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Tongue atrophy and fasciculations in transthyretin familial amyloid neuropathy An ALS mimicker Objective: Macroglossia is a well-known feature of amyloidosis; however, tongue atrophy and fas-ciculations 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 fasci-culations in the setting of a severe neuropathy. Results: Both patients were confirmed to have transthyretin-related familial amyloid polyneurop-athy (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 mimick-ers of ALS include polyglucosan body disease, hexosaminidase A deficiency, multisystem proteinop-athy, and Allgrove syndrome. Neurol Genet 2015;1:e18; doi: 10.1212/NXG.18 GLOSSAR