Severe neonatal-onset encephalopathy with microcephaly

Orpha code: 209370OMIM code: 300673

Definition

Severe neonatal-onset encephalopathy with microcephaly is a rare monogenic disease with epilepsy characterized by neonatal-onset encephalopathy, microcephaly, severe developmental delay or absent development, breathing abnormalities (including central hypoventilation and/or respiratory insufficiency), intractable seizures, abnormal muscle tone and involuntary movements. Early death is usual.

Disease data
Classification

Disease

Synonyms
Severe congenital encephalopathy due to MECP2 mutation
Ciężka wrodzona encefalopatia z powodu mutacji MECP2
ORPHA code
209370
OMIM code
300673
ICD10 code
Q02
ICD11 code
LD20.2

No additional description.

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