Leigh's subacute necrotizing encephalopathy: clinical and biochemical study, with special reference to therapy with lipoate.
نویسندگان
چکیده
منابع مشابه
MR of Leigh's disease (subacute necrotizing encephalomyelopathy).
MR images of three patients with Leigh's disease (subacute necrotizing encephalomyelopathy) were compared with CT findings. In all patients typical lesions in the basal ganglia were identified with both MR and CT. In two patients MR permitted identification of additional lesions not detected with CT. In one patient progression of MR abnormalities over a 4-month period correlated well with clini...
متن کاملErythrocyte transketolase activity in suspected cases of Leigh's disease, or subacute necrotising encephalomyelopathy.
Erythrocyte transketolase activity and the effect of adding thiamine pyrophosphate (% thiamine pyrophosphate effect) were measured in 111 subjects suspected to suffer from Leigh's disease (subacute necrotising encephalomyelopathy). From clinical evidence these subjects were divided into five groups: (1) necropsy-proved cases of subacute necrotising encephalomyelopathy, (2) cases positive for ur...
متن کاملLeigh's Disease: The Acute Clinical Course of a Two-Year-Old Child with Subacute Necrotizing Encephalomyelopathy
We report the untypical clinical course of a previously healthy two-year-old girl, who died suddenly and unexpectedly after an episode of vomiting. At forensic autopsy no other pathological findings could be diagnosed than multiple reddish, sunken areas in brain stem, mesencephalon, and pons. Histologically they presented as areas of spongiosis of the neuropil with prominent endothelial hyperpl...
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Dementia is defined as significant acquired cognitive impairment in one or more cognitive domains that represents a significant decline from previous baseline and interferes with independence in daily activities. Autoimmune dementia is a term that has been used to describe a steroid-responsive autoimmune disorder characterized by a rapidly progressive dementia with a fluctuating course. Even co...
متن کاملSubacute necrotizing encephalopathy in an acidotic child.
Subacute necrotizing encephalopathy was described originally by Leigh (1951). Further case reports reviewed by Ebels, Blokzijl, and Troelstra (1965) have confirmed this condition as a neuropathological entity but ideas concerning its aetiology have remained speculative. Recently Worsley, Brookfield, Elwood, Noble, and Taylor (1965) have reported a case, with confirmation at necropsy, in which t...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 42 225 شماره
صفحات -
تاریخ انتشار 1967