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Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathy

Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met... We report here two patients with amyloidogenic transthyretin (ATTR) Val30Met familial amyloid polyneuropathy (FAP) who developed numbness in both hands and were diagnosed as having bilateral carpal tunnel syndrome (CTS). In both patients systemic TTR amyloidosis consisting of polyneuropathy affecting both upper and lower limbs and/or autonomic dysfunction gradually appeared after surgery for CTS. Although CTS associated with TTR amyloidosis has been known as an initial symptom in some patients with ATTR non-Val30Met FAP and those with senile systemic amyloidosis, this is the first report of ATTR Val30Met FAP patients starting with upper limb neuropathy including CTS-like symptoms. It is also notable that both patients had no genealogical relationship with two Japanese endemic foci of this disease. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Amyloid: Journal of Protein Folding Disorders Taylor & Francis

Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathy

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References (27)

Publisher
Taylor & Francis
Copyright
© 2010 Informa UK Ltd
ISSN
1744-2818
eISSN
1350-6129
DOI
10.3109/13506121003619369
pmid
20132088
Publisher site
See Article on Publisher Site

Abstract

We report here two patients with amyloidogenic transthyretin (ATTR) Val30Met familial amyloid polyneuropathy (FAP) who developed numbness in both hands and were diagnosed as having bilateral carpal tunnel syndrome (CTS). In both patients systemic TTR amyloidosis consisting of polyneuropathy affecting both upper and lower limbs and/or autonomic dysfunction gradually appeared after surgery for CTS. Although CTS associated with TTR amyloidosis has been known as an initial symptom in some patients with ATTR non-Val30Met FAP and those with senile systemic amyloidosis, this is the first report of ATTR Val30Met FAP patients starting with upper limb neuropathy including CTS-like symptoms. It is also notable that both patients had no genealogical relationship with two Japanese endemic foci of this disease.

Journal

Amyloid: Journal of Protein Folding DisordersTaylor & Francis

Published: Mar 1, 2010

Keywords: Carpal tunnel syndrome; familial amyloid polyneuropathy; Val30Met

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