Leigh syndrome with nephrotic syndrome

Orpha code: 255249OMIM code: 614652

Definition

A rare, genetic neurometabolic disease characterized by encephalomyopathy (including developmental delay, nystagmus, progressive ataxia, dystonia, amyotrophy, visual loss, sensorineural deafness, seizures) and bilateral, symmetrical lesions in the basal ganglia or brainstem on imaging, associated with nephrotic syndrome.

Disease data
Classification

Disease

Synonyms
Infantile subacute necrotizing encephalopathy with nephrotic syndrome
Choroba Leigha z zespołem nerczycowym
Dziecięca podostra encefalopatia martwicza z zespołem nerczycowym
Leigh disease with nephrotic syndrome
ORPHA code
255249
OMIM code
614652
ICD10 code
G31.8
ICD11 code
5C53.24

No additional description.

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