Early-onset epilepsy-intellectual disability-brain anomalies syndrome

Orpha code: 488635OMIM code: 616917

Definition

A rare congenital disorder of glycosylation characterized by early onset of hypotonia, severe global developmental delay, intellectual disability, and seizures. Ataxia, mild facial dysmorphism, and autistic behavior have also been reported. Brain MRI findings are variable and include cerebral atrophy, cerebellar hypoplasia/atrophy, and thin corpus callosum.

Disease data
Classification

Disease

Synonyms
Congenital disorder of glycosylation due to PIGG deficiency
Wrodzone zaburzenie glikolizacji z powodu niedoboru PIGG
PIGG-CDG
ORPHA code
488635
OMIM code
616917
ICD10 code
E77.8
ICD11 code
-

No additional description.

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