Abstract Details

Title Bioimpedence analysis, hand grip and neurophysiology in hereditary transthyretin amyloidosis with polyneuropathy

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 408

Objective The aim of this study is to characterize the disease progression through bioimpedence analysis, hand grip and neurophysiological techniques in patients affected by hereditary transthyretin amyloidosis with polyneuropathy (ATTR-PN).

Background ATTR-PN is caused by mutations in the TTR gene, leading to misfolded monomers which aggregate generating amyloid fibrils. The clinical phenotype is characterized by a multisystemic disease affecting the sensorimotor, autonomic functions along with other organs. Hence, the disease progression might be assessed through neurophysiology which evaluates the progression of polyneuropathy, hand grip exploring the residual strength and bioimpedence analysis (BIA) which measures the constitution of tissues in specific parts of the body.

Design/Methods All patients were assessed with a standardized questionnaire on well-recognized symptoms of ATTR-PN, neurophysiological evaluation (ENG/EMG), hand grip and BIA. The data are presented as means and standard deviations. Pearson’s correlation has been carried out when appropriate. A linear regression model has been created to identify associated variables to disease's severity.

Results 16 patients with ATTR-PN (62.8±8.9 years; 165.0±12.5 cm; 74.9±14.9 kg) of which 7 women have been recruited. The data indicate that at increased pathology severity as indicated by FAP, increased presence of bilateral carpal tunnel syndrome and increased overall symptoms of neuropathy. A concomitant decrease of the CMAP amplitude of the median nerve was also observed along with hand grip strength. Also, when severity of disease was associated to BIA, we observed increased presence of weight loss with increased severity, which was in turn associated to a reduction only in lean muscle mass. Increased FAP was also strongly associated to a reduction of the phase angle.

Conclusions

The progression of bilateral carpal tunnel syndrome, polyneuropathy, lumbar stenosis and weight loss is related to the FAP grade in ATTR-PN. BIA, hand grip and neurophysiology can be useful tools for monitoring disease progression in ATTR-PN.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
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	No disclosure on file
Filippo Brighina, MD (University of Palermo)	Prof. Brighina has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Prof. Brighina has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TEVA. Prof. Brighina has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Lilly. Prof. Brighina has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alnylam Pharmaceuticals.

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