Intellectual disability-coarse face-macrocephaly-cerebellar hypotrophy syndrome

Orpha code: 397709OMIM code: 616354

Definition

Intellectual disability-coarse face-macrocephaly-cerebellar hypotrophy syndrome is a rare, genetic, central nervous system malformation syndrome characterized by early-onset, progressive, severe cerebellar ataxia associated with progressive, moderate to severe intellectual disability, global developmental delay, progressively coarsening facial features, relative macrocephaly and absence of seizures. Sensorineural hearing loss may be associated. Neuroimaging reveals cerebellar atrophy/hypoplasia.

Disease data
Classification

Malformation syndrome

Synonyms
Autosomal recessive spinocerebellar ataxia type 20
Autosomalna recesywna ataksja rdzeniowo-móżdżkowa typu 20
SCAR20
Intellectual disability-coarse face-macrocephaly-cerebellar hypoplasia syndrome
SCAR20
ORPHA code
397709
OMIM code
616354
ICD10 code
Q87.8
ICD11 code
-

No additional description.

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