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Гіпоплазія мозочка, тип 1

ORPHA:2254· ICD-10 Q04.3· Pontocerebellar hypoplasia type 1

Визначення(English summary)

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

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Neonatal