Microcephalic primordial dwarfism due to ZNF335 deficiency

Orpha code: 329228OMIM code: 615095

Definition

Microcephalic primordial dwarfism due to ZNF335 deficiency is characterized by severe antenatal microencephaly, simplified gyration, agenesis of the corpus callosum, absence of basal ganglia (very rare), pontocerebellar atrophy and involvement of the white matter with secondary cerebral atrophy. Congenital cataract, choanal atresia, multiple arthrogryposis and spastic tetraparesis can occur.

Disease data
Classification

Malformation syndrome

Synonyms
Microcephalic primordial dwarfism, Walsh type
Pierwotna karłowatość mikrocefaliczna, typ Walsha
ORPHA code
329228
OMIM code
615095
ICD10 code
Q87.1
ICD11 code
-

No additional description.

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