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NEUROPHYSIOLOGICAL STUDIES IN HEREDITARY AMYLOIDOSIS WITH POLYNEUROPATHY
Author(s) -
Andersson Rune,
Blom Sigfrid
Publication year - 1972
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1111/j.1365-2796.1972.tb00044.x
Subject(s) - polyneuropathy , medicine , amyloidosis , denervation , electromyography , biopsy , physical examination , nerve biopsy , nerve conduction study , pathology , peripheral neuropathy , nerve conduction , surgery , anatomy , physical medicine and rehabilitation , endocrinology , diabetes mellitus
Sixteen cases with histologically verified amyloidosis with polyneuropathy have been investigated electro‐myographically (EMG) and motor conduction velocity (MCV) has been recorded. Eleven cases were familial and five sporadic. Clinically the polyneuropathy was pronounced in nine cases, moderate in six, and slight in one case. The EMG changes correlated well with the clinical findings and with the duration of symptoms, and were more marked in the long‐standing cases. The MCV was abnormal, but correlated less well with the clinical pattern. The denervation was most obvious in the distal parts of the lower extremities. EMG of the short toe extensor muscles was found to be an especially useful examination. The findings in familial and sporadic cases were similar. The same examinations were performed on nine members of one afflicted family. Examinations of biopsy specimens of the skin did not reveal amyloid deposits. In two of the nine cases no clinical or neuropnysiological findings of peripheral polyneuropathy were revealed. In seven cases clinical symptoms and/or signs of polyneuropathy were observed. They were, however, discrete. Three of these seven cases showed neurophysiological changes. In four patients no conclusive neurophysiological abnormalities were found.