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)

There are three different neurological complications of measles infections in the brain: acute postinfectious encephalitis, acute progressive infectious encephalitis, and subacute sclerosing panencephalitis. The diagnosis of measles encephalitis (ME) is established when supported by the clinical picture, mainly of juvenile onset, and confirmed by the presence of cerebrospinal measles antibodies. Although ME is clinically characterized by progressive behavioral and mental deterioration associated with myoclonus, prior reports have suggested that adult-onset may have atypical features. We describe a 28 year-old immunocompetent man, admitted into the hospital due to a rapid motor and cognitive decline after an episode of fever and gastroenteritis. His neurological examination was significant for cognitive impairment, cervical dystonia, spontaneous and action induced myoclonus, choreiform movements, parkinsonism and ataxic gait. He was diagnosed of acute postinfectious ME based on the presence of elevated intrathecal synthesis of measles antibodies in his CSF, and a lymphocytic infiltrate of perivascular distribution without viral inclusions, with PCR negative for measles from brain biopsy. The patient continued to deteriorate to an akinetic mutism state, dying a few weeks later. Adult-onset ME is an entity rarely seen in the Western world. Although myoclonus is the most common movement disorder related to juvenile-onset ME, ataxia and other dyskinesias such as chorea, dystonia, and parkinsonism, can result from this infection when presenting in adult life.
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PMID:[Movement disorders in adult-onset measles encephalitis]. 1259 Mar 79

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

Niemann-Pick disease type C (NPC) is an autosomal recessive neurometabolic disorder that rarely presents in adulthood, and is associated with cognitive decline, various movement disorders (ataxia, chorea, dystonia, and myoclonus), a vertical supranuclear gaze palsy (VSGP), and seizures. A recent case report demonstrated a delay in diagnosis of eight years when a patient with NPC presented with psychosis. This article reviewed all cases seen at the Mayo Clinic with a possible diagnosis of NPC between 1976 and 2000. Of the 52 possible cases, five had an established diagnosis of adult onset NPC. Of these, two presented with psychosis and were not diagnosed with NPC for 5 and 15 years, respectively. NPC may initially present in adulthood with psychosis, and when psychosis is associated with VSGP, various dyskinesias, and seizures, NPC should be suspected.
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PMID:Adult onset Niemann-Pick disease type C presenting with psychosis. 1264 83

Movement disorders as postinfectious manifestation of group A streptococcal infections have been reported and are thought to occur on an autoimmune basis. We describe an unusual case of multifocal myoclonus following strep throat infection. Clinical description and chart review were the method used. A 10-year-old boy developed focal myoclonus involving his right arm and shoulder 1 week after streptococcal throat infection treated with penicillin. His magnetic resonance image was normal, and he initially responded to clonazepam but did not sustain a response. The myoclonus spread to involve all limbs and the trunk becoming multifocal over the next few weeks. He did not have choreoathetosis of Syndenham's chorea. He was given one course of intravenous immunoglobulin and became asymptomatic after treatment. He remained symptom free for 8 months following intravenous immunoglobulin treatment. Various symptoms have been reported following group A streptococcal infections in children. These neurobehavioral abnormalities may be mediated through antineuronal antibodies. Our case demonstrates multifocal myoclonus as a poststreptococcal autoimmune phenomenon. To our knowledge, only two other cases of poststreptococcal myoclonus have been reported in the literature. Recognition of this unusual condition as a manifestation of autoimmune poststreptococcal disease in children is essential to avoid overinvestigation and to ensure early treatment.
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PMID:Multifocal myoclonus following group A streptococcal infection. 1288 83

Pediatric movement disorders constitute a relatively small cluster of symptoms that can be associated with many different underlying diseases. To provide effective treatment, it is essential to understand the relationship between etiology and clinical expression. This article reviews the recent literature on several common pediatric movement disorders, including spasticity, dystonia, chorea, myoclonus, bradykinesia, and tics, and it discusses current models of physiology that may help link the cellular pathology of specific diseases to the expression of clinical symptoms.
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PMID:Pathophysiology of pediatric movement disorders. 1367 68

Six cases representing the most commonly encountered movement disorders-restless legs syndrome with periodic limb movements, tics, myoclonus, chorea, essential tremor, and cervical dystonia-are presented. Discussion of each case focuses on a practical approach to recognizing the important clinical features of each movement disorder as well as the current therapeutic options. A detailed discussion of botulinum toxin follows the case on cervical dystonia, focusing on its mechanism of action, clinical indications, side effects, and dosing.
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PMID:Case studies in movement disorders. 1472 23

Upon clinical examination of involuntary movement, its correct diagnosis and classification is most important for choosing its most appropriate treatment. Correct diagnosis of involuntary movements should be based on their clinical characteristics rather than laboratory data. First attention should be paid to the way of its occurrence; whether it repeats itself at a relatively regular period or it occurs irregularly. Among irregular movements, chorea and ballism (us) belong to the same category clinically as well as neuropharmacologically; both respond to benzodiazepine and D2 receptor blockers. Dyskinesia and akathisia are often drug-induced. Athetosis often overlaps chorea and dystonia. Dystonia is characterized by excessive muscle contraction in terms of strength, spread and duration. Recently involvement of the sensori-motor cortex is emphasized in generation of focal dystonia. Myoclonus is usually caused by abrupt muscle contraction but also by sudden cessation of muscle contraction (negative myoclonus). Myoclonus and tics are usually irregular, but they can be rhythmic or quasiperiodic. Tremor is often associated with dysfunction of basal ganglia, cerebellum or thalamus, but recently an increasing attention has been paid to involvement of the sensori-motor cortex. Some involuntary movement could show features of two or more kinds of involuntary movements or it may shift among different kinds of involuntary movements or among different subgroups of that particular involuntary movement. Therefore, it is not advisable to feel obliged to classify every single involuntary movement into any pre-existing category. Instead a more flexible approach is warranted.
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PMID:[How to clinically approach involuntary movements]. 1515 56

The main clinical characteristics and aetiology of drug-induced tremor and acute movement disorders (myoclonus, chorea, athetosis, dyskinesia, dystonia, tics) are reviewed and discussed in this article. Tremor is the most frequent drug-induced acute movement disorder. Drug intake (and, initially, true or 'hidden' neuroleptic or psychoactive drugs) should be suspected as the cause of every abnormal movement. Drug-induced acute movement disorders usually disappear spontaneously after withdrawal of the suspected drug without any drug treatment. Drug-induced tremor and movement disorders are often poorly described in the Summary of Products Characteristics (SPC).
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PMID:[Drug-induced tremor and acute movement disorders]. 1520 22

Movement disorders are a potential neurologic complication of acquired immune deficiency syndrome (AIDS), and may sometimes represent the initial manifestation of HIV infection. Dopaminergic dysfunction and the predilection of HIV infection to affect subcortical structures are thought to underlie the development of movement disorders such as parkinsonism in AIDS patients. In this review, we will discuss the clinical presentations, etiology and treatment of the various AIDS-related hypokinetic and hyperkinetic movement disorders, such as parkinsonism, chorea, myoclonus and dystonia. This review will also summarize current concepts regarding the pathophysiology of parkinsonism in HIV infection.
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PMID:Movement disorders and AIDS: a review. 1526 74

Head injury can cause extrapyramidal movement disorders such as tremors, parkinsonism, dystonia, chorea, myoclonus, and tics. Pure adventitious movements are rare, but combinations with paresis, spasticity, apraxia, or ataxia occur in approximately 20% of cases of severe head injury, in many cases appearing or evolving in the months following the injury. Tremors may improve in time but many of the other syndromes tend to persist. Reversible causes such as medications or metabolic derangements are occasionally identifiable. Some of these adventitious movements can be improved using neuroactive drugs, botulinum toxin injections, or stereotactic brain surgery.
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PMID:Movement disorders after head injury: diagnosis and management. 1526 58


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