title: Tongue atrophy and fasciculations in transthyretin familial amyloid neuropathy: An ALS mimicker.
abstract: OBJECTIVE
  Macroglossia is a well-known feature of amyloidosis; however, tongue atrophy and fasciculations are rarely seen and can lead to the misdiagnosis of amyotrophic lateral sclerosis (ALS).
  
  
  METHODS
  We identified 2 unrelated patients with atypical features of tongue atrophy and fasciculations in the setting of a severe neuropathy.
  
  
  RESULTS
  Both patients were confirmed to have transthyretin-related familial amyloid polyneuropathy (TTR-FAP) by genetic testing.
  
  
  CONCLUSIONS
  TTR-FAP should be considered as a possible mimicker of ALS when tongue atrophy and fasciculations are seen in the setting of a severely progressive polyneuropathy. Other atypical mimickers of ALS include polyglucosan body disease, hexosaminidase A deficiency, multisystem proteinopathy, and Allgrove syndrome.
authors.affiliation: Department of Neurology (N.A.G., T.M.) and Department of Orthopaedic Surgery (T.M.), University of California, Irvine.
authors.forename: Namita A
authors.initials: NA
authors.lastname: Goyal
authors.affiliation: Department of Neurology (N.A.G., T.M.) and Department of Orthopaedic Surgery (T.M.), University of California, Irvine.
authors.forename: Tahseen
authors.initials: T
authors.lastname: Mozaffar