Abstract
Subacute necrotizing encephalomyopathy, first reported by Leigh more than 30 years ago, is an autosomal recessive disorder characterized by multiple symmetrical foci of incomplete necrosis (spongy degeneration) in brainstem, spinal cord, basal ganglia and cerebellum. The clinical picture is heterogeneous, depending on the variability of lesions, but usually it consists of disorders of respiratory rhythm, nuclear and supranuclear oculomotor paralysis, other signs of cranial nerve dysfunction, abnormal movements, ataxia and optic atrophy.
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