Conditions / Genetic

pontocerebellar hypoplasia type 2D

info · Genetic · ICD-10: Q04.3

A pontocerebellar hypoplasia that is characterized by progressive microcephaly, profound intellectual disability, spasticity and seizure, has_material_basis_in autosomal recessive inheritance of mutation in the SEPSECS gene.

Signs and symptoms

  • Seizure
  • Global developmental delay
  • Appendicular spasticity
  • Paroxysmal tonic upgaze
  • Cerebellar vermis atrophy
  • Progressive microcephaly
  • Spastic tetraplegia
  • Clonus
  • Cerebral atrophy
  • Hypoplasia of the corpus callosum