Pontocerebellar hypoplasia type 2

Orpha code: 2524OMIM code: 617026

Definition

A rare, genetic form of pontocerebellar hypoplasia characterized by pontocerebellar hypoplasia and progressive neocortical atrophy that manifests clinically with uncoordinated sucking and swallowing, and generalized clonus in the neonate. In early childhood, spasticity, chorea/dyskinesia, seizures and progressive microcephaly develop. Voluntary motor development is lacking.

Disease data
Classification

Malformation syndrome

Synonyms
PCH2
PCH2
ORPHA code
2524
OMIM code
617026
ICD10 code
Q04.3
ICD11 code
LD20.01

No additional description.

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