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)

A 56-year-old woman had a 50-year history of childhood-onset chorea without progressive neurologic deficit. The patient's father had a lifelong extrapyramidal disorder characterized by a head-nodding tremor and involuntary movements especially evident with anxiety. The computerized tomographic scan was normal, without evidence of cortical or caudate atrophy. The computerized tomographic scan supports the notion of a functional rather than a structural lesion, and may aid in the discrimination of hereditary nonprogressive chorea from more devastating forms of hereditary chorea.
 ...
PMID:Computerized tomography in hereditary nonprogressive chorea. 15 81

The first documented case of Huntington's Disease (HD) in Thailand is reported. The patient presented with classical clinical picture, clinical course and family history. Although hereditary chorea is most frequently associated with HD, other hereditary basal ganglion diseases, Syndenham's chorea, and other causes of abnormal movements were considered and excluded by clinical profiles and investigations. Various recent aspects of HD are briefly mentioned.
 ...
PMID:Huntington's disease in Thailand: a case report. 138 18

Positron emission tomography (PET) with [18F]-2-fluoro-2-deoxy-D-glucose (FDG) was used to investigate the regional cerebral metabolic rate of glucose consumption (rCMRGlc) in two patients with benign hereditary chorea (BHC) and 21 normal subjects. Relative and absolute values of cerebellar, striatal, thalamic, and cortical rCMRGlc were within normal limits for both patients with BHC, indicating that the choreic movement disorder encountered in these two patients was not caused by a decrease of energy metabolism in the striatum such as that found regularly in most patients with other forms of chorea (e.g. Huntington's and Wilson's disease).
 ...
PMID:Normal striatal glucose consumption in two patients with benign hereditary chorea as measured by positron emission tomography. 214 58

A family is reported with five generations showing hereditary chorea. In the youngest generation, two members had so severe choreic hyperkinesis that gait was impaired in one of them and totally impeded in the other; they also had associated mental retardation, which was not present in other members with variable degrees of hyperkinesis. There was no therapeutic response to several drugs. The literature regarding genetical, clinical and therapeutic aspects of this condition is reviewed, and it is concluded that the most adequate denomination for the disease is familial nonprogressive chorea rather that the accepted one of benign familiar chorea.
 ...
PMID:[Benign familial chorea. A not so benign entity]. 253 15

The pathophysiology of chorea in systemic lupus erythematosus (SLE) is uncertain. Pathologic examination has not identified a specific location for the causative lesion(s) and immunologic mechanisms have been suggested in its etiology. In other choreic disorders, such as Huntington's disease and benign hereditary chorea, glucose hypometabolism in the striatum has been demonstrated by positron computed tomography (PCT) using [18F]deoxyglucose. With this technique we have studied four patients with chorea secondary to SLE. In these patients the regional distribution of cerebral glucose metabolism was normal. In particular, striatal glucose metabolism was within the normal range, even though the ratio of striatal to cortical glucose metabolism was increased. Our results show that striatal hypometabolism, as seen in other disorders manifesting chorea, is not the PCT correlate of the dyskinesia.
 ...
PMID:Regional cerebral glucose metabolism in SLE chorea: further evidence that striatal hypometabolism is not a correlate of chorea. 350 75

Chorea is a hyperkinetic involuntary movement disorder characterized by a random pattern of irregular muscle jerks. This movement may involve any parts of the body. Emotional stress or voluntary movements may exacervate chorea and sleep abolish it. Luys body and striatum are the most important anatomical sites to evoke chorea. The lesion of inner segment of pallidum or ventrolateral thalamus may abolish chorea. Measurements of neurotransmitter changes of Huntington's disease show diminution of striatal GABA neurons and preserving nigrostriatal dopamine neurons. Dopamine antagonists can reduce chorea because doperminergic hyperactivity contribute to exacervate chorea. Precise pathophysiological mechanism of chorea is controversial, therefore its classification is not established. On the clinical point of view, classification according to heredity is useful to make diagnosis because hereditary diseases are easily confirmed by family history or specific biochemical markers. There are two groups of underlying diseases of non hereditary chorea. One is unilateral chorea usually due to contralateral hemispheric lesions to chorea. Another is bilateral chorea usually due to degenerative, metabolic or toxic brain diseases. Recent identification of abnormal DNA structure (trinucleotide repeat) in Huntington's disease may greatly contribute to classify underlying diseases of chorea.
 ...
PMID:[Chorea: general aspect and classification]. 827 57

We report on 2 brothers with chorea and monocular horizontal nystagmus beginning in early infancy, both of which remit during the first decade of life, and peripheral cataracts. While this condition shares manifestations with benign hereditary chorea and several other familial movement disorders, the slowly remitting course of the chorea combined with the visual abnormalities appears to be unique.
 ...
PMID:Familial remitting chorea, nystagmus, and cataracts. 829 59

We report a nonconsanguineous family in whom two (of three) sons developed isolated chorea in early childhood, suggesting a diagnosis of benign hereditary chorea (BHC). However, cerebellar ataxia and oculomotor apraxia, without telangiectasia, subsequently developed. Chromosome analysis showed increased radiosensitivity in both brothers and translocations in the younger one. We conclude that ataxia with chromosomal instability may masquerade as BHC in some patients.
 ...
PMID:Ataxia without telangiectasia masquerading as benign hereditary chorea. 1148 21

A large Dutch family of 88 members, running through five generations, is described with benign hereditary chorea of early onset. The clinical presentation was heterogeneous. The chorea manifested in late infancy or childhood, interfered with writing, was non-disabling, stable or even improved in adulthood in most cases, but was slowly progressive with gait impairment in some. There was mild dysarthria and normal intelligence. EEG brain CT-scanning and MRI were normal. Huntington's disease was excluded by analysis of the I T 15 gene, which showed a normal number of the CAG trinucleotide repeats in two patients. It is concluded that benign hereditary chorea of early onset is an entity different from Huntington's disease and that in cases of early onset chorea the diagnostic accuracy is markedly improved by DNA testing.
 ...
PMID:A Dutch family with benign hereditary chorea of early onset: differentiation from Huntington's disease. 883 92

Stimulant therapy is usually contraindicated in patients with movement disorders such as tics or chorea. A young boy is reported who had benign hereditary chorea and attention deficit disorder, whose chorea, handwriting, and independent ambulation paradoxically improved with methylphenidate treatment.
 ...
PMID:Benign hereditary chorea improved on stimulant therapy. 896 90


1 2 3 4 Next >>