Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sydenham's chorea (SC) occurs weeks or months after Group A streptococcal infection, and is characterized by involuntary, purposeless movements of the limbs, in addition to behavioural alteration. There is a body of evidence which suggests that SC is an immune-mediated brain disorder with regional localization to the basal ganglia. Recent reports have suggested that the spectrum of post-streptococcal CNS disease is broader than chorea alone, and includes other hyperkinetic movement disorders (tics, dystonia and myoclonus). In addition, there are high rates of behavioural sequelae, particularly emotional disorders such as obsessive-compulsive disorder, anxiety and depression. These findings have lead to the hypothesis that similar immune-mediated basal ganglia processes may be operating in common neuropsychiatric disease such as tic disorders, Tourette syndrome and obsessive-compulsive disorder. This review analyses the historical aspects of post-streptococcal CNS disease, and the recent immunological studies which have addressed the hypothesis that common neuropsychiatric disorders may be secondary to basal ganglia autoimmunity.
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PMID:Autoimmunity and the basal ganglia: new insights into old diseases. 1261 82

We report an adult patient presenting with choreiform movements 4 days after a large intravenous dose of cocaine. These movements were transitory and they normalized a week after admission. We believe this to be the first video case of acute chorea secondary to cocaine--a phenomenon popularly known as "crack dancing. " Cocaine abuse is associated with a wide range of movement disorders, including dystonia and exacerbation of Tourette's syndrome, multifocal tics, opsoclonus-myoclonus, choreiform movements, and stereotyped behavior known as "punding." Transient choreiform movements with a typical duration of 2 to 6 days are recognized by cocaine abusers themselves as crack dancing, but are infrequently reported. We present a video report of a patient with cocaine dependency and choreiform movements that normalized within a week of admission.
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PMID:Crack dancing in the United Kingdom: apropos a video case presentation. 1741 1

Movement disorders have been known to be associated with a variety of autoimmune diseases, including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcus, systemic lupus erythematosus, antiphospholipid syndrome, gluten sensitivity, paraneoplastic and autoimmune encephalopathies. Tremors, dystonia, chorea, ballism, myoclonus, parkinsonism, and ataxia may be the initial and even the only presentation of these autoimmune diseases. Although antibodies directed against various cellular components of the central nervous system have been implicated, the pathogenic mechanisms of these autoimmune movement disorders have not yet been fully elucidated. Clinical recognition of these autoimmune movement disorders is critically important as many improve with immunotherapy or dietary modifications, particularly when diagnosed early. We discuss here the clinical features, pathogenic mechanisms, and treatments of movement disorders associated with autoimmune diseases, based on our own experience and on a systematic review of the literature.
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PMID:Movement disorders in autoimmune diseases. 2255 4

Chorea is an involuntary movement disorder characterised by flowing and rhythmic in nature. Hyperkinetic movement disorders such as myoclonus may be mistaken for chorea. Pathogenes of chorea is complex and results from dysfunction of network between motor nucleus of thalamus and subcortical nuclei including globus pallidus interna. There are genetic and non genetic causes of chorea. Huntington's disease is most common genetic cause of chorea. Clinical manifestations of Huntington's disease are mainly neurological and psychiatric. Recently non neurological clinical manifestations of this disease have been described. Genetic test for Huntington's disease is available which may be done for diagnosis and detection of family members at risk of developing disease. Other genetic causes of chorea are neuroacanthocytosis and Wilson's disease. Treatment of genetic causes of chore is usually symptomatic with exception of Wilson's disease. Sydenham's chorea is a neurological manifestation of acute rheumatic fever and most important cause of chorea seen in paediatric population. Treatment includes penicillin prophylaxis and drugs such as sodium valproate and carbamazepine. Diagnosis of chorea is mainly clinical. Family history is very important in diagnosis of genetic causes of chorea. In other patients a detailed work up is required before a final diagnosis is made. Hematological and blood chemistry investigations are helpful in diagnosis of some of the patients. Neuro imaging may also be useful mainly in Huntington's disease patients. Metabolic disorders and drugs are very important causes of non genetic chorea. Early diagnosis is important because majority of the patients respond to the treatment.
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PMID:Chorea. 2477 51

Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens. Anti-NMDA receptor encephalitis is one of the most common and may present with a variety of MDs, including: chorea, stereotypies, dystonia and myorhythmia. The recognition of other abnormal motor phenomena such as "faciobrachial dystonic seizures" and neuromyotonia, observed in patients with LGI1 and Caspr-2 antibodies, is important because they may herald the onset of overt limbic encephalitis. Autoimmunity directed against the intracellular enzyme glutamic acid decarboxylase usually presents with MDs, most commonly stiff-person syndrome or cerebellar ataxia. Chorea may be observed in rheumatologic disorders such as systemic lupus erythematosus or antiphospholipid syndrome. Disorders with uncertain autoimmune mechanisms such as Hashimoto's encephalitis and idiopathic opsoclonus-myoclonus syndrome commonly present with tremor, myoclonus and ataxia. A rapid diagnosis of an autoimmune disorder, which typically presents with subacute onset, is critical as early therapeutic intervention improves long-term prognosis and may be life-saving. Treatment usually involves some form of immunotherapy and symptomatic therapy of the abnormal movements with dopamine depleters, dopamine receptor antagonists, or GABAergic drugs. Detection and removal of an underlying tumor is essential for optimal outcome.
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PMID:Autoimmune and paraneoplastic movement disorders: An update. 2940 2