Electrophysiological features of familial amyloid polyneuropathy in endemic area
| Autor: | Shu-ichi Ikeda, Hiroshi Morita, Yoshio Shimojima, Minori Kodaira |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Amyloid Endemic Diseases Neural Conduction Action Potentials Sensory system nerve conduction study transthyretin Nerve conduction velocity Young Adult Sex Factors Japan Internal Medicine medicine Humans Prealbumin Ulnar Nerve Amyloid Neuropathies Familial medicine.diagnostic_test biology business.industry Anatomy Middle Aged Compound muscle action potential body regions Electrophysiology Transthyretin medicine.anatomical_structure Familial amyloid polyneuropathy peripheral nerve dysfunction Nerve conduction study biology.protein Female Tibial Nerve business Orthodromic Sensory nerve |
| Zdroj: | Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis. 18(1) |
| ISSN: | 1744-2818 |
| Popis: | 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) |
| Databáze: | OpenAIRE |
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