Subacute necrotising encephalomyelopathy (Leigh's disease; Leigh syndrome)
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چکیده
منابع مشابه
Subacute necrotising encephalomyelopathy (Leigh’s disease; Leigh syndrome)
Like so many complex and incurable neurological disorders, the disease first described in this journal in 1951 by (Archibald) Denis Leigh (1915–1998; figure 1) as ‘Subacute Necrotising Encephalomyelopathy’ (SNE) remained for many years a rare and intriguing enigma, of interest mainly to paediatric neurologists and neuropathologists. The more recent history of this disorder, now designated Leigh...
متن کاملImpact commentaries. Subacute necrotising encephalomyelopathy (Leigh's disease; Leigh syndrome).
Like so many complex and incurable neurological disorders, the disease first described in this journal in 1951 by (Archibald) Denis Leigh (1915–1998; figure 1) as ‘Subacute Necrotising Encephalomyelopathy’ (SNE) remained for many years a rare and intriguing enigma, of interest mainly to paediatric neurologists and neuropathologists. The more recent history of this disorder, now designated Leigh...
متن کاملMR findings in patients with subacute necrotizing encephalomyelopathy (Leigh syndrome): correlation with biochemical defect.
MR studies were correlated with biochemical results in nine children who presented with lactic acidosis and/or abnormal MR findings in the basal ganglia. Neurologic development was delayed in all nine children. Seven of these patients were diagnosed as having subacute necrotizing encephalomyelopathy (SNE, or Leigh syndrome) on the basis of history, clinical findings, and biochemical studies; of...
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 disease (subacute necrotizing encephalomyelopathy): MR documentation of the evolution of an acute attack.
The radiologic evolution of Leigh disease is documented with sequential brain MR in the acute phase of the illness, at 3 weeks, and at 3 months. High-signal-intensity lesions seen on T2-weighted images in the first week resolved by 3 months, whereas new lesions appear during the chronic stage. Putamenal involvement is not a pathognomonic radiologic finding. Brain stem tegmentum, particularly th...
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ژورنال
عنوان ژورنال: Journal of Neurology, Neurosurgery & Psychiatry
سال: 2015
ISSN: 0022-3050,1468-330X
DOI: 10.1136/jnnp-2012-304601