2024-03-29T15:13:38Z
https://soar-ir.repo.nii.ac.jp/oai
oai:soar-ir.repo.nii.ac.jp:00008050
2022-12-14T04:00:36Z
882:883
Electrophysiological features of familial amyloid polyneuropathy in endemic area
Kodaira, Minori
Morita, Hiroshi
Shimojima, Yoshio
Ikeda, Shu-ichi
Familial amyloid polyneuropathy
peripheral nerve dysfunction
amyloid
transthyretin
nerve conduction study
The process of deterioration of peripheral nerve function in familial amyloid polyneuropathy (FAP) with amyloidogenic transthyretin (ATTR) Val30Met has not been systematically evaluated hitherto. We performed nerve conduction studies in 69 patients with FAP with ATTR Val30Met from one of the endemic areas in Japan. Sensory conduction velocity (SCV), motor conduction velocity (MCV), the size of the compound muscle action potential (CMAP) and distal latency (DL) were measured in the ulnar and tibial nerves. SCV was evaluated using the orthodromic method with needle recording electrodes. These electrophysiological parameters were compared with clinical stage of FAP and duration of neuropathy. When subjects noted minimal neuropathic symptoms only in the feet, motor and sensory nerve function in both the hands and feet had already been disturbed. Sensory nerve action potential on the foot disappeared more rapidly than CMAP. CMAP on foot muscle rapidly decreased during the initial 2 years and completely disappeared within 10 years. The duration of illness and deterioration parameters (CMAP of the abductor digiti minimi muscle, MCV and SCV of the ulnar nerve and DL of both ulnar and tibial nerves) were linearly correlated. CMAP was the most sensitive and reliable parameter to evaluate motor nerve degeneration in FAP.</.
Article
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS. 18(1):10-18 (2011)
journal article
INFORMA HEALTHCARE
2011-05
application/pdf
AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS
1
18
10
18
1350-6129
AA11096426
https://soar-ir.repo.nii.ac.jp/record/8050/files/Electrophysiological_features_familial_amyloid_polyneuropathy.pdf
eng
21261557
https://pubmed.ncbi.nlm.nih.gov/21261557
10.3109/13506129.2010.548424
https://doi.org/10.3109/13506129.2010.548424
Copyright© 2011 Informa UK, Ltd.