A middle age Portuguese woman was investigated for renal amyloidosis. She 
presented with progressive renal failure, proteinuria, hypertension, and sensory 
symptoms in the feet. Clinical and neurophysiological evaluation disclosed 
sensory-autonomic neuropathy. Cardiovascular tests and 123-MIBG investigation 
showed parasympathetic dysfunction and decrease of myocardial innervation, in 
accordance with small fiber neuropathy, as usually observed in amyloidosis. 
Immunohistochemical studies revealed AFib amyloidosis and genetic studies the 
amino acid exchange Glu526Val of the fibrinogen Aalpha-chain mutation, which was 
also present in one of her sons. The mutant gene in this patient was associated 
with the same haplotype as all other reported cases of Glu526Val mutations. This 
is the first reported AFibamyloidosis in Portugal, and the first case of AFib in 
which sensory and autonomic nerve fiber dysfunction is described, indicating 
that small nerve fiber lesion can occur in the fibrinogen Aalpha chain mutation. 
This can be important for prognosis, in particular when liver transplantation is 
considered for treatment.