Conditions / Genetic

homocystinuria

info · Genetic · ICD-10: E72.11

An amino acid metabolic disorder that involves an accumulation of homocysteine in the serum and an increased excretion of homocysteine in the urine.

Signs and symptoms

  • Hypermethioninemia
  • Ataxia
  • Intellectual disability
  • Dysarthria
  • Flushing
  • Hyperhomocystinemia
  • Brain atrophy
  • Unsteady gait
  • Exotropia
  • Disproportionate tall stature

Medications that may treat it

betaine

Also known as: CBS deficiency; cystathionine beta synthase deficiency; cystathionine synthase deficiency