Results 161 to 170 of about 2,772 (216)
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HEPATOLENTICULAR DEGENERATION

Archives of Neurology & Psychiatry, 1950
THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1Westphal2and Strumpell3and the numerous subsequent reports make it possible to delimit this ...
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HEPATOLENTICULAR DEGENERATION

Archives of Neurology & Psychiatry, 1930
The concept of progressive lenticular degeneration as a clinicopathologic entity has changed since its first delineation by Wilson1in 1912. Characterized clinically by progressive symptoms referable to the basal ganglia, often clearly familial in incidence, usually occurring in young persons, running either an acute or a chronic course and always fatal,
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Osteoarthropathy of hepatolenticular degeneration

Acta Neurologica Scandinavica, 2009
Ninety-six joints of 25 patients with hepatolenticular degeneration have been studied by means of scintigraphs with technetium (99mTc), a method which can disclose early synovial inflammation and postinflammatory articular degeneration. Signs of synovitis were found in 22 joints of 10 patients (40%) and degenerative changes were present in 15 joints of
H M, Canelas   +5 more
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HEPATOLENTICULAR DEGENERATION

Archives of Internal Medicine, 1945
Wilson1in 1912, in an article entitled "Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver," first clearly defined the condition now most widely known as hepatolenticular degeneration. His accurate description included and amplified the poorly characterized so-called pseudosclerosis, described by ...
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HEPATOLENTICULAR DEGENERATION

Journal of the American Medical Association, 1933
Hepatolenticular degeneration is the name suggested by Barnes and Hurst 1 in 1925 for a rare, often familial, disease characterized by symptoms of extrapyramidal disturbance associated with a marked lobular cirrhosis of the liver. At autopsy, the extrapyramidal symptoms are accounted for by bilateral degenerative processes in the lenticular nuclei ...
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Heart involvement in hepatolenticular degeneration

Acta Neurologica Scandinavica, 2009
The case of a 10-year-old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin-eosine and the histochemical method with rubeanic ...
E M, Azevedo   +4 more
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HISTOCHEMICAL STUDY ON HEPATOLENTICULAR DEGENERATION

Psychiatry and Clinical Neurosciences, 1963
SummaryThe brain and liver of five classical cases of hepatolenticular degeneration were histochemically examined for copper and iron. Within the brain, both copper and iron were detected in two cases. Coppsr was demonstrated in the liver of all cases and iron in two cases. The histochemical findings obtained were discussed.
I, ARIOKA   +4 more
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Penicillamine Therapy for Hepatolenticular Degeneration

JAMA, 1964
Ingestion of copper was reduced and its excretion was enhanced pharmacologically, principally by the administration of penicillamine, in 33 patients with hepatolenticular degeneration, of whom eight were asymptomatic. Concomitant clinical improvement was noted most markedly and regularly in the neurological manifestations of the disease, whereas the ...
I, STERNLIEB, I H, SCHEINBERG
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HEPATOLENTICULAR DEGENERATION

Archives of Neurology & Psychiatry, 1941
The question whether the entity described by Kinnier Wilson should be expanded to include additional symptoms and signs reported by others in similar cases has been debated in the scientific literature for the past two decades. The case presented here is of interest because of the nature of the onset, the absence of tremor, the clinical methods used in
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[Hepatolenticular degeneration].

Neurologija, 1991
Hepatolenticular degeneration (Wilson's disease) is a hereditary disease in which metabolic disorder of copper leads to its accumulation in the liver, brain, cornea and kidneys with consequent pathologic changes in those organs. Hereditary mechanism of the disease is autosomal recessive with prevalence of 30-100 per 1,000,000 inhabitants.
D, Zudenigo, M, Relja
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