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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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Journal of Computer Assisted Tomography, 1989
Three cases of Leigh disease (subacute necrotizing encephalomyelopathy) have been investigated recently in our institute by CT. Bilateral, low attenuation areas were observed in the basal ganglia in all cases. These areas corresponded to the typical necrosis areas seen pathologically in this disease.
A, Taccone +3 more
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Three cases of Leigh disease (subacute necrotizing encephalomyelopathy) have been investigated recently in our institute by CT. Bilateral, low attenuation areas were observed in the basal ganglia in all cases. These areas corresponded to the typical necrosis areas seen pathologically in this disease.
A, Taccone +3 more
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Leigh syndrome: an adult presentation of a paediatric disease
Practical Neurology, 2023A previously healthy 27-year-old man was admitted to the acute neurology ward with events involving his face, throat and upper limb, which video telemetry later confirmed were refractory focal seizures. He also had progressive pyramidal features, dysarthria and ataxia.
Taylor Watson-Fargie +11 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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Bull’s-Eye Maculopathy in an Infant With Leigh Disease
American Journal of Ophthalmology, 2006To report a bull's-eye maculopathy-like fundus abnormality in an infant with Leigh disease.Observational case report.We reviewed the medical, ophthalmic, and genetic records of an eight-month-old boy who presented with hypotonia and bilaterally decreased vision.The ophthalmic examination revealed poor fixation, marked hyperopia, attenuated retinal ...
Philip W, Laird +2 more
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Diagnostic clues in an adult case of Leigh's disease
Medical Journal of Australia, 1988A previously-well 32-year-old woman presented with episodes of severe headache, neck pain, numbness of the tongue and facial sweating. In the course of her illness she became abulic, blind and quadriparetic and she suffered recurrent episodes of coma during which she became decerebrate and her brain-stem reflexes were absent.
M, Fulham, C, Lawrence, C, Harper
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Leigh's Disease Associated With a Dorsal Midbrain Syndrome
Journal of Pediatric Ophthalmology & Strabismus, 2009Leigh's disease is a rare progressive neurodegenerative condition caused by a mitochondrial cytopathy. The authors present the case of a 9-year-old girl with dorsal midbrain syndrome causing convergence retraction nystagmus. Magnetic resonance imaging, skin biopsy, and genetic testing confirmed the cause to be Leigh's disease due to two
Stephanie K, West +3 more
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Efficacy of Recommended Therapeutic Regimens in Leigh's Disease
Developmental Medicine & Child Neurology, 1974SUMMARYAn infant, diagnosed as having subacute necrotizing encephalopathy (Leigh's disease), was treated with a combination of recommended therapies, but there was no significant improvement and the child died at the age of 11 months. Autopsy supported the diagnosis. Hepatic pyruvate carboxyiase activity had been normal during the course of the disease.
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2014
The mitochondrial cytopathies in children are clinically and genetically heterogeneous group of disorders leading to involvement of nervous system and other organ systems. Subacute necrotizing encephalomyelopathy, called Leigh Disease is a type of these disorders.
SEREN, Lale Pulat +3 more
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The mitochondrial cytopathies in children are clinically and genetically heterogeneous group of disorders leading to involvement of nervous system and other organ systems. Subacute necrotizing encephalomyelopathy, called Leigh Disease is a type of these disorders.
SEREN, Lale Pulat +3 more
openaire +1 more source