TY - JOUR
T1 - Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy)
AU - Toyoshima, Eitoku
AU - Yeager, Andrew M.
AU - Brennan, Susan
AU - Santos, George W.
AU - Moser, Hugo W.
AU - Mayer, Richard F.
N1 - Funding Information:
Presented at the 37th Annual Meeting of the American Academy of Neurology, 30 April, 1985, Dallas, TX. Supported in part by the Veterans Administration Research Service, Grant Nos. HD00535 (A.M.Y.), CA 15396 (G.W.S.) and NS 135 l 3 (H.W.M.) from the National Institutes of Health, and by Basil O'Connor Starter Research Grant No. 5-485 from the March of Dimes Birth Defects Foundation (A.M.Y.) Address correspondence to: Richard F. Mayer, M.D., Department of Neurology, Room N5W68, University of Maryland Hospital, 22 South Greene Street, Baltimore, MD 21201, U.S.A.
PY - 1986/7
Y1 - 1986/7
N2 - Progression of the neuropathy in the Twitcher mouse (twi-C57BL/6J), an animal model of globoid cell leukodystrophy, was assessed with serial motor nerve conduction studies from just after birth until near death (day 45) and after hematopoietic cell transplantation (HCT). Under ether anesthesia, the tibial nerve was stimulated percutaneously at the sacral notch and at the ankle, and recordings were made from plantar foot muscles. Motor conduction velocity (MCV), distal latency, amplitude, duration and number of phases of compound muscle action potentials on proximal (pCMAP) and distal (dCMAP) stimulation were measured. In 15-19 day-old Twitcher, despite the absence of motor signs, MCV was significantly reduced, 12.8 ± 2.8 (10) m/s (M ± SD, No. of recordings), compared with unaffected siblings, 18.1 ± 2.6 (21) m/s (P < 0.01). The ratio of pCMAP to dCMAP amplitudes was reduced in the Twitcher, 0.39 ± 0.13 (10), compared with controls 0.72 ± 0.17 (21) and the ratio of pCMAP to dCMAP phases was increased (2.8 ± 0.8 (10) vs 1.0 ± 0.2 (21), P < 0.01 for all). As neurologic signs progressed by 35-39 days, MCV became slower, 5.8 ± 1.0 (11) m/s, pCMAP and dCMAP became smaller, but the ratio of pCMAP to dCMAP amplitudes in the Twitcher (0.55 ± 0.36, 11) was similar to controls (0.71 ± 1.0, 20) as was the ratio of pCMAP to dCMAP phases (1.0 ± 0.4 vs 1.0 ± 0.1). These results suggest that there is diffuse non-uniform slowing of nerve conduction with block especially in proximal nerve fibers initially. With HCT, mean MCV remained slow (6.7 ± 1.2 (18) m/s, vs 34.5 ± 3.9 (12) m/s) but motor function persisted.
AB - Progression of the neuropathy in the Twitcher mouse (twi-C57BL/6J), an animal model of globoid cell leukodystrophy, was assessed with serial motor nerve conduction studies from just after birth until near death (day 45) and after hematopoietic cell transplantation (HCT). Under ether anesthesia, the tibial nerve was stimulated percutaneously at the sacral notch and at the ankle, and recordings were made from plantar foot muscles. Motor conduction velocity (MCV), distal latency, amplitude, duration and number of phases of compound muscle action potentials on proximal (pCMAP) and distal (dCMAP) stimulation were measured. In 15-19 day-old Twitcher, despite the absence of motor signs, MCV was significantly reduced, 12.8 ± 2.8 (10) m/s (M ± SD, No. of recordings), compared with unaffected siblings, 18.1 ± 2.6 (21) m/s (P < 0.01). The ratio of pCMAP to dCMAP amplitudes was reduced in the Twitcher, 0.39 ± 0.13 (10), compared with controls 0.72 ± 0.17 (21) and the ratio of pCMAP to dCMAP phases was increased (2.8 ± 0.8 (10) vs 1.0 ± 0.2 (21), P < 0.01 for all). As neurologic signs progressed by 35-39 days, MCV became slower, 5.8 ± 1.0 (11) m/s, pCMAP and dCMAP became smaller, but the ratio of pCMAP to dCMAP amplitudes in the Twitcher (0.55 ± 0.36, 11) was similar to controls (0.71 ± 1.0, 20) as was the ratio of pCMAP to dCMAP phases (1.0 ± 0.4 vs 1.0 ± 0.1). These results suggest that there is diffuse non-uniform slowing of nerve conduction with block especially in proximal nerve fibers initially. With HCT, mean MCV remained slow (6.7 ± 1.2 (18) m/s, vs 34.5 ± 3.9 (12) m/s) but motor function persisted.
KW - Conduction block
KW - Demyelinating polyneuropathy
KW - Hematopoetic cell transplantation
KW - Murine globoid cell leukodystrophy
KW - Nerve conduction studies
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U2 - 10.1016/0022-510X(86)90116-4
DO - 10.1016/0022-510X(86)90116-4
M3 - Article
C2 - 3525759
AN - SCOPUS:0022618928
SN - 0022-510X
VL - 74
SP - 307
EP - 318
JO - Journal of the Neurological Sciences
JF - Journal of the Neurological Sciences
IS - 2-3
ER -