic therapies. RECENT FINDINGS Status epilepticus remains undertreated and mortality persists to be unchanged over the past 30 years. New anticonvulsant choices, such as levetiracetam and lacosamide have been explored as alternative emergent therapies. Anecdotal reports on the use of other generation anticonvulsants and nonpharmacologic therapies for the treatment of refractory and super-refractory status epilepticus have been described.Finally, recent evidence has examined etiology-guided management of status epilepticus in certain patient populations, such as immune-mediated, paraneoplastic or infectious encephalitis and anoxic brain injury. SUMMARY Randomized clinical trials are needed to determine the role for newer generation anticonvulsants and nonpharmacologic modalities for the treatment of epilepticus remains and evaluate the long-term outcomes associated with continuous anesthetics.
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Affiliation(s)
| | - Clio Rubinos
- Division of Neurocritical Care, Department of Neurology, Columbia University, New York, New York, USA
| | - Ayham Alkhachroum
- Division of Neurocritical Care, Department of Neurology, Columbia University, New York, New York, USA
| | - Jan Claassen
- Division of Neurocritical Care, Department of Neurology, Columbia University, New York, New York, USA
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Rissardo JP, Caprara ALF. Pregabalin-associated movement disorders: A literature review. Brain Circ 2020; 6:96-106. [PMID: 33033779 PMCID: PMC7511912 DOI: 10.4103/bc.bc_57_19] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2019] [Revised: 05/12/2020] [Accepted: 06/03/2020] [Indexed: 12/21/2022] Open
Abstract
Central nervous system adverse effects are commonly reported with pregabalin (PGB). On the other hand, movement disorders (MDs) associated with this drug were rarely described. However, their occurrence could significantly affect the quality of life of PGB users. This literature review aims to evaluate the clinical epidemiological profile, pathological mechanisms, and management of PGB-associated MDs. Relevant reports in six databases were identified and assessed by two reviewers without language restriction. A total of 46 reports containing 305 cases from 17 countries were assessed. The MDs encountered were as follows: 184 individuals with ataxia, 61 with tremors, 39 with myoclonus, 8 with parkinsonism, 1 with restless legs syndrome, 1 with dystonia, 1 with dyskinesia, and 1 with akathisia. The mean age was 62 years (range: 23-94). The male sex was slightly predominant with 54.34%. The mean PGB dose when the MD occurred was 238 mg, and neuropathic pain was the most common indication of PGB. The time from PGB start to MD was < 1 month at 75%. The time from PGB withdrawal to recovery was < 1 week at 77%. All the individuals where the follow-up was reported had a full recovery. The most common management was PGB withdrawal. In the literature, the majority of the cases did not report information about timeline events, neurological examination details, or electrodiagnostic studies. The best management for all MDs is probably PGB withdrawal. If the patient is on dialysis program, perhaps an increased number of sessions will decrease recovery time. Furthermore, the addition of a benzodiazepine could accelerate recovery.
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Abstract
Common genetic generalised epilepsy syndromes encountered by clinicians include childhood and juvenile absence epilepsies, juvenile myoclonic epilepsy and generalised tonic-clonic seizures on awakening. Treatment of these syndromes involves largely the use of broad-spectrum antiseizure drugs. Those effective for the generalised epilepsies include sodium valproate, phenobarbital, ethosuximide, clobazam, clonazepam, lamotrigine, levetiracetam, topiramate, zonisamide and, more recently, perampanel and brivaracetam. Results from the few rigorous studies comparing outcomes with drugs for genetic generalised epilepsies show valproate to be the most effective. The majority of patients with genetic generalised epilepsy syndromes will become seizure free on antiseizure monotherapy; those for whom control proves elusive may benefit from combination regimens. Early counselling regarding management may assist the patient to come to terms with their diagnosis and improve long-term outcomes. Treatment can be lifelong in some individuals, although others may remain seizure free without medication. Choice of antiseizure medication depends on the efficacy for specific seizure types, as well as tolerability. For patients prescribed comedication, drug interactions should be considered. In particular, for young women taking oral hormonal contraceptives, ≥ 200 mg/day of topiramate can decrease the circulating concentration of ethinylestradiol and ≥ 12 mg/day of perampanel can induce levonorgestrel metabolism. The use of valproate in women of childbearing potential is limited by associated teratogenic and neurodevelopmental effects in offspring. Given that valproate is often the antiseizure drug of choice for genetic generalised epilepsies, this creates a dilemma for patients and clinicians. Decision making can be aided by comprehensive assessment and discussion of treatment options. Psychiatric comorbidities are common in adolescents and adults with genetic generalised epilepsies. These worsen the prognosis, both in terms of seizure control and quality of life. Attendant lifestyle issues can impact significantly on the individual and society. Frontal lobe dysfunction, which can present in patients with juvenile myoclonic epilepsy, can adversely affect the long-term outlook, regardless of the nature of seizure control. Ongoing management requires consideration of psychosocial and behavioural factors that can complicate diagnosis and treatment. An assured supportive attitude by the neurologist can be an important contributor to a positive outcome. The mechanisms underlying genetic generalised epilepsies, including genetic abnormalities, are unclear at present. As the pathophysiology is unravelled, this may lead to the development of novel therapies and improved outcomes for patients with these syndromes.
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Affiliation(s)
- Linda J Stephen
- West Glasgow Ambulatory Care Hospital, Dalnair St, Glasgow, G3 8SJ, UK.
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25
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Hong JS, Moran MT, Eaton LA, Grafton LM. Neurologic, Cognitive, and Behavioral Consequences of Opioid Overdose: a Review. CURRENT PHYSICAL MEDICINE AND REHABILITATION REPORTS 2019. [DOI: 10.1007/s40141-019-00247-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
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Riboldi GM, Frucht SJ. Increasing Evidence for the Use of Sodium Oxybate in Multi-Drug-Resistant Lance-Adams Syndrome. TREMOR AND OTHER HYPERKINETIC MOVEMENTS (NEW YORK, N.Y.) 2019; 9:642. [PMID: 31413889 PMCID: PMC6691605 DOI: 10.7916/d8-rnsh-c024] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/24/2019] [Accepted: 03/04/2019] [Indexed: 12/01/2022]
Abstract
Background Treatment of posthypoxic myoclonus (PHM) can be a challenge in patients not responsive to first-line medications. PMH is a rare condition that has a dramatic impact on patients' quality of life. Refractory cases are not uncommon. Case report We report a patient with PHM non-responsive to conventional treatments who showed a dramatic improvement with sodium oxybate (SBX). Cases of PHM treated with SBX reported in the literature were reviewed. Discussion Resting and stimulus-induced myoclonus respond robustly to SBX, with significant improvement in patients' quality of life. SBX may be considered in patients with PHM resistant to first-line medications.
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Affiliation(s)
- Giulietta M Riboldi
- The Marlene and Paolo Fresco Institute for Parkinson's and Movement Disorders, Department of Neurology, New York University School of Medicine, New York, NY, USA
| | - Steven J Frucht
- The Marlene and Paolo Fresco Institute for Parkinson's and Movement Disorders, Department of Neurology, New York University School of Medicine, New York, NY, USA
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Freitas ME, Ruiz-Lopez M, Dalmau J, Erro R, Privitera M, Andrade D, Fasano A. Seizures and movement disorders: phenomenology, diagnostic challenges and therapeutic approaches. J Neurol Neurosurg Psychiatry 2019; 90:920-928. [PMID: 30796133 DOI: 10.1136/jnnp-2018-320039] [Citation(s) in RCA: 20] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2018] [Revised: 02/06/2019] [Accepted: 02/07/2019] [Indexed: 02/07/2023]
Abstract
Seizures and movement disorders (MDs) are distinct neurological conditions presenting with abnormal movements. Despite sharing an overlap in phenomenology, these movements have different origins. In order to explore the overlaps and the narrow boundaries between these two conditions, we performed a review of the literature to explore the risk of seizures in MDs. We discussed the mimics and chameleons including MDs that look like seizure (eg, paroxysmal dyskinesia, status dystonicus) and seizures that look like MDs (eg, epilepsia partialis continua, nocturnal frontal lobe epilepsy). Additionally, we examined the therapeutic challenges as well as the anatomical and chemical pathways relevant in the interplay between epilepsy and MDs. Finally, we proposed an algorithm to guide clinicians towards the final diagnosis of conditions characterised by the co-occurrence of MDs and seizures.
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Affiliation(s)
- Maria Eliza Freitas
- Medicine, McMaster University Division of Neurology, Hamilton, Ontario, Canada
| | - Marta Ruiz-Lopez
- Service of Neurology, Fundación Jimenez Diaz University Hospital, Madrid, Spain
| | - Josep Dalmau
- Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Roberto Erro
- Department of Medicine, Surgery and Dentistry 'Scuola Medica Salernitana', University of Salerno, UCL Institute of Neurology, Baronissi, Italy
| | - Michael Privitera
- Epilepsy Center, University of Cincinnati Gardner Neuroscience Institute, Cincinnati, Ohio, USA
| | | | - Alfonso Fasano
- Neurology, Krembil Brain Institute; Movement Disorders Centre and the Edmond J Safra Program in Parkinson's Disease, Toronto, Ontario, Canada
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28
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Camargo CHF, Teive HAG. Use of botulinum toxin for movement disorders. Drugs Context 2019; 8:212586. [PMID: 31258617 PMCID: PMC6586173 DOI: 10.7573/dic.212586] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2019] [Revised: 05/23/2019] [Accepted: 05/23/2019] [Indexed: 01/04/2023] Open
Abstract
The term movement disorders encompasses all disorders hypokinetic and hyperkinetic, which were previously known as extrapyramidal syndromes. With the definition of movement disorders and their diagnostic criteria and classifications, new studies for therapeutics could be performed. New drugs were launched, functional neurosurgery was developed, and the introduction of botulinum toxin (BoNT) for hyperkinesias was introduced. BoNT is an important therapy for dystonia, tics, myoclonus, and tremors. The aim of this review is to present the new and well-established uses of BoNT for movement disorders.
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Affiliation(s)
- Carlos Henrique Ferreira Camargo
- Neurological Diseases Group, Graduate Program of Internal Medicine, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil
| | - Hélio Afonso Ghizoni Teive
- Neurological Diseases Group, Graduate Program of Internal Medicine, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil.,Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil
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Abstract
INTRODUCTION Epilepsy is a prominent feature of myoclonic epilepsy with ragged-red fibers (MERRF)-syndrome. The most frequent seizure type is myoclonic seizures, of which the treatment is challenging and empiric. AREAS COVERED Herein, the author summarises and discusses previous and recent findings of antiepileptic drug (AED) treatment in MERRF-syndrome. EXPERT OPINION MERRF-syndrome is a predominantly maternally inherited, multisystem mitochondrial disorder caused by pathogenic variants predominantly of the mitochondrial DNA (mtDNA). Canonical clinical features of MERRF include myoclonus, epilepsy, ataxia, and myopathy. Additionally, other manifestations in the CNS, peripheral nerves, eyes, ears, heart, gastrointestinal tract, and endocrine organs may occur (MERRF-plus). Today, MERRF is considered rather as myoclonic ataxia than as myoclonic epilepsy. Genotypically, MERRF is due to mutations in 13 mtDNA-located genes and 1 nDNA-located gene. According to the modified Smith-score, the strongest gene-disease relationship exists for MT-TK, MT-TL1, and POLG1. Epilepsy is the second most frequent phenotypic feature of MERRF. Seizure-types associated with MERRF include focal myoclonic, focal clonic, and focal atonic seizures, generalized myoclonic, tonic-clonic, atonic, and myoclonic-atonic seizures, or typical absences. Treatment of myoclonic epilepsy relies on expert judgments recommending levetiracetam, together with clonazepam, or topiramate, zonisamide, or piracetam in monotherapy as the first line AEDs.
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Affiliation(s)
- Josef Finsterer
- a Krankenanstalt Rudolfstiftung , Messerli Institute , Vienna , Austria
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Desai A, Kherallah Y, Szabo C, Marawar R. Gabapentin or pregabalin induced myoclonus: A case series and literature review. J Clin Neurosci 2019; 61:225-234. [DOI: 10.1016/j.jocn.2018.09.019] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2018] [Revised: 08/21/2018] [Accepted: 09/24/2018] [Indexed: 01/26/2023]
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Su LJ, Wang YL, Han T, Qiao S, Zang KJ, Wu HK, Su YX, Liu LL, Liu XW. Antimyoclonic Effect of Levetiracetam and Clonazepam Combined Treatment on Myoclonic Epilepsy with Ragged-Red Fiber Syndrome with m.8344A>G Mutation. Chin Med J (Engl) 2018; 131:2433-2438. [PMID: 30334528 PMCID: PMC6202596 DOI: 10.4103/0366-6999.243568] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2018] [Indexed: 11/27/2022] Open
Abstract
BACKGROUND Treatment of myoclonic seizures in myoclonic epilepsy with ragged-red fibers (MERRFs) has been empirical and ineffective. Guideline on this disease is not available. Additional trials must be conducted to find more suitable treatments for it. In this study, the antimyoclonic effects of monotherapies, including levetiracetam (LEV), clonazepam (CZP), valproic acid (VPA), and topiramate (TPM) compared to combination therapy group with LEV and CZP on MERRF, were evaluated to find a more advantageous approach on the treatment of myoclonic seizures. METHODS Treatments of myoclonic seizures with VPA, LEV, CZP, and TPM were reported as monotherapies in 17 MERRF patients from Qilu Hospital between 2003 and 2016, who were diagnosed through clinical data and genetic testing. After 1-4 months of follow-up (mean: 82.9 ± 28.1 days), 12 patients that exhibited poor responses to monotherapy were given a combined treatment consisting of LEV and CZP subsequently. The follow-up period was 4-144 months (mean: 66.3 ± 45.3 months), the effective rates of monotherapy group (17 patients) and combination therapy group (12 patients) were analyzed by Chi-square test. RESULTS The m.8344 A>G mutation was detected in all patients. There were four patients with partial response (4/17, two in the CZP group and two in the LEV group), ten patients with stable disease (10/17, six in the CZP group, three in the LEV group, and one in the TPM group), and three patients with progressive disease (3/18, two in the VPA group and one in the TPM group). Twelve of the patients with LEV combined with CZP showed a positive effect and good tolerance (12/12), eight of them demonstrated improved cognition and coordination. There was a significant difference between the monotherapy group and combination therapy group in the efficacy of antimyoclonic seizures (χ2 = 13.7, P < 0.001). CONCLUSIONS LEV in combination with CZP is an efficient and safe treatment for myoclonic seizures in patients with this disease exhibiting the m.8344A>G mutation.
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Affiliation(s)
- Li-Jun Su
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
| | - Yu-Liang Wang
- Department of Neurology, Binzhou Medical University Hospital, Binzhou, Shandong 256603, China
| | - Tao Han
- Department of Neurology, Xuanwu Hospital Capital Medical University, Beijing 100053, China
| | - Shan Qiao
- Department of Neurology, Qianfoshan Hospital of Shandong Province, Jinan, Shandong 250014, China
| | - Ke-Jun Zang
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
| | - Huai-Kuan Wu
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
| | - Yong-Xin Su
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
| | - Ling-Ling Liu
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
| | - Xue-Wu Liu
- Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong 251102, China
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Bally JF, Méneret A, Roze E, Anderson M, Grabli D, Lang AE. Systematic review of movement disorders and oculomotor abnormalities in Whipple's disease. Mov Disord 2018; 33:1700-1711. [DOI: 10.1002/mds.27419] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2017] [Revised: 03/20/2018] [Accepted: 03/25/2018] [Indexed: 12/19/2022] Open
Affiliation(s)
- Julien F. Bally
- Movement Disorders Research Center, Toront Western Hospital; Toronto Ontario Canada
- Department of Neurology; University Hospitals of Geneva; Geneva Switzerland
| | - Aurélie Méneret
- AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Neurologie; Paris France
| | - Emmanuel Roze
- AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Neurologie; Paris France
| | - Melanie Anderson
- Library and Information Services; University Health Network; Toronto Ontario Canada
| | - David Grabli
- AP-HP, Hôpital de la Pitié-Salpêtrière, Département de Neurologie; Paris France
| | - Anthony E. Lang
- Movement Disorders Research Center, Toront Western Hospital; Toronto Ontario Canada
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Ocular flutter, generalized myoclonus, and ataxia associated with anti-GM1, GD1a, and GD1b antibodies in a 6-year-old child. Neurol Sci 2018; 39:1801-1803. [DOI: 10.1007/s10072-018-3476-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2018] [Accepted: 06/12/2018] [Indexed: 10/28/2022]
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Kapoor A, Kinsella L. A rare case of isolated myoclonus in an elderly male without a history of epilepsy. Clin Neurophysiol Pract 2018; 3:96-98. [PMID: 30215016 PMCID: PMC6133782 DOI: 10.1016/j.cnp.2017.11.002] [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: 11/16/2017] [Accepted: 11/29/2017] [Indexed: 11/16/2022] Open
Abstract
We describe a unique presentation of isolated lingual myoclonus presenting as intermittent expressive aphasia associated with uninterrupted intrusion-protrusion movement of the posterior tongue and adjoining hyoid. We discuss the paucity of evidence and suggest differential diagnosis as well as a brief discussion on possible underlying pathophysiology. Highlight previously described movement disorders and associated cases and draw parallels to isolated lingual myoclonus. Consolidate literature regarding the management of lingual myoclonus Aim Through this case report we attempt to highlight the presentation, initial investigation and management of lingual myoclonus as well as consolidate relevant literature. Case We present a unique case of a 72-year-old man who was admitted to the hospital for a sudden onset episodic speech arrest. Lingual myoclonus, an isolated movement disorder, manifested as an intermittent expressive aphasia secondary to the intrusion-protrusion movements of his tongue. During this time, the patient remained conscious and was able to continue to follow commands. Initial diagnostic evaluation with a CT scan, MRI and EEG failed to illicit a clear underlying etiology and the patient was empirically treated with valproic acid with complete resolution of his symptoms. Discussion This unusual presentation represents a rare disorder which is not well described in literature. Initial evaluation of which required excluding associated etiologies including strokes, seizures, medications/toxins or CNS infections. Without a clear etiology on initial diagnostic evaluation, the patient was empirically treated as no clear guidelines exist. This case presentation is an attempt to add to the current understanding of lingual myoclonus.
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Affiliation(s)
- Aniruddh Kapoor
- Dept of Internal Medicine, SSM Health St Mary's Hospital, 6420 Clayton Rd, Richmond Heights, MO 63117, United States
| | - Laurence Kinsella
- Dept of Internal Medicine, SSM Health St Mary's Hospital, 6420 Clayton Rd, Richmond Heights, MO 63117, United States
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Mioclonías inducidas por salbutamol. BIOMEDICA 2018; 38:303-307. [DOI: 10.7705/biomedica.v38i3.3813] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/20/2017] [Revised: 02/06/2018] [Indexed: 01/22/2023]
Abstract
El salbutamol es un agonista adrenérgico β2 ampliamente empleado en pacientes con enfermedades pulmonares obstructivas y restrictivas. Sus principales efectos secundarios son la taquicardia y el temblor. Las mioclonías son contracciones musculares involuntarias, irregulares, bruscas, breves y repentinas, y pueden ser generalizadas, focales o multifocales.Se presenta el caso de un paciente de 61 años con mioclonías de difícil manejo que solo presentó mejoría tras la suspensión definitiva del agonista adrenérgico β2. Se describen los hallazgos clínicos, las intervenciones y el resultado en las mioclonías asociadas con el uso de salbutamol y se discuten la posible génesis y la importancia de este efecto adverso. Para documentar el caso, se siguieron las recomendaciones de las guías para el reporte de casos (CAse REport, CARE).Aunque en diversos estudios se han descrito mioclonías secundarias al uso de diferentes fármacos, hasta donde se sabe, este sería el cuarto reporte de un caso asociado específicamente con el uso del salbutamol.
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Nardone R, Versace V, Höller Y, Sebastianelli L, Brigo F, Lochner P, Golaszewski S, Saltuari L, Trinka E. Transcranial magnetic stimulation in myoclonus of different aetiologies. Brain Res Bull 2018; 140:258-269. [DOI: 10.1016/j.brainresbull.2018.05.016] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2018] [Revised: 05/12/2018] [Accepted: 05/18/2018] [Indexed: 12/29/2022]
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Reynolds AS, Rohaut B, Holmes MG, Robinson D, Roth W, Velazquez A, Couch CK, Presciutti A, Brodie D, Moitra VK, Rabbani LE, Agarwal S, Park S, Roh DJ, Claassen J. Early myoclonus following anoxic brain injury. Neurol Clin Pract 2018; 8:249-256. [PMID: 30105165 DOI: 10.1212/cpj.0000000000000466] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2017] [Accepted: 03/06/2018] [Indexed: 11/15/2022]
Abstract
Background It is unknown whether postanoxic cortical and subcortical myoclonus are distinct entities with different prognoses. Methods In this retrospective cohort study of 604 adult survivors of cardiac arrest over 8.5 years, we identified 111 (18%) patients with myoclonus. Basic demographics and clinical characteristics of myoclonus were collected. EEG reports, and, when available, raw video EEG, were reviewed, and all findings adjudicated by 3 authors blinded to outcomes. Myoclonus was classified as cortical if there was a preceding, time-locked electrographic correlate and otherwise as subcortical. Outcome at discharge was determined using Cerebral Performance Category. Results Patients with myoclonus had longer arrests with less favorable characteristics compared to patients without myoclonus. Cortical myoclonus occurred twice as often as subcortical myoclonus (59% vs 23%, respectively). Clinical characteristics during hospitalization did not distinguish the two. Rates of electrographic seizures were higher in patients with cortical myoclonus (43%, vs 8% with subcortical). Survival to discharge was worse for patients with myoclonus compared to those without (26% vs 39%, respectively), but did not differ between subcortical and cortical myoclonus (24% and 26%, respectively). Patients with cortical myoclonus were more likely to be discharged in a comatose state than those with subcortical myoclonus (82% vs 33%, respectively). Among survivors, good functional outcome at discharge was equally possible between those with cortical and subcortical myoclonus (12% and 16%, respectively). Conclusions Cortical and subcortical myoclonus are seen in every sixth patient with cardiac arrest and cannot be distinguished using clinical criteria. Either condition may have good functional outcomes.
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Affiliation(s)
- Alexandra S Reynolds
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Benjamin Rohaut
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Manisha G Holmes
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - David Robinson
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - William Roth
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Angela Velazquez
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Caroline K Couch
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Alex Presciutti
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Daniel Brodie
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Vivek K Moitra
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - LeRoy E Rabbani
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Sachin Agarwal
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Soojin Park
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - David J Roh
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
| | - Jan Claassen
- Departments of Neurology (ASR, BR, MGH, DR, WR, AV, CKC, AP, SA, SP, DJR, JC), Medicine (DB, LER), and Anesthesiology (VKM), Columbia University Medical Center; and Department of Neurology (MGH), New York University Medical Center, New York
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Affiliation(s)
- H. Mihrimah Ozturk
- Department of Psychiatry, Ufuk University Faculty of Medicine, Ankara, Turkey
| | - Gülin Morkavuk
- Department of Neurology, Ufuk University Faculty of Medicine, Ankara, Turkey
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Ryu D, Kim J. Stimulus‐sensitive myoclonus with trigeminal neuralgia. Eur J Neurol 2017; 24:e75. [DOI: 10.1111/ene.13386] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2017] [Accepted: 06/28/2017] [Indexed: 11/28/2022]
Affiliation(s)
- D.‐W. Ryu
- Department of Neurology College of Medicine Catholic University of Korea Seoul Korea
| | - J.‐S. Kim
- Department of Neurology College of Medicine Catholic University of Korea Seoul Korea
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Maruyoshi H, Maruyoshi N, Hirosue M, Ikeda K, Shimamoto M. Clonazepam-associated Bradycardia in a Disabled Elderly Woman with Multiple Complications. Intern Med 2017; 56:2301-2305. [PMID: 28794360 PMCID: PMC5635303 DOI: 10.2169/internalmedicine.8234-16] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
We herein report an 87-year-old woman who was taking clonazepam at 1.5 mg/day. She was hospitalized with an old cerebral infarction complicated with symptomatic epilepsy, dementia, dyslipidemia, and chronic cholecystitis. Electrocardiogram revealed severe bradycardia at 31 beats/min. The bradycardia disappeared on day 3 after clonazepam withdrawal, although the serum clonazepam level had been within normal limits. She was diagnosed with clonazepam-associated bradycardia, which was likely related to the potential calcium channel-blocking properties of clonazepam. Because of age-related pharmacokinetic and pharmacodynamic changes, the adverse effects of clonazepam should be considered, especially in disabled elderly individuals with multiple comorbidities.
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Affiliation(s)
| | | | - Motone Hirosue
- Department of Internal Medicine, Shimamoto Hospital, Japan
| | - Komei Ikeda
- Department of Neurological Surgery, Shimamoto Hospital, Japan
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Tunc S, Brüggemann N, Baaske MK, Hartmann C, Grütz K, Westenberger A, Klein C, Münchau A, Bäumer T. Facial twitches in ADCY5 -associated disease - Myokymia or myoclonus? An electromyography study. Parkinsonism Relat Disord 2017; 40:73-75. [DOI: 10.1016/j.parkreldis.2017.04.013] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/18/2016] [Revised: 04/13/2017] [Accepted: 04/19/2017] [Indexed: 02/07/2023]
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Sawyer KN, Callaway CW, Wagner AK. Life After Death: Surviving Cardiac Arrest—an Overview of Epidemiology, Best Acute Care Practices, and Considerations for Rehabilitation Care. CURRENT PHYSICAL MEDICINE AND REHABILITATION REPORTS 2017. [DOI: 10.1007/s40141-017-0148-7] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
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Abstract
INTRODUCTION Myoclonic seizures are brief, involuntary muscular jerks arising from the central nervous system that can occur in different epilepsy syndromes, including idiopathic generalized epilepsies or the most severe group of epileptic encephalopathies. Valproate is commonly the first choice alone or in combination with some benzodiazepines or levetiracetam. However, more treatment options exist today as there is emerging evidence to support the efficacy of some newer antiepileptic drugs. In addition, of major importance remains avoidance of medications (e.g., carbamazepine, phenytoin) that may aggravate myoclonic seizures. This is an updated review on the available therapeutic options for treatment of myoclonic seizures. Areas covered: Key efficacy, tolerability and efficacy data are showed for different antiepileptic drugs with antimyoclonic effect, alone and/or in combination. Expert opinion: Pharmacological treatment of myoclonic seizures is based on clinical experience with little evidence from randomized clinical trials. Valproate, levetiracetam, and some benzodiazepines, are widely used. There is still insufficient evidence for the use of other antiseizure drugs, such as topiramate or zonisamide as monotherapy. Better understanding of pathophysiologic mechanisms of myoclonic epilepsies could yield great improvement in the treatment and quality of life of patients.
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Affiliation(s)
- Pasquale Striano
- a Pediatric Neurology and Muscular Diseases Unit, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health , University of Genoa, 'G. Gaslini' Institute , Genova , Italy
| | - Vincenzo Belcastro
- b Neurology Unit, Department of Medicine , Sant'Anna Hospital , Como , Italy
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Termsarasab P, Thammongkolchai T, Frucht SJ. Medical treatment of dystonia. JOURNAL OF CLINICAL MOVEMENT DISORDERS 2016; 3:19. [PMID: 28031858 PMCID: PMC5168853 DOI: 10.1186/s40734-016-0047-6] [Citation(s) in RCA: 40] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/14/2016] [Accepted: 11/08/2016] [Indexed: 11/25/2022]
Abstract
Therapeutic strategies in dystonia have evolved considerably in the past few decades. Three major treatment modalities include oral medications, botulinum toxin injections and surgical therapies, particularly deep brain stimulation. Although there has been a tremendous interest in the later two modalities, there are relatively few recent reviews of oral treatment. We review the medical treatment of dystonia, focusing on three major neurotransmitter systems: cholinergic, GABAergic and dopaminergic. We also provide a practical guide to medication selection, therapeutic strategy and unmet needs.
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Affiliation(s)
- Pichet Termsarasab
- Movement Disorder Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, USA
| | | | - Steven J. Frucht
- Movement Disorder Division, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, USA
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Löscher W, Gillard M, Sands ZA, Kaminski RM, Klitgaard H. Synaptic Vesicle Glycoprotein 2A Ligands in the Treatment of Epilepsy and Beyond. CNS Drugs 2016; 30:1055-1077. [PMID: 27752944 PMCID: PMC5078162 DOI: 10.1007/s40263-016-0384-x] [Citation(s) in RCA: 120] [Impact Index Per Article: 13.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
The synaptic vesicle glycoprotein SV2A belongs to the major facilitator superfamily (MFS) of transporters and is an integral constituent of synaptic vesicle membranes. SV2A has been demonstrated to be involved in vesicle trafficking and exocytosis, processes crucial for neurotransmission. The anti-seizure drug levetiracetam was the first ligand to target SV2A and displays a broad spectrum of anti-seizure activity in various preclinical models. Several lines of preclinical and clinical evidence, including genetics and protein expression changes, support an important role of SV2A in epilepsy pathophysiology. While the functional consequences of SV2A ligand binding are not fully elucidated, studies suggest that subsequent SV2A conformational changes may contribute to seizure protection. Conversely, the recently discovered negative SV2A modulators, such as UCB0255, counteract the anti-seizure effect of levetiracetam and display procognitive properties in preclinical models. More broadly, dysfunction of SV2A may also be involved in Alzheimer's disease and other types of cognitive impairment, suggesting potential novel therapies for levetiracetam and its congeners. Furthermore, emerging data indicate that there may be important roles for two other SV2 isoforms (SV2B and SV2C) in the pathogenesis of epilepsy, as well as other neurodegenerative diseases. Utilization of recently developed SV2A positron emission tomography ligands will strengthen and reinforce the pharmacological evidence that SV2A is a druggable target, and will provide a better understanding of its role in epilepsy and other neurological diseases, aiding in further defining the full therapeutic potential of SV2A modulation.
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Affiliation(s)
- Wolfgang Löscher
- Department of Pharmacology, Toxicology and Pharmacy, University of Veterinary Medicine Hannover, Bünteweg 17, 30559, Hannover, Germany.
- Center for Systems Neuroscience, Hannover, Germany.
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Koens LH, Kuiper A, Coenen MA, Elting JWJ, de Vries JJ, Engelen M, Koelman JHTM, van Spronsen FJ, Spikman JM, de Koning TJ, Tijssen MAJ. Ataxia, dystonia and myoclonus in adult patients with Niemann-Pick type C. Orphanet J Rare Dis 2016; 11:121. [PMID: 27581084 PMCID: PMC5007743 DOI: 10.1186/s13023-016-0502-3] [Citation(s) in RCA: 28] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2016] [Accepted: 08/12/2016] [Indexed: 11/14/2022] Open
Abstract
Background Niemann-Pick type C (NP-C) is a rare autosomal recessive progressive neurodegenerative disorder caused by mutations in the NP-C 1 or 2 gene. Besides visceral symptoms, presentation in adolescent and adult onset variants is often with neurological symptoms. The most frequently reported presenting symptoms of NP-C in adulthood are psychiatric symptoms (38 %), cognitive decline (23 %) and ataxia (20 %). Myoclonus can be present, but its value in early diagnosis and the evolving clinical phenotype in NP-C is unclear. In this paper we present eight Dutch cases of NP-C of whom five with myoclonus. Methods Eight patients with genetically confirmed NP-C were recruited from two Dutch University Medical Centers. A structured interview and neuropsychological tests (for working and verbal memory, attention and emotion recognition) were performed. Movement disorders were assessed using a standardized video protocol. Quality of life was evaluated by questionnaires (Rand-36, SIP-68, HAQ). In four of the five patients with myoclonic jerks simultaneous EEG with EMG was performed. Results A movement disorder was the initial neurological symptom in six patients: three with myoclonus and three with ataxia. Two others presented with psychosis. Four experienced cognitive deficits early in the course of the disease. Patients showed cognitive deficits in all investigated domains. Five patients showed myoclonic jerks, including negative myoclonus. In all registered patients EEG-EMG coherence analysis and/or back-averaging proved a cortical origin of myoclonus. Patients with more severe movement disorders experienced significantly more physical disabilities. Conclusions Presenting neurological symptoms of NP-C include movement disorders, psychosis and cognitive deficits. At current neurological examination movement disorders were seen in all patients. The incidence of myoclonus in our cohort was considerably higher (63 %) than in previous publications and it was the presenting symptom in 38 %. A cortical origin of myoclonus was demonstrated. Our data suggest that myoclonus may be overlooked in patients with NP-C. All patients scored significantly lower on physical domains of HRQoL. Symptomatic treatment of movement disorders may improve physical functioning and subsequently HRQoL.
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Affiliation(s)
- L H Koens
- Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - A Kuiper
- Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - M A Coenen
- Department of Clinical Neuropsychology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - J W J Elting
- Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - J J de Vries
- Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - M Engelen
- Department of Neurology, University of Amsterdam, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
| | - J H T M Koelman
- Department of Neurology, University of Amsterdam, Academic Medical Center, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands
| | - F J van Spronsen
- Division of Metabolic Diseases, University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - J M Spikman
- Department of Clinical Neuropsychology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.,Department of Clinical and Developmental Neuropsychology, University of Groningen, Faculty of Behavioral and Social Sciences, Grote Kruisstraat 2/1, 9712 TS, Groningen, The Netherlands
| | - T J de Koning
- Division of Metabolic Diseases, University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.,Department of Genetics, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands
| | - M A J Tijssen
- Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands.
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