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Serial electrophysiologic studies in rhesus monkeys with Krabbe disease
Author(s) -
Weimer Maria B.,
Gutierrez Amparo,
Baskin Gary B.,
Borda Juan T.,
Veazey Ronald S.,
Myers Leann,
PhillippiFalkenstein Kathrine M.,
Bunnell Bruce A.,
Ratterree Marion S.,
England John D.
Publication year - 2005
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20350
Subject(s) - nerve conduction velocity , krabbe disease , nerve conduction , medicine , peripheral nervous system , leukodystrophy , central nervous system , median nerve , peripheral neuropathy , pathology , biology , anatomy , disease , endocrinology , diabetes mellitus
Abstract Krabbe disease is a progressive leukodystrophy that results in demyelination in the central and peripheral nervous systems in humans. It has been described in a number of mammalian species including the rhesus monkey. We performed serial nerve conduction studies beginning within the first 2 months of life in four homozygous, two heterozygous, and two normal rhesus monkeys ( Macaca mulatta ) to characterize the peripheral neuropathy. Mean conduction velocities of the median, ulnar, and tibial nerves were significantly slower in the affected than unaffected monkeys at all ages ( P < 0.0001). The conduction velocity differences became more apparent between the affected and unaffected as the monkeys aged. When compared to the unaffected monkeys, the serial conduction velocities suggested occurrence of dysmyelination followed by demyelination in the affected monkeys. These observations provide further insight into the disease process and suggest an early window of opportunity for treating Krabbe disease. Muscle Nerve, 2005