Isolated cerebellar agenesis

Orpha code: 1398OMIM code:

Definition

A rare non-syndromic central nervous system malformation characterized by complete or near-complete absence of the cerebellum with a normal sized posterior fossa, possibly accompanied by hypoplasia of the brainstem. The clinical picture is highly variable, but typically includes ataxia, dysarthria, tremor, dysmetria, dysdiadochokinesia, and oculomotor abnormalities, in addition to impaired mental, motor, and language development and intellectual disability.

Disease data
Classification

Morphological anomaly

Synonyms
Near total absence of cerebellum
Subtotal absence of cerebellum
ORPHA code
1398
OMIM code
-
ICD10 code
Q04.3
ICD11 code
-

No additional description.

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