Conditions / Cardiovascular

hereditary systemic amyloidosis 1

info · Cardiovascular · ICD-10: E85.82

An amyloidosis that is characterized by a loss of sensation in the extremities, cardiomyopathy, nephropathy, vitreous opacities, and CNS amyloidosis resulting from abnormal deposits of amyloid protein in the body's organs and tissues and has_material_basis_in

An amyloidosis that is characterized by a loss of sensation in the extremities, cardiomyopathy, nephropathy, vitreous opacities, and CNS amyloidosis resulting from abnormal deposits of amyloid protein in the body's organs and tissues and has_material_basis_in autosomal dominant inheritance of mutations in the TTR gene.

Signs and symptoms

  • Amyloid deposition
  • Increased CSF protein concentration
  • Peripheral axonal neuropathy
  • Hearing impairment
  • Seizure
  • Ataxia
  • Nystagmus
  • Amyloid deposition in the vitreous humor
  • Muscle weakness
  • Constipation

Also known as: ATTR amyloidosis; ATTRm amyloidosis; Amyloidosis, hereditary, transthyretin-related; Corino de Andrade's disease; Familial transthyretin amyloidosis