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Definition

An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia.

Acronym

LS

Synonyms

Leigh Disease
Leigh syndrome due to mitochondrial complex I deficiency
Leigh syndrome due to mitochondrial complex II deficiency
Leigh syndrome due to mitochondrial complex III deficiency
Leigh syndrome due to mitochondrial complex IV deficiency
Leigh syndrome due to mitochondrial complex V deficiency
Necrotizing encephalopathy infantile subacute of Leigh
SNE

Keywords

Leigh syndrome

 
Disclaimer

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