The Copper and Iron Content of Brain and Liver in the Normal and in Hepato-Lenticular Degeneration

Cumings, J. N.

doi:10.1093/brain/71.4.410

Cited by 356 publications

(74 citation statements)

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“…Ceruloplasmin, which transports not only copper but also iron, is decreased in Wilson's disease, and this enhances iron accumulation in the body [89,90]. Indeed, in autopsy studies, both copper and iron accumulation were found to be higher in brains with Wilson's disease than in control brains [103,104]. In addition, a PET study with radioactively labeled iron in human subjects showed increased iron uptake by the brain in Wilson's disease patients compared to healthy volunteers [105].…”

Section: Coppermentioning

confidence: 99%

Contribution of metals to brain MR signal intensity: review articles

et al. 2016

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Section: Coppermentioning

confidence: 99%

Contribution of metals to brain MR signal intensity: review articles

et al. 2016

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“…Wilson comme une maladie familiale constamment mortelle, caracté-risée par une atteinte neurologique associée à une cirrhose [2]. L'implication de la surcharge en cuivre dans le foie et le cerveau des patients atteints n'a été mise en évidence qu'en 1948 par Cumings [3]. La connaissance de ce rôle du cuivre a permis de disposer d'un traitement efficace dès 1956 quand Walshe a proposé l'utilisation d'un chélateur du cuivre : la pénicillamine [4].…”

Section: Discussion Et Revue De La Littératureunclassified

Maladie de Wilson : présentation d’un cas et revue de la littérature

Pesudo

Willmann

Cousty

2016

Med Buccale Chir Buccale

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“…It was twelve years later, in 1948, that Cumings 18 described the copper abnormalities in Wd and posteriorly, in 1952, Scheinberg and Gitlin 19 showed that the ceruloplasmin levels were low in most of Wd patients.…”

Section: Discussionmentioning

confidence: 99%

“…In a two-hour period the patient with Wd excreted 41.7 µg of copper, whereas none of the other twelve patients studied excreted more than 18 µg. In the same year Cumings 18 20 reported the increase of serum free copper, not bound to ceruloplasmin, in patients with Wd.…”

mentioning

confidence: 92%

Wilson's Disease: a case report and a historical review

Barbosa

Machado

Cançado

et al. 2009

Arq. Neuro-Psiquiatr.

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-The purpose of this report is to present a short review of the history of Wilson's disease and to describe the first diagnosed case at the Neurologic Clinic of Hospital das Clínicas of São Paulo University Medical School. The topics of the historical review are the first contributions of authors along the second half of the XIX century, the seminal monograph of Samuel Alexander Kinnier Wilson (1912), the landmarks in the investigation of mechanisms of the disease and the introduction of the first effective treatment by John Walshe (1956). The first case studied in our Clinic, in 1946, was a 20 year-old male whose main neurological manifestations were postural tremor ("wing beat") and dysarthria and could be characterized as WestphalStrümpell form of the disease. Along the discussion of this case difficulties to establish the diagnosis and to treat the patient at that time are highlighted. We conclude with a brief history of the development of researches on Wilson's disease in our Clinic, with an honor to the pioneer contributions of Horacio Martins Canelas.Key WordS: Wilson's disease, historical review, copper metabolism. Doença de Wilson: relato de caso e revisão históricaResumo -Neste artigo inicialmente é feito um retrospecto dos principais marcos na história dos conhecimentos sobre a doença de Wilson, desde as primeiras descrições de casos no século XIX, passando pela magnífica monografia de Samuel Alexander Kinnier Wilson, m 1912, pelas descobertas sobre a causa da doença e chegando à era do tratamento efetivo da moléstia inaugurada por Walshe em 1956. A seguir, relata-se o primeiro caso de doença de Wilson estudado na Clínica Neurológica do HC-FMUSP. o paciente admitido na Clínica Neurológica em 1946, aos 20 anos de idade, apresentava a variante da doença em que predominavam tremor postural e disartria, conhecida como forma de Westphal-Strümpell. Na discussão, são ressaltadas as dificuldades da época para a confirmação do diagnóstico e para o tratamento; além de se realizar um breve histórico do estudo da doença na Clínica Neurológica, com o devido realce para a figura de Horácio Martins Canelas, pela sua participação pioneira nas pesquisas sobre a doença de Wilson em nosso meio.PAlAvrAS-CHAve: doença de Wilson, revisão histórica, metabolismo do cobre.

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The Copper and Iron Content of Brain and Liver in the Normal and in Hepato-Lenticular Degeneration

Cited by 356 publications

References 0 publications

Contribution of metals to brain MR signal intensity: review articles

Contribution of metals to brain MR signal intensity: review articles

Maladie de Wilson : présentation d’un cas et revue de la littérature

Wilson's Disease: a case report and a historical review

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