نتایج جستجو برای: huntington chorea
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Aims and background: Huntington’s chorea )HC( is a rare, autosomal, dominant hereditary disorder of the nervous system. This disease is characterized by progressive chorea movement, dementia and psychiatric disorders. In these patients, an abnormal response to many anesthetic drugs has been observed. The primary goal of general anesthesia in these patients is airway protection and a fast and ...
Huntington disease has been linked to increased dopaminergic neurotransmission in the striatum, and clinical studies have demonstrated that the associated chorea can be treated with dopamine antagonist or dopamine-depleting drugs. The origin of this hyperdopaminergic status is unknown. Because substantia nigra pars compacta and the ventral tegmental area are the main sources of striatal dopamin...
Huntington's chorea is an hereditary disorder of the central nervous system characterized by the appearance in adult life of progressive chorea and dementia, and inherited in single autosomal dominant fashion with complete penetrance. The essential pathological features of Huntington's chorea are a primary loss of cells in the caudate nucleus and the putamen, and similar involvement of the cere...
Autosomal dominant choreas are genetically heterogeneous disorders including Huntington disease (HD), Huntington disease like 1 (HDL1), Huntington disease like 2 (HDL2), dentatorubro-pallidoluysian atrophy (DRPLA), spinocerebellar ataxia type 17 (SCA17) and benign hereditary chorea (BHC). We identified two Japanese families with adult-onset benign chorea without dementia inherited in an autosom...
Chorea-the term itself is a rather peculiar one. The word chorea is derived from the Greek word for dance and originally was applied to describe the dancelike gait of the dancing mania, a hysterical disorder of the late Middle Ages. The term is now used to describe an entire class of abnormal spontaneous movements. Each of these movements is a single, isolated muscle action: a short, rapid , un...
INTRODUCTION Tetrabenazine (TBZ) reduces chorea related to Huntington disease (HD); however, it is uncertain whether this effect improves functionally relevant motor skills such as hand coordination and balance. The objective of this study was to provide pilot data regarding three motor function tests, which might be useful in monitoring symptom progression and therapeutic response, pending for...
Huntington chorea is a rare autosome dominant disorder. Men and women are equally affected as it is an autosomally transmitted. Symptoms occur in the third and fourth decade of life and manifest as involuntary choreiform movements, ataxia, and progressive mental deterioration. Patients are at higher risk for some intraoperative complications. Only a few case reports have been published reportin...
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