Pontocerebellar hypoplasia type 1

Orpha code: 2254OMIM code: 616081

Definition

A severe, genetic form of pontocerebellar hypoplasia (PCH) characterized by spinal cord anterior horn cell degeneration in addition to pontocerebellar hypoplasia. Clinically, patients manifest with a severe global development deficit that is evident early on from difficulties in feeding and swallowing

Disease data
Classification

Malformation syndrome

Synonyms
Norman disease
Choroba Normana
PCH1
PCH1
ORPHA code
2254
OMIM code
616081
ICD10 code
Q04.3
ICD11 code
LD20.01

No additional description.

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