Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Published reports provide contradictory evidence concerning the cause, nature, and course of Syndeham's chorea: physiological, psychological, and interractional hypotheses have been advanced. Reports supporting any of these views suffer from lack of detail, objective observations, and/or longitudinal behavioral sampling. This case was studied longitudinally with numerous psychological tests. They revealed acute encephalopathy and severe personality disturbance in the acute stage, followed by rapid and lasting clearing of both symptoms. Thus, this case provided evidence consistent with virtually all published reports and with each hypothesis about Sydenham's chorea, suggesting the need for more careful longitudinal studies and a conceptual framework which can more fully embrace all the data.
 ...
PMID:Psychological correlates of the course of Sydenham's chorea. Repeat evaluations of a case. 116 35

Antiphospholipid antibodies are a relatively heterogeneous mix of immunoglobulins with binding specificities for negatively charged or neutral phospholipids. Currently, the most commonly detected antiphospholipid antibodies include the anticardiolipin antibody, the lupus anticoagulant, and an antibody implicated in false-positive VDRL testing. Recently, a clinical syndrome of vaso-occlusive disorders associated with antiphospholipid antibodies has been identified and may result from immune-mediated disruption of endothelial function. This clinical syndrome encompasses arterial and venous thrombosis, recurrent fetal loss, neurologic dysfunction (eg, migraine, chorea, and encephalopathy), systemic and pulmonary arterial hypertension, and endocardial disease. Although most commonly associated with systemic lupus erythematosus, the antiphospholipid antibody syndrome also has been identified in patients with vaso-occlusive disease without systemic lupus erythematosus. Recently, identification of antiphospholipid antibodies has been facilitated by the development of a more sensitive assay for anticardiolipin antibody. In this article, case histories of three patients with arterial thrombosis and associated anticardiolipin antibodies, including the first associated case of terminal aortic thrombosis, are reviewed and the subject of the antiphospholipid antibody syndrome is discussed.
 ...
PMID:Antiphospholipid antibodies and arterial thrombosis. Case reports and a review of the literature. 155 Apr 84

A five-year-old male was admitted to the hospital with generalized seizures. Enlarged lymph nodes raised the suspicion of cat-scratch disease. The diagnosis was confirmed by a positive history of a cat bite, typical histopathologic findings in the biopsy of the lymph nodes, and a positive skin test. Brain CT scan and LP were repeatedly normal. The clinical course was remarkable for recurrent episodes of status epilepticus refractory to usual anticonvulsant therapy and prolonged encephalopathy consisting of mental confusion, hemiparesis, tremor, chorea, and vomiting. All neurologic symptoms gradually resolved within nine months, without sequelae. Cat-scratch encephalopathy should be suspected in a child presenting with status epilepticus and enlarged lymph nodes. Aggressive and prolonged anticonvulsant therapy is strongly recommended.
 ...
PMID:Cat-scratch encephalopathy presenting as status epilepticus and lymphadenitis. 232 Apr 87

A 15 year old boy was evaluated in the psychiatric emergency room for the acute onset of "confusion,"insomnia, headache, and shaking of one week's duration. Two days later hallucinations, formication and a movement disorder emerged characterized by action tremor, myoclonus, chorea and ataxia. Further history revealed inhalation of gasoline for its euphoric effects. Plasma lead levels were in the toxic range. Chelation therapy reversed the clinical symptoms. Behavioral changes and a movement disorder in the context of gasoline inhalation are highly suggestive of organic lead encephalopathy. Recognition of this syndrome is important as chelation therapy is effective.
 ...
PMID:Organic lead encephalopathy: behavioral change and movement disorder following gasoline inhalation. 705 7

To document possible changing characteristics of Sydenham chorea, we reviewed records of 240 patients with this diagnosis who were seen between 1951 and 1976. A dramatic progressive decline in the number of cases was observed. The syndrome occurred mainly in childhood. Female predominance was apparent only after the 10. There was a high femilial incidence for both chorea and rheumatic fever. Most patients had generalized chorea, and fewer than 20% had hemichorea. Dysarthria, probably of extrapyramidal origin, was frequent but neurologic abnormalities other than diffuse encephalopathy were rare. One-third of the patients had coexisting heart disease. Repeat attacks of Sydenham chorea occurred, but the recurrence rate was much less than noted in previous studies.
 ...
PMID:Sydenham chorea: an update. 718 38

A previously healthy young man developed encephalopathy after a wasp sting in 1968. Neurological complications included chorea, currently of a minor degree, replaced by buccofacial dyskinesias and compulsive movements. The most disabling consequences were of psychic nature, the patient presenting an unusually marked compulsive activity of an obsessional type. The whole psychic picture however was quite different from that of the usual neurosis, since there was neither anxiety nor doubt, but a mental vacuum contrasting with almost normal intellectual and affective capacities. It was as though there was a loss of the auto-activation system of psychic life, postulated by dynamic psychologists. C.A.T. demonstrated bilateral pallidostriatal necrosis. This suggests that the striatum plays a motor and psychic role. In the field of motor control, its hypoactivity appears to determine not only chorea but dyskinesias similar to tics and compulsive movements. In the psychic field, the striatum appears to play the very general role of an auto-activation system of intellectual and affective life, its deficiency leading to mental activity of a compulsive nature. Improvement in knowledge of striatal biology should clarify not only the basic mechanisms of chorea, but those of tics and obsessions.
 ...
PMID:[Obsessional-type compulsive behavior caused by bilateral circumscribed pallidostriatal necrosis. Encephalopathy caused by a wasp sting]. 728 Apr 38

Leigh syndrome (LS) is the clinical prototype of a genetically-determined mitochondrial encephalopathy. Twenty-two of 34 patients with LS had evidence of a movement disorder (MD). Dystonia, the most common MD, was present in 19 cases, rigidity in 4, tremor in 2, chorea in 2, hypokinesia in 2, myoclonus in 1, and tics in 1. Dystonia was most commonly multifocal at onset and showed progression in six patients. In half of the cases an enzymatic defect was detected, most commonly cytochrome C oxidase. The neuroradiologic findings showed prominent basal ganglia lesions in 20/21 patients. Putamen, caudate, substantia nigra and globus pallidus were involved in this order of frequency. This experience was reflected in a literature review encompassing 284 cases of LS. However, only 26.4% had MD. Eleven patients, including one of our cases, presented as the primary torsion dystonia phenotype. There are clinical and pathological similarities between LS and other metabolic diseases affecting the central nervous system. The enhanced vulnerability of the nervous system to metabolic stress and the resemblance in the distribution of the pathology of these diverse conditions suggests a common pathogenetic mechanism. An excitotoxin-mediated mechanism is favored, one which might account for the frequent involvement of the basal ganglia in LS.
 ...
PMID:Disorders of movement in Leigh syndrome. 839 42

We studied 53 patients (64% females) with static brain lesions who developed progressive movement disorders. Of these, 50 (94%) had dystonia, 17 (32%) tremor, eight (15%) parkinsonism, seven (13%) myoclonus, and three (6%) chorea. The precipitating insults included perinatal hypoxia/ischemia in 22 (42%), stroke in 12 (23%), head injury in eight (15%), encephalitis in eight (15%), and carbon monoxide poisoning, kernicterus, and radiation necrosis in one patient (2%) each. Among the 30 patients with initial insult occurring at age 2 years or younger (Infant group), distribution of dystonia at follow-up was focal in three (10%), segmental in eight (27%), unilateral in 10 (33%), and generalized in nine (30%). The mean latency between the original injury and onset of movement disorder was 25.5 +/- 16.7 years. Among the nine patients who developed dystonia after an insult occurring between ages 6 and 17 (Childhood group), the distribution of dystonia at follow-up was segmental in two (33%) and unilateral in seven (78%); the mean latency of dystonia onset was 4.9 +/- 7.8 years. Of the 14 patients in the Adult group (injury at age 25 or older), 11 developed dystonia, two developed parkinsonism, and one had carbon monoxide encephalopathy and parkinsonism. The distribution of dystonia in the 11 patients at follow-up was segmental in three (27%) and unilateral in eight (73%). The mean latency of movement disorder onset in the 14 patients of the Adult group was 2.5 +/- 4.9 years. No individuals in the Childhood or Adult groups became left-hand dominant; by comparison, nine of the 30 individuals in the Infant group became left-handed. In conclusion, brain injury at a young age is associated with a longer latency to onset of subsequent movement disorder, a greater tendency to development of generalized dystonia, and a greater probability of altered handedness. These tendencies may result from differences in age-related neuroplasticity.
 ...
PMID:Delayed-onset progressive movement disorders after static brain lesions. 890 76

We report a case of rapidly progressive encephalopathy and generalized chorea due to HIV encephalitis. The patient was a 24-year-old man known to be HIV-seropositive for 4 years. The severity of the movement disorder resulted in rhabdomyolysis. Sepsis developed and he died after a 21-day hospitalization. Pathologic study revealed prominent neuronal loss and gliosis of subcortical regions. Acute encephalopathy with generalized chorea may be a rare consequence of HIV encephalitis.
 ...
PMID:HIV encephalitis presenting with severe generalized chorea. 878 Jan 14

Antiphospholipid antibodies (aPL) have been associated with a variety of neurological disorders, mostly linked to focal neuroparenchymal ischemia or infarction. Cerebral ischemia associated with the antiphospholipid syndrome (APS) occurs at a younger age than typical atherothrombotic cerebrovascular disease, is often recurrent, and high positive GPL values are usually linked to the presence of a lupus anticoagulant. When other features of the syndrome are not present and cerebral ischemia occurs only associated with anticardiolipin immunoreactivity, there appears to be no discerning features of these patients unless GPL > 40 for which recurrent thrombo-occlusive events appear to occur more frequently. Other neurological manifestations associated with aPL include cerebral venous sinus thrombosis, ocular ischemia, dementia, including ischemic encephalopathy, and chorea. The role of aPL in migrainous events is controversial and may not play a role in recent, large case-controlled studies. Most seizures in patients harboring aPL are associated with focal brain infarction.
 ...
PMID:Neurological aspects of antiphospholipid antibody syndrome. 890 59


1 2 3 4 5 6 Next >>