Conditions / Nervous system

SPOAN syndrome

info · Nervous system · ICD-10: G11.4

A neurodegenerative disease characterized by spastic paraplegia, axonal neuropathy, dysarthria, acoustic startle, and congenital optical atrophy and that has_material_basis_in homozygous mutation in the KLC2 gene on chromosome 11q13.2.

Signs and symptoms

  • Exaggerated startle response
  • Optic atrophy
  • Distal amyotrophy
  • Dysarthria
  • Proximal hyperreflexia
  • Nystagmus
  • Hyperhidrosis
  • Scoliosis
  • Kyphosis
  • Absent Achilles reflex

Also known as: spastic paraplegia, optic atropy, and neuropathy; spastic paraplegia, optic atropy, and neuropathy syndrome