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Review Article
Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data
Weibin He, Chenhui Li, Hongjuan Dong, Lingmin Shao, Bo Yin, Dianyou Li, Liguo Ye, Ping Hu, Chencheng Zhang, Wei Yi
Received January 7, 2022  Accepted May 27, 2022  Published online July 26, 2022  
DOI: https://doi.org/10.14802/jmd.22003    [Epub ahead of print]
  • 218 View
  • 33 Download
AbstractAbstract PDFSupplementary Material
A significant proportion of patients with chorea-acanthocytosis (ChAc) fail to respond to standard therapies. Recent evidence suggests that globus pallidus internus (GPi) deep brain stimulation (DBS) is a promising treatment option; however, reports are few and limited by sample sizes. We conducted a systematic literature review to evaluate the clinical outcome of GPi-DBS for ChAc. PubMed, Embase, and Cochrane Library databases were searched for relevant articles published before August 2021. The improvement of multiple motor and nonmotor symptoms was qualitatively presented. Improvements in the Unified Huntington’s Disease Rating Scale motor score (UHDRS-MS) were also analyzed during different follow-up periods. A multivariate linear regression analysis was conducted to identify potential predictors of clinical outcomes. Twenty articles, including 27 patients, were eligible. Ninety-six percent of patients with oromandibular dystonia reported significant improvement. GPi-DBS significantly improved the UHDRS-motor score at < 6 months (p < 0.001) and ≥ 6 months (p < 0.001). The UHDRS-motor score improvement rate was over 25% in 75% (15/20 cases) of patients at long-term follow-up (≥ 6 months). The multiple linear regression analysis showed that sex, age at onset, course of disease, and preoperative movement score had no linear relationship with motor improvement at long-term follow-up (p > 0.05). GPi-DBS is an effective and safe treatment in most patients with ChAc, but no reliable predictor of efficacy has been found. Oromandibular dystonia-dominant patients might be the best candidates for GPi-DBS.
Brief communication
Movement Disorders Resulting From Bilateral Basal Ganglia Lesions in End-Stage Kidney Disease: A Systematic Review
Kah Hui Yap, Nurul Husna Baharudin, Abdul Halim Abdul Gafor, Rabani Remli, Shen-Yang Lim, Wan Asyraf Wan Zaidi, Shahrul Azmin, Shahizon Azura Mohamed Mukari, Raihanah Abdul Khalid, Norlinah Mohamed Ibrahim
Received December 15, 2021  Accepted February 19, 2022  Published online May 26, 2022  
DOI: https://doi.org/10.14802/jmd.21185    [Epub ahead of print]
  • 602 View
  • 30 Download
AbstractAbstract PDFSupplementary Material
Objective
The basal ganglia (BG) are susceptible to fluctuations in blood urea levels, sometimes resulting in movement disorders. We described patients with end-stage kidney disease (ESKD) presenting with movement disorders associated with bilateral BG lesions on imaging.
Methods
We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid).
Results
Of the 72 patients identified, 55 (76.4%) were on regular dialysis. Parkinsonism was the most common movement disorder (n = 39; 54.2%), followed by chorea (n = 24; 33.3%). Diabetes mellitus (n = 51; 70.8%) and hypertension (n = 16; 22.2%) were the most common risk factors. Forty-three (59.7%) were of Asian ethnicity. Complete clinical resolution was reported in 17 (30.9%) patients, while 38 (69.1%) had incomplete clinical resolution with relapse. Complete radiological resolution occurred in 14 (34.1%) patients.
Conclusion
Movement disorders associated with BG lesions should be recognized as a rare and potentially reversible metabolic movement disorder in patients with ESKD.
Case Report
Treatment of Hemichoreoathetosis with Arrhythmic Proximal Tremor after Stroke: The Role of Zona Incerta as a Target for Deep Brain Stimulation
Andrei Koerbel, Augusto Radünz do Amaral, Helena Bedatti Zeh, Eduardo Wollmann, Renata Fabiola Heil Koerbel, Carla Moro, Alexandre Luiz Longo
J Mov Disord. 2019;12(1):47-51.   Published online January 30, 2019
DOI: https://doi.org/10.14802/jmd.18032
  • 4,839 View
  • 100 Download
  • 2 Citations
AbstractAbstract PDFSupplementary Material
Deep brain stimulation (DBS) of the zona incerta has shown promising results in the reduction of medically refractory movement disorders. However, evidence supporting its efficacy in movement disorders secondary to hemorrhagic stroke or hemichoreoathetosis is limited. We describe a 48-year-old man who developed progressive hemichoreoathetosis with an arrhythmic, proximal tremor in his right arm following a thalamic hemorrhagic stroke. Pharmacological treatment was carried out with no change in the Abnormal Involuntary Movement Scale (AIMS) score after 4 weeks (14). After six sessions of botulinum toxin treatment, a subtle improvement in the AIMS score (13) was registered, but no clinical improvement was noted. The arrhythmic proximal movements were significantly improved after DBS of the zona incerta with a major decrease in the patient’s AIMS score (8). The response to DBS occurring after the failure of pharmacological and botulinum toxin treatments suggests that zona incerta DBS may be an alternative for postthalamic hemorrhage movement disorders.
Review Articles
Untangling the Thorns: Advances in the Neuroacanthocytosis Syndromes
Ruth H. Walker
J Mov Disord. 2015;8(2):41-54.   Published online May 31, 2015
DOI: https://doi.org/10.14802/jmd.15009
  • 24,653 View
  • 313 Download
  • 29 Citations
AbstractAbstract PDF
There have been significant advances in neuroacanthocytosis (NA) syndromes in the past 20 years, however, confusion still exists regarding the precise nature of these disorders and the correct nomenclature. This article seeks to clarify these issues and to summarise the recent literature in the field. The four key NA syndromes are described here–chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, and pantothenate kinase- associated neurodegeneration. In the first two, acanthocytosis is a frequent, although not invariable, finding; in the second two, it occurs in approximately 10% of patients. Degeneration affecting the basal ganglia is the key neuropathologic finding, thus the clinical presentations can be remarkably similar. The characteristic phenotype comprises a variety of movement disorders, including chorea, dystonia, and parkinsonism, and also psychiatric and cognitive symptoms attributable to basal ganglia dysfunction. The age of onset, inheritance patterns, and ethnic background differ in each condition, providing diagnostic clues. Other investigations, including routine blood testing and neuroimaging can be informative. Genetic diagnosis, if available, provides a definitive diagnosis, and is important for genetic counseling, and hopefully molecular therapies in the future. In this article I provide a historical perspective on each NA syndrome. The first 3 disorders, chorea-acanthocytosis, McLeod syndrome, Huntington’s disease-like 2, are discussed in detail, with a comprehensive review of the literature to date for each, while pantothenate kinase-associated neurodegeneration is presented in summary, as this disorder has recently been reviewed in this journal. Therapy for all of these diseases is, at present, purely symptomatic.
Cell Therapy Strategies vs. Paracrine Effect in Huntington’s Disease
Wooseok Im, Manho Kim
J Mov Disord. 2014;7(1):1-6.   Published online April 30, 2014
DOI: https://doi.org/10.14802/jmd.14001
  • 12,329 View
  • 86 Download
  • 5 Citations
AbstractAbstract PDF
Huntington’s disease (HD) is a genetic neurodegenerative disorder. The most common symptom of HD is abnormal involuntary writhing movements, called chorea. Antipsychotics and tetrabenazine are used to alleviate the signs and symptoms of HD. Stem cells have been investigated for use in neurodegenerative disorders to develop cell therapy strategies. Recent evidence indicates that the beneficial effects of stem cell therapies are actually mediated by secretory molecules, as well as cell replacement. Although stem cell studies show that cell transplantation provides cellular improvement around lesions in in vivo models, further work is required to elucidate some issues before the clinical application of stem cells. These issues include the precise mechanism of action, delivery method, toxicity and safety. With a focus on HD, this review summarizes cell therapy strategies and the paracrine effect of stem cells.
Case Reports
Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
J Mov Disord. 2013;6(1):17-20.
DOI: https://doi.org/10.14802/jmd.13004
  • 14,546 View
  • 88 Download
  • 9 Citations
AbstractAbstract PDF

Involuntary movement associated with deep watershed ischemic lesions has been rarely reported. A 67-year-old woman presented with acute hemichorea on the left side. Magnetic resonance imaging showed acute infarcts in the anterior border zone. On perfusion studies, impaired cerebral blood flow was observed on the subcortical region sparing the basal ganglia. Cerebral angiogram confirmed severe stenosis in the right internal carotid artery. Her hemichorea gradually improved along with normalization of perfusion after carotid artery stenting with angioplasty. We suggest that impaired cerebral blood flow in critical watershed territories may be an important contributing factor in hemichorea associated with carotid occlusive disease.

A Case of Isolated Middle Cerebral Artery Stenosis with Hemichorea and Moyamoya Pattern Collateralization
Seok Jong Chung, Hyung Seok Lee, Han Soo Yoo, Kyung Min Kim, Ki Jeong Lee, Jong-Soo Kim, Jae-Wook Lee, Jong Hun Kim, Jeong Hee Cho, Gyu Sik Kim, Jun Hong Lee, Sun-Ah Choi
J Mov Disord. 2013;6(1):13-16.
DOI: https://doi.org/10.14802/jmd.13003
  • 9,856 View
  • 90 Download
  • 3 Citations
AbstractAbstract PDF

Isolated middle cerebral artery (MCA) stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA) to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.

Hemichorea-Hemiballism with a Diabetic Patient
Yoo Hwan Kim, Ju Yeon Kim, Hung Youl Seok, Seong-Beom Koh
J Mov Disord. 2010;3(1):20-21.
DOI: https://doi.org/10.14802/jmd.10006
  • 8,960 View
  • 53 Download
  • 2 Citations
AbstractAbstract PDF

Chorea and ballism are movement disorders that result from a variety of conditions. They are an uncommon manifestation of diabetes mellitus. We report a 52-year-old diabetic man who presented with acute onset chorea-ballism with a putaminal high-signal-intensity lesion on T1-weighted magnetic resonance imaging (MRI).

Chorea in the Both Lower Limbs Associated with Nonketotic Hyperglycemia
Young-Hee Sung, Ki-Hyung Park, Yeung-Bae Lee, Hyeon-Mi Park, Dong-Jin Shin
J Mov Disord. 2009;2(2):98-100.
DOI: https://doi.org/10.14802/jmd.09027
  • 17,089 View
  • 77 Download
  • 4 Citations
AbstractAbstract PDF

Hemichorea-hemiballism (HC-HB) is a complication of non-ketotic hyperglycemia (NKH); in NKH patients, the frequency of occurrence of HC-HB is greater than that of bilateral chorea. We report the case of a hyperglycemic patient who showed chorea in both the lower limbs. Magnetic resonance imaging (MRI) of the brain revealed high signal intensity on T1-weighted images of the bilateral dorsolateral putamen. The abnormal involuntary movements disappeared after oral administration of haloperidol. Our case report that chorea associated with NKH is correlated with the topography of the basal ganglia.

A Case of Vascular Hemichorea Responding to Topiramate
Jee-Ae Kim, San Jung, Min-Ju Kim, Seok-Beom Kwon, Sung-Hee Hwang, Ki-Han Kwon
J Mov Disord. 2009;2(2):80-81.
DOI: https://doi.org/10.14802/jmd.09021
  • 15,457 View
  • 43 Download
  • 3 Citations
AbstractAbstract PDF

Although vascular chorea often comes into remission spontaneously, a few patients may remain with persistent movement disorder. Most movements respond well to neuroleptics as well as other antidopaminergic drugs, but some patients show poor responses to those neuroleptics. Topiramate is a widely used of broad-spectrum anticonvulsant possessing a complex mechanism of action. It has been proven to enhance gamma-aminobutyrate acid activity and to be effective in the control of other movement disorders. We describe a 63-year-old woman with intractable vascular hemichorea which was controlled with anti-convulsant, topiramate.

Paroxysmal Chorea as a Relapse of Myelopathy in a Patient with Neuromyelitis Optica
Sang-Soo Lee, Ho-Sung Han, Dong-Ick Shin
J Mov Disord. 2009;2(2):78-79.
DOI: https://doi.org/10.14802/jmd.09020
  • 18,620 View
  • 74 Download
AbstractAbstract PDF

Movement disorders secondary to intrinsic spinal cord disease are rare. Paroxysmal chorea has not yet been reported in the neuromyelitis optica (NMO). We report a 43-year-old woman with relapsing-remitting cervical myelopathy who developed paroxysmal chorea during clinical exacerbation of NMO. MRI scan of the cervical spine revealed a long segmental enhancing lesion, but brain MRI did not show any responsible abnormalities. Acute exacerbation of recurrent myelopathy in NMO may be associated with transient movement disorder.

Review Article
Secondary Dystonia-Clinical Clues and Syndromic Associations
Susanne A Schneider, Kailash P Bhatia
J Mov Disord. 2009;2(2):58-63.
DOI: https://doi.org/10.14802/jmd.09016
  • 16,276 View
  • 367 Download
  • 9 Citations
AbstractAbstract PDF
Background:

Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.

Purpose:

The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.

Methods:

We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.

Results:

Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.

Conclusions:

When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.

Case Reports
Generalized Chorea Induced by an Unilateral Anterior Cerebral Artery Territorial Infarction
Jae Hong Chang, Woo-Keun Seo, Moon-Ho Park, Jong-Mun Lee, Do-Young Kwon, Seong-Beom Koh
J Mov Disord. 2009;2(1):37-39.
DOI: https://doi.org/10.14802/jmd.09009
  • 6,838 View
  • 81 Download
  • 2 Citations
AbstractAbstract PDF

Generalized chorea caused by unilateral cerebral infarction has rarely been reported. A 58-year-old woman presented involuntary movement in her all extremities after acute cerebral infarction on her right anterior cerebral artery territory. The involuntary movements were diagnosed as generalized chorea. We didn’t find any cause of generalized chorea except the acute cerebral infarction. Here, we described the case of generalized chorea after unilateral cerebral infarction discussing the possible mechanisms.

A Case of Chorea as the Initial Manifestation of SLE Triggered by Estrogen
Su-Hyun Kim, Dong-Jin Shin, Hyeon-Mi Park, Yeong-Bae Lee, Kee-Hyung Park, Young-Hee Sung
J Mov Disord. 2008;1(2):86-89.
DOI: https://doi.org/10.14802/jmd.08016
  • 8,019 View
  • 46 Download
AbstractAbstract PDF

Neurological complications of systemic lupus erythematosus (SLE) are relatively common, but chorea as the initial manifestation of SLE unmasked by exogenous estrogen is very rare. A-46-year old right handed woman presented with generalized chorea since 2 weeks ago. Her medical records revealed that the chorea had appeared within one month after estrogen medication. The laboratory test revealed positive antinuclear antibody (ANA), positive anti-dsDNA and positive anti-histone antibody. After discontinuation of estrogen, her choreic movement was not diminished. We report a case of newly diagnosed SLE attribute to chorea which triggered by estrogen.

Chorea as an Initial Manifestation of Polycythemia Vera
Ji Eun Lee, Hae-Won Shin, Young H. Sohn
J Mov Disord. 2008;1(2):82-85.
DOI: https://doi.org/10.14802/jmd.08015
  • 9,693 View
  • 122 Download
AbstractAbstract PDF

Chorea is a rare complication of polycythemia vera (PV). We report a 58-year-old woman with acute onset chorea without structural lesion in the basal ganglia. The physical and laboratory findings were compatible with the diagnosis of PV. After repeated phlebotomies her chorea was improved. PV should be considered as one of the possible etiologies of chorea, as early diagnosis is important to lead to the effective treatment and prevention of complications.


JMD : Journal of Movement Disorders