Results 211 to 220 of about 117,949 (253)
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Clinical Pediatrics, 1995
Two children are described with pathology-proven Leigh disease. Rather than the typical degenerative course with loss of acquired development, they presented with a static encephalopathy manifested by seizures from birth and failure to acquire any milestones.
S B, Coker, C, Thomas
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Two children are described with pathology-proven Leigh disease. Rather than the typical degenerative course with loss of acquired development, they presented with a static encephalopathy manifested by seizures from birth and failure to acquire any milestones.
S B, Coker, C, Thomas
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PERIPHERAL NEUROPATHY IN LEIGH'S DISEASE
Brain, 1990Sural nerves were examined in 3 childhood cases of Leigh's disease (from 2 families), each with electrophysiological documentation of peripheral neuropathy. Postmortem confirmation of Leigh's disease was made in 2 cases; the third had characteristic CT scan appearances.
J M, Jacobs +4 more
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Cardiomyopathy associated with Leigh's disease
Virchows Archiv A Pathological Anatomy and Histopathology, 1985Clinical and postmortem findings in a female infant, suffering from Leigh's disease and cardiomegaly are described. The cardiac enlargement was due to symmetrical thickening of both ventricular walls and the septum. On light microscopy a widespread fibre disarray with a slight predilection for the ventricular septum was observed.
K, Langes +3 more
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Archives of Neurology, 1974
Leigh disease has distinct neuropathologic characteristics, but a poorly understood pathogenesis. All patients with this diagnosis proved at autopsy have had an inhibitor to the synthesis of thiamine triphosphate in their body fluids and reduced thiamine triphosphate concentration in their brains. To understand better the role of the inhibitor in Leigh
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Leigh disease has distinct neuropathologic characteristics, but a poorly understood pathogenesis. All patients with this diagnosis proved at autopsy have had an inhibitor to the synthesis of thiamine triphosphate in their body fluids and reduced thiamine triphosphate concentration in their brains. To understand better the role of the inhibitor in Leigh
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Subacute necrotizing encephalomyelopathy (Leigh's disease)
Pediatrics, 1977Subacute necrotizing encephalomyelopathy (SNE) was first described in 1951 by Denis Leigh.1 Since then, well over 100 cases have been described, and probably as many as 100 more have been diagnosed (at postmortem examination) and not recorded. With an increased awareness of the clinical features of this disease comes an increase in early diagnosis ...
D W, McCandless, W E, Hodgkin
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“Ragged‐red” fibers in Leigh's disease
Neurology, 1974A patient with chronic Leigh9s necrotizing encephalomyelopathy also had cardiomyopathy and a prominent “mitochondria1 myopathy.” It is speculated that (1) muscle often may be involved in Leigh9s disease and might serve as an important tissue for biochemical investigation, (2) certain “neurodegenerative disorders” known in the literature by other terms ...
T W, Crosby, S M, Chou
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Congenital Leigh's disease: Panencephalomyelopathy and peripheral neuropathy
Acta Neuropathologica, 1984A dystrophic newborn girl (38th week of gestation) presented as a floppy infant with relapsing episodes of lactic acidosis and progressive cerebral deterioration. She died after serious apnoea at the age of 8 weeks. Neuropathological examination demonstrated widespread changes of Leigh's subacute polioencephalomyelopathy affecting the cerebral cortex ...
R J, Seitz +4 more
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The “Double Panda” Sign in Leigh Disease
Journal of Child Neurology, 2013Although the “face of the giant panda” sign on magnetic resonance imaging (MRI) is traditionally considered to be characteristic of Wilson disease, it has also been reported in other metabolic disorders. This study describes the characteristic “giant panda” sign on MRI in a child with Leigh disease.
Kothari, Sonam +9 more
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2009
The disease subacute necrotizing encephalomyelopathy (SNE) was first described in 1951 by Denis Leigh and therefore is also called Leigh’s disease (Leigh, 1951). This disorder, although afflicting mainly children, has many features similar to those of Wernicke’s disease.
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The disease subacute necrotizing encephalomyelopathy (SNE) was first described in 1951 by Denis Leigh and therefore is also called Leigh’s disease (Leigh, 1951). This disorder, although afflicting mainly children, has many features similar to those of Wernicke’s disease.
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