Cases reported "Chorea"

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11/23. Delayed choreoathetosis following acute carbon monoxide poisoning.

    Ten days after accidental exposure to carbon monoxide, a 17-year-old youth developed transitory choreoathetosis of both arms, face, and neck, with moderate dysarthria. CT revealed symmetric bilateral infarction in the head of the caudate nucleus, the putamen, and the small parts of the anterolateral globus pallidus.
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ranking = 1
keywords = putamen
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12/23. Hypertensive putaminal hemorrhage presenting with hemichorea.

    A progressive motor deficit primarily manifested by hemichorea developed in a 42-year-old hypertensive man. CT scan demonstrated contralateral putaminal hemorrhage. The patient's course was benign. Previous cases of acute hemichorea familiar to use and documented by CT scans have involved nonhemorrhagic lesions of either the putamen or caudate.
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ranking = 1
keywords = putamen
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13/23. Generalized chorea due to bilateral small, deep cerebral infarcts.

    We present a case of generalized chorea, with complete recovery, due to MRI-documented bilateral lacunar infarcts of the caudate nucleus, putamen, and deep frontal white matter. MRI is probably more sensitive than CT in disclosing small deep cerebral infarcts.
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ranking = 1
keywords = putamen
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14/23. Familial degeneration of the basal ganglia with acanthocytosis: a clinical, neuropathological, and neurochemical study.

    Three siblings, offspring of normal consanguineous parents, had a progressive neurological disorder characterized primarily by chorea and leading to death in the fourth or fifth decade. The most carefully studied patient had neither malabsorption nor absent serum beta-lipoprotein but did have erythrocyte acanthocytosis. Postmortem examination showed marked neuronal loss and gliosis of the caudate nucleus and putamen. Activities of glutamic acid decarboxylase and choline acetyltransferase were normal in cortex, caudate, and putamen. Autosomal recessive inheritance, acanthocytosis, and probable peripheral neuropathy help differentiate this disorder from Huntington's disease.
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ranking = 2
keywords = putamen
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15/23. Neuropathological study of chorea-acanthocytosis.

    Neuropathological studies of a woman with chorea-acanthocytosis were reported. The patient clinically showed generalized epileptic seizures, choreatic involuntary movements, hypotonia , areflexia, neurogenic muscular atrophy, tongue-biting and acanthocytosis of the peripheral blood and died from asphyxia at the age of 34. autopsy revealed atrophy of the caudate nucleus but the cerebral cortex was well preserved. Histological examination showed severe neuronal loss associated with moderate fibrous gliosis of the caudate. The putamen was similarly but less markedly affected. The small striatal neurons were more severely depopulated than the large neurons, although the latter were not spared. Cytometrical study of the caudate head revealed that the remaining small neurons were significantly larger in size than the normal small neurons of the same area. The anatomical substratum of the choreatic involuntary movements in chorea-acanthocytosis is thought to be the degeneration of the striatum, especially the caudate nucleus. Neuropathological differentiation of chorea-acanthocytosis from Huntington's chorea which shows similar pathology was discussed.
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ranking = 1
keywords = putamen
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16/23. Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis.

    A boy with glutaric acidemia had psychomotor retardation first noted at age 6 months, recurrent metabolic acidosis, and a progressive quadriparesis with choreoathetosis. He died at age 3 1/2 years. Cultured skin fibroblasts lacked glutaryl-coa dehydrogenase activity. There was a biochemical, but not a clinical, response to dietary restriction of lysine and tryptophan. The caudate and putamen of the brain showed severe loss of nerve cells and fibers with proliferation of astrocytes, as well as markedly reduced gamma-aminobutyric acid and glutamate decarboxylase activity.
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ranking = 1
keywords = putamen
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17/23. Hemichorea-hemiballism and lacunar infarction in the basal ganglia.

    We present a case of acute hemichorea-hemiballism associated with lacunar infarct documented by computerized tomography scan in the contralateral putamen and caudate nuclei. The pathoanatomic data of similar cases in the literature are reviewed with reference to the location of the responsible lesions. Acute hemichorea-hemiballism is most frequently caused by lacunar infarcts in the basal ganglia contralateral to the dyskinesia.
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ranking = 1
keywords = putamen
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18/23. Paroxysmal kinesigenic choreoathetosis associated with prenatal brain damage.

    We describe a 15 year old patient with paroxysmal kinesigenic choreoathetosis. Neurological examinations revealed a paresis of the right arm and hand that was similar to ulnar nerve palsy, a right homonymous hemianopsia and an ocular movement disturbance of smooth pursuit to left. Attacks of dystonic spasms began abruptly, usually following running, and lasted less than 5 min. magnetic resonance imaging displayed a linear area of increased signal in the T2-weighted images along the lateral margin to the left putamen, atrophies of the frontal and temporal opercula and a large porencephalic cyst in the left parieto-temporo-occipital region. A cerebral blood flow study with single photon emission computed tomography showed hypoperfusion of the lenticular nucleus and the regions corresponding to the atrophies and the porencephalic cyst. Electroencephalograms during the attacks could not demonstrate epileptic abnormality. Only the neuronal plasticity of an immature brain could explain the discrepancy between the observed huge lesions of the brain and the minor neurological symptoms present. Attacks of paroxysmal kinesigenic choreoathetosis might occur when the basal ganglia maturate to some extent, even if the lesions in the brain were caused before birth.
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ranking = 1
keywords = putamen
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19/23. Hyperintense putamen on T1-weighted MR images in a case of chorea with hyperglycemia.

    A 77-year-old woman had an acute onset of chorea after hyperglycemic coma. She had no family history of neurologic disorders. Although brain CT showed no detectable lesions in the putamen, MR revealed a high intensity on T1-weighted images and a low intensity on T2-weighted images in both putamina.
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ranking = 5
keywords = putamen
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20/23. Reversible striatal hypermetabolism in a case of Sydenham's chorea.

    We studied a 10-year-old girl with Sydenham's chorea (SC) using positron emission tomography (PET) with fluorodeoxyglucose (FDG). Choreic movements involved the head and the left side of her body. PET showed increased glucose metabolism in the right caudate nucleus and putamen. Three months after complete recovery, striatal glucose metabolism had returned to normal in the caudate nucleus. In the right putamen, glucose metabolism had decreased compared to that in the first study but remained elevated compared to that of normal young adults. We propose that the transient striatal hypermetabolism may have been due to increased afferent inputs to the striatum as a consequence of striatal or subthalamic nucleus dysfunction.
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ranking = 2
keywords = putamen
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