Late-onset and fast progressive neuropathy and cardiomyopathy in Val32Ala transthyretin gene mutation

Neurol Sci. 2019 Jun;40(6):1267-1269. doi: 10.1007/s10072-019-3716-z. Epub 2019 Jan 26.

Abstract

More than 100 mutations of the transthyretin gene have been reported in autosomal dominant familial amyloid polyneuropathy. This rare disease causes severe motor and sensory disability, dysautonomia, and in some patients also cardiomyopathy. The diagnosis can be challenging mainly in sporadic adult patients showing clinical, laboratory, and neurophysiological findings overlapping other forms of chronic neuropathy. We describe the clinical features and course of a patient harboring the rare p.V32A (c.155T>C) variant that was previously described in only two patients and whose pathogenicity was unclear.

Keywords: Cardiomyopathy; Neuropathy; Transthyretin; Val32Ala.

MeSH terms

  • Amyloid Neuropathies, Familial / genetics*
  • Amyloid Neuropathies, Familial / pathology
  • Amyloid Neuropathies, Familial / physiopathology
  • Cardiomyopathies / genetics*
  • Cardiomyopathies / pathology*
  • Disease Progression
  • Humans
  • Male
  • Middle Aged
  • Mutation
  • Pedigree
  • Prealbumin / genetics*
  • Sural Nerve / pathology

Substances

  • Prealbumin
  • TTR protein, human

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related