Peripheral nerve conduction in fabry disease

Annals of Neurology - Tập 7 Số 4 - Trang 319-323 - 1980
Kumudchandra J. Sheth1, Herbert M. Swick1
1Departments of Pediatrics and Neurology, The Medical College of Wisconsin and Milwaukee Children's Hospital, Milwaukee, WI

Tóm tắt

AbstractPeripheral nerve conduction was studied in 34 members, aged 3 to 54 years, from three separate kindreds with Fabry disease. Nerve conduction velocity was prolonged in one‐third (11 of 34) of the study group. Of the 9 control family members with normal plasma α‐galactosidase levels, 8 had normal velocity. A 6‐year‐old girl with normal plasma and leukocyte α‐galactosidase levels and absent corneal deposits had slightly lower than normal nerve conduction velocity. One of the 3 obligate female carriers and 1 of 10 suspected carriers of Fabry disease had prolonged conduction velocity but normal distal latency. On the other hand, 8 of 12 males affected with Fabry disease demonstrated slow nerve conduction velocity while 4 of the 8 had prolonged distal latency. Frequently, only one of the two nerves studied showed abnormalities. These abnormalities were not related to patient age. Interfamilial variations in the abnormalities were present.

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Tài liệu tham khảo

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Thông tin xuất bản

Annals of Neurology

Tập 7 Số 4

319-323

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