title: [Pathological status of catecholamines in the corpus striatum in hepatocerebral dystrophy (Wilson-Konovalov disease)].
abstract: The content of catecholamines in the striatum was measured in 2 patients suffering from hepatocerebral dystrophy (Wilson-Konovalov disease). It is noted that in different clinical manifestations of the disease, the changes in the content of noradrenaline in the striatum varied. A male patient with marked tremor spreading manifested a considerable rise of the content of catecholamines, primarily in the n. caudatus. At the same time in a female patient with a grave akinetic -rigid syndrome and the signs of liver failure, the content of catecholamines, particularly dopamine, in the putamen was low. In view of this fact it is assumed that disorders of cerebral metabolism of catecholamines, dopamine in particular, evidently related to deposition of excess copper, leading to the changes of their content in basal ganglia underline the clinical pleomorphism of the disease and play the key role in the development of extrapyramidal motor disturbances characteristic of hepatocerebral dystrophy.
authors.forename: V P
authors.initials: VP
authors.lastname: Barkhatova
authors.forename: E G
authors.initials: EG
authors.lastname: Larskiĭ
authors.forename: I A
authors.initials: IA
authors.lastname: Ivanova-Smolenskaia
authors.forename: E D
authors.initials: ED
authors.lastname: Markova
authors.forename: E G
authors.initials: EG
authors.lastname: Demina
mesh_terms: D000328:Adult; D001479:Basal Ganglia / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D002421:Caudate Nucleus / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D003300:Copper / Q000737:chemistry / Q000378:metabolism*; D003342:Corpus Striatum / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology; D004298:Dopamine / Q000737:chemistry / Q000378:metabolism*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008297:Male; D009638:Norepinephrine / Q000172:deficiency / Q000378:metabolism*; D011699:Putamen / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D013050:Spectrometry, Fluorescence
chemical_list: D003300:Copper; D004298:Dopamine; D009638:Norepinephrine
vernacular_title: K patologii katekholaminov v polosatom tele pri gepatotserebral'noĭ distrofii (bolezni Vil'sona-Konovalova).