|
1
|
Mohamed AA, Faragalla S, Khan A, Flynn G, Rainone G, Johansen PM, Lucke-Wold B. Neurosurgical and pharmacological management of dystonia. World J Psychiatry 2024; 14:624-634. [PMID: 38808085 PMCID: PMC11129150 DOI: 10.5498/wjp.v14.i5.624] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/04/2024] [Revised: 04/20/2024] [Accepted: 04/26/2024] [Indexed: 05/16/2024] Open
Abstract
Dystonia characterizes a group of neurological movement disorders characterized by abnormal muscle movements, often with repetitive or sustained contraction resulting in abnormal posturing. Different types of dystonia present based on the affected body regions and play a prominent role in determining the potential efficacy of a given intervention. For most patients afflicted with these disorders, an exact cause is rarely identified, so treatment mainly focuses on symptomatic alleviation. Pharmacological agents, such as oral anticholinergic administration and botulinum toxin injection, play a major role in the initial treatment of patients. In more severe and/or refractory cases, focal areas for neurosurgical intervention are identified and targeted to improve quality of life. Deep brain stimulation (DBS) targets these anatomical locations to minimize dystonia symptoms. Surgical ablation procedures and peripheral denervation surgeries also offer potential treatment to patients who do not respond to DBS. These management options grant providers and patients the ability to weigh the benefits and risks for each individual patient profile. This review article explores these pharmacological and neurosurgical management modalities for dystonia, providing a comprehensive assessment of each of their benefits and shortcomings.
Collapse
Affiliation(s)
- Ali Ahmed Mohamed
- Charles E Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, United States
| | - Steven Faragalla
- Charles E Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, United States
| | - Asad Khan
- Charles E Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, United States
| | - Garrett Flynn
- Charles E Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, United States
| | - Gersham Rainone
- Department of Neurosurgery and Brain Repair, University of South Florida, Tampa, FL 33606, United States
| | - Phillip Mitchell Johansen
- Department of Neurosurgery and Brain Repair, University of South Florida, Tampa, FL 33606, United States
| | - Brandon Lucke-Wold
- Department of Neurosurgery, University of Florida, Gainesville, FL 32611, United States
| |
Collapse
|
|
2
|
Abstract
Introduction: Symptoms of cervical dystonia (CD) can vary in severity and cause significant pain. OnabotulinumtoxinA is an approved treatment for CD. This study assessed health-related quality of life (HRQoL) in patients with CD who received multiple onabotulinumtoxinA treatments. Methods: This prospective, observational standard-of-care study was conducted at multiple neurology centers in Québec, Canada. Patients reported the health impact of CD using the Cervical Dystonia Impact Profile (CDIP)-58, before and after up to eight onabotulinumtoxinA treatments. Other measures included the Cervical Dystonia Severity Rating Scale by physician, employment status using the Work Productivity Questionnaire and pain using the Pain Numeric Rating Scale (PNRS). Adverse events (AEs) were recorded. Results: Sixty-two patients were enrolled (safety population, n = 61; modified efficacy population, n = 58). Participants were mostly females who were employed; most (79.3%) had torticollis. In all, 21/62 patients (33.9%) discontinued the study. At the final visit, there was a statistically significant (p < 0.001) improvement in all eight CDIP-58 subscales, particularly head and neck symptoms (−31.0) and psychosocial functioning (−28.2). Employment increased from baseline (55%) to the end of the study (64%), and there was improvement in work productivity. There was a significant (p < 0.0001) reduction in pain measured by the PNRS, from −0.5 post-treatment 1 to −2.4 at end of study. AEs (neck pain, muscular weakness, dysphagia, nausea) were consistent with onabotulinumtoxinA use. Conclusion: These real-world data indicate that after repeated, long-term use, onabotulinumtoxinA continues to be a safe and effective treatment for CD, improving HRQoL and work productivity.
Collapse
|
|
3
|
Charous SJ, Comella C. Dystonias of the head and neck: An overview. EAR, NOSE & THROAT JOURNAL 2011; 90:E4. [PMID: 21328221 DOI: 10.1177/014556131109000209] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Dystonias are a group of disorders characterized by muscle contractions that can produce twisting and repetitive movements or abnormal postures. Dystonias of the head and neck region, except for spasmodic dysphonia, are rarely described in the otolaryngology literature. Ironically, it is the otolaryngologic surgeon's knowledge of anatomy and physiology of the head and neck that can be of greatest benefit for patients suffering from these disorders. Medical and surgical treatment options are available in treating this disorder. This article is intended to serve as an introduction and overview of dystonias for the otolaryngologist-head and neck surgeon.
Collapse
Affiliation(s)
- Steven J Charous
- Department of Otolaryngology, Loyola University Medical Center, 3633 West Lake Ave., Suite 300, Glenview IL 60026, USA.
| | | |
Collapse
|
|
4
|
Cervical Dystonia. Pain Manag 2011. [DOI: 10.1016/b978-1-4377-0721-2.00061-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
|
|
5
|
Deon LL, Gaebler-Spira D. Assessment and treatment of movement disorders in children with cerebral palsy. Orthop Clin North Am 2010; 41:507-17. [PMID: 20868881 DOI: 10.1016/j.ocl.2010.06.001] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Cerebral palsy is the most common motor disability in childhood. Orthopedic care depends on the appreciation and the identification of muscle tone abnormalities and how they affect growth and development of the child. Abnormal muscle tone is a common diagnostic feature of cerebral palsy and can include hypotonia or hypertonia. Hypertonia is the most frequent tone abnormality in children with cerebral palsy. This article reviews hypertonia and provides information on discriminating between spasticity, dystonia, and rigidity. Medication and neurosurgical options for the management of hypertonia are presented and compared.
Collapse
Affiliation(s)
- Laura L Deon
- Pediatric Physical Medicine and Rehabilitation, Rehabilitation Institute of Chicago at Northwestern University, Chicago, IL 60611, USA
| | | |
Collapse
|
|
6
|
Felicio AC, Godeiro C, Moriyama TS, Laureano MR, Felix EPV, Borges V, Silva SMA, Ferraz HB. Speech-induced lingual dystonia. ARQUIVOS DE NEURO-PSIQUIATRIA 2010; 68:653-5. [PMID: 20730328 DOI: 10.1590/s0004-282x2010000400034] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
|
|
7
|
Haselden K, Powell T, Drinnan M, Carding P. Comparing Health Locus of Control in Patients with Spasmodic Dysphonia, Functional Dysphonia and Nonlaryngeal Dystonia. J Voice 2009; 23:699-706. [DOI: 10.1016/j.jvoice.2008.03.011] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2007] [Accepted: 03/31/2008] [Indexed: 10/21/2022]
|
|
8
|
Stacy M. Epidemiology, clinical presentation, and diagnosis of cervical dystonia. Neurol Clin 2008; 26 Suppl 1:23-42. [PMID: 18603166 DOI: 10.1016/s0733-8619(08)80003-5] [Citation(s) in RCA: 40] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Affiliation(s)
- Mark Stacy
- Duke University Medical Center, 932 Morreene Road, Durham, NC 27705, USA.
| |
Collapse
|
|
9
|
|
|
10
|
Dashtipour K, Barahimi M, Karkar S. Cervical Dystonia. J Pharm Pract 2007. [DOI: 10.1177/0897190007311452] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Cervical dystonia, which is the most common form of focal dystonia, presents with sustained neck spasms, abnormal head posture, head tremor, and pain. One of the interesting and unique features of cervical dystonia is the geste antagoniste. There is not a well-described pathophysiology for cervical dystonia, but several hypotheses report involvement at the central and peripheral level. Treatment options include: oral medical therapy, botulinum toxin injection, and surgery. Oral medical therapy has limited efficacy in control of the symptoms of cervical dystonia. Two types of botulinum toxin, types A and B, are being used for treatment of cervical dystonia, with equivalent benefit. Surgery is an option when other treatments fail or become ineffective. The surgical procedures are brain lesioning, brain stimulation, and peripheral surgical intervention. Several trials are currently ongoing in the United States and Europe to evaluate the efficacy of deep brain surgery in cervical dystonia.
Collapse
Affiliation(s)
- Khashayar Dashtipour
- Department of Neurology and School of Medicine, Loma Linda University, Loma Linda, California,
| | - Mandana Barahimi
- Department of Family Practice, Northridge Hospital Medical Center, Northridge, California
| | - Samia Karkar
- School of Pharmacy, Loma Linda University, Loma Linda, California
| |
Collapse
|
|
11
|
Childers MK, Markert C. Cervical Dystonia. Pain Manag 2007. [DOI: 10.1016/b978-0-7216-0334-6.50060-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
|
|
12
|
Abstract
Animal models indicate that the abnormal movements of focal dystonia result from disordered sensorimotor integration. Sensorimotor integration involves a comparison of sensory information resulting from a movement with the sensory information expected from the movement. Unanticipated sensory signals identified by sensorimotor processing serve as signals to modify the ongoing movement or the planning for subsequent movements. Normally, this process is an effective mechanism to modify neural commands for ongoing movement or for movement planning. Animal models of the focal dystonias spasmodic torticollis, writer's cramp, and benign essential blepharospasm reveal different dysfunctions of sensorimotor integration through which dystonia can arise. Animal models of spasmodic torticollis demonstrate that modifications in a variety of regions are capable of creating abnormal head postures. These data indicate that disruption of neural signals in one structure may mutate the activity pattern of other elements of the neural circuits for movement. The animal model of writer's cramp demonstrates the importance of abnormal sensory processing in generating dystonic movements. Animal models of blepharospasm illustrate how disrupting motor adaptation can produce dystonia. Together, these models show mechanisms by which disruptions in sensorimotor integration can create dystonic movements.
Collapse
Affiliation(s)
- Craig Evinger
- Departments of Neurobiology & Behavior and Ophthalmology, SUNY Stony Brook, New York 11794-5230, USA.
| |
Collapse
|
|
13
|
Seet RCS, Lim ECH. Symptomatic segmental dystonia from a cavernous angioma in the centrum semiovale. Parkinsonism Relat Disord 2005; 11:65-7. [PMID: 15619465 DOI: 10.1016/j.parkreldis.2004.08.003] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2004] [Revised: 08/10/2004] [Accepted: 08/10/2004] [Indexed: 10/26/2022]
Abstract
We describe a 68 year-old Chinese man who presented with segmental dystonia involving the neck and right upper limb, which was exacerbated with action. EEG and MRI of the cervical spine were unremarkable, but MRI of the brain revealed a small cavernous angioma measuring one centimeter in diameter in the left centrum semiovale. The dystonia was ameliorated but not abolished with trihexyphenidyl. Arteriovenous malformations have been described to cause movement disorders, but dystonia has not previously been reported in association with cavernous angiomas. A search of the literature revealed one report of a patient with chorea arising from a cavernous angioma. This interesting case illustrates how an uncomplicated cavernous angioma can rarely cause segmental dystonia and highlights the importance of neuroimaging in patients with late-onset movement disorders.
Collapse
Affiliation(s)
- Raymond C S Seet
- Division of Neurology, National University Hospital, 5 Lower Kent Ridge Road, Singapore, Singapore 119074
| | | |
Collapse
|
|
14
|
Abstract
Dystonia is a state of continuous contraction of groups of agonist and antagonist muscles resulting in a sustained abnormal posture. Dopa-responsive dystonia was first described in 1976 by Segawa. Patients typically have diurnal variation of their symptoms with worsening at the end of the day and a dramatic response to low-dose L-dopa. This report presents five consecutive children with dopa-responsive dystonia who were misdiagnosed initially as spastic diplegic cerebral palsy, intractable epilepsy, hereditary spastic paraplegia, or a neurodegenerative disorder. There were two males and three females aged 3-13 years (mean 8.6 years). They were monitored for up to 2 years (mean 14.8 months). One had focal, one axial, one segmental, and two generalized dystonia. The dystonia was paroxysmal in two (tiptoe walking and opisthotonus), and all had a progressive course. All children responded dramatically to L-dopa (mean 200 mg/day), including three who were wheelchair-bound for several years. The difficulties in early diagnosis, variability of clinical presentation, and dramatic response to L-dopa will be illustrated. To conclude, dopa-responsive dystonia should be considered in any child who presents with paroxysmal or progressive hypertonia of unknown etiology, because it responds so dramatically to L-dopa.
Collapse
Affiliation(s)
- Mohammed M S Jan
- Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia
| |
Collapse
|
|
15
|
Herskowitz A, Herskowitz B. Treatment of Neck and Shoulder Pain with Botulinum Neurotoxins. Pain Pract 2004; 4 Suppl 1:S27-37. [PMID: 17129273 DOI: 10.1111/j.1533-2500.2004.04012.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
|
|
16
|
Abstract
Spasticity or dystonia of the upper limb in children with CP commonly is seen as part of a more involved clinical picture. Each can cause functional problems in and can interfere with the quality of life of children with CP. Pharmacologic manipulation of the spasticity and dystonia requires a patient and ordered approach. Treatment often is performed using an open trial method, with medications introduced slowly but often being limited in their usefulness by significant side effects. Despite multiple medications being available for spasticity and dystonia management, few of the oral treatments have been assessed systematically in children with CP. Specific evaluation of the use of enteral medications in upper limb management is even rarer.
Collapse
Affiliation(s)
- Stephen O'Flaherty
- Department of Rehabilitation, Children's Hospital at Westmead, Locked Bag 4001, Westmead, Sydney NSW 2145, Australia.
| | | |
Collapse
|