Conditions / Nervous system

pontocerebellar hypoplasia type 7

info · Nervous system · ICD-10: Q04.3

A pontocerebellar hypoplasia that is characterized by delayed psychomotor development, hypotonia, gonadal abnormalities and respiratory failure, has_material_basis_in autosomal recessive mutation in the TOE1 gene.

Signs and symptoms

  • Hypoplasia of the pons
  • Muscle weakness
  • Intellectual disability
  • Skeletal muscle atrophy
  • Feeding difficulties
  • Global developmental delay
  • Hypoplasia of the brainstem
  • Progressive microcephaly
  • Ambiguous genitalia
  • Hyperreflexia