Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy)

Eitoku Toyoshima, Andrew M Yeager, Susan Brennan, George W. Santos, Hugo W. Moser, Richard F. Mayer

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)307-318
Number of pages12
JournalJournal of the Neurological Sciences
Volume74
Issue number2-3
DOIs
StatePublished - 1986
Externally publishedYes

Fingerprint

Globoid Cell Leukodystrophy
Neural Conduction
Action Potentials
Muscles
Cell Transplantation
Tibial Nerve
Neurologic Manifestations
Inbred C57BL Mouse
Nerve Fibers
Ankle
Ether
Foot
Anesthesia
Animal Models
Parturition

Keywords

  • Conduction block
  • Demyelinating polyneuropathy
  • Hematopoetic cell transplantation
  • Murine globoid cell leukodystrophy
  • Nerve conduction studies

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

Cite this

Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy). / Toyoshima, Eitoku; Yeager, Andrew M; Brennan, Susan; Santos, George W.; Moser, Hugo W.; Mayer, Richard F.

In: Journal of the Neurological Sciences, Vol. 74, No. 2-3, 1986, p. 307-318.

Research output: Contribution to journalArticle

Toyoshima, Eitoku ; Yeager, Andrew M ; Brennan, Susan ; Santos, George W. ; Moser, Hugo W. ; Mayer, Richard F. / Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy). In: Journal of the Neurological Sciences. 1986 ; Vol. 74, No. 2-3. pp. 307-318.
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