Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1992 May;55(5):394–397. doi: 10.1136/jnnp.55.5.394

Central motor and sensory conduction in X-linked recessive bulbospinal neuronopathy.

T Kachi 1, G Sobue 1, I Sobue 1
PMCID: PMC489083  PMID: 1318357

Abstract

Central conduction was studied in 12 patients with X-linked recessive bulbospinal neuronopathy (XBSN) using percutaneous electrical cortical, cervical and lumbar stimulation and somatosensory evoked potentials (SEPs). The central motor conduction time from the motor cortex to the cervical and lumbar segments of the spinal cord was normal in XBSN. SEPs, however, were abnormal or central sensory conduction time was prolonged in patients with XBSN. These results are consistent with the clinicopathological findings of XBSN in which the primary sensory neurons are involved as well as the lower motor neurons in the CNS, whereas the upper motor neurons are well preserved.

Full text

PDF
395

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Anziska B. J., Cracco R. Q. Short-latency somatosensory evoked potentials to median nerve stimulation in patients with diffuse neurologic disease. Neurology. 1983 Aug;33(8):989–993. doi: 10.1212/wnl.33.8.989. [DOI] [PubMed] [Google Scholar]
  2. Barker A. T., Jalinous R., Freeston I. L. Non-invasive magnetic stimulation of human motor cortex. Lancet. 1985 May 11;1(8437):1106–1107. doi: 10.1016/s0140-6736(85)92413-4. [DOI] [PubMed] [Google Scholar]
  3. Berardelli A., Inghilleri M., Formisano R., Accornero N., Manfredi M. Stimulation of motor tracts in motor neuron disease. J Neurol Neurosurg Psychiatry. 1987 Jun;50(6):732–737. doi: 10.1136/jnnp.50.6.732. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Berardelli A., Inghilleri M., Manfredi M., Zamponi A., Cecconi V., Dolce G. Cortical and cervical stimulation after hemispheric infarction. J Neurol Neurosurg Psychiatry. 1987 Jul;50(7):861–865. doi: 10.1136/jnnp.50.7.861. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Cosi V., Poloni M., Mazzini L., Callieco R. Somatosensory evoked potentials in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1984 Aug;47(8):857–861. doi: 10.1136/jnnp.47.8.857. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Cowan J. M., Rothwell J. C., Dick J. P., Thompson P. D., Day B. L., Marsden C. D. Abnormalities in central motor pathway conduction in multiple sclerosis. Lancet. 1984 Aug 11;2(8398):304–307. doi: 10.1016/s0140-6736(84)92683-7. [DOI] [PubMed] [Google Scholar]
  7. Harding A. E., Thomas P. K., Baraitser M., Bradbury P. G., Morgan-Hughes J. A., Ponsford J. R. X-linked recessive bulbospinal neuronopathy: a report of ten cases. J Neurol Neurosurg Psychiatry. 1982 Nov;45(11):1012–1019. doi: 10.1136/jnnp.45.11.1012. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Henley S., Pettit S., Todd-Pokropek A., Tupper A. Who goes home? Predictive factors in stroke recovery. J Neurol Neurosurg Psychiatry. 1985 Jan;48(1):1–6. doi: 10.1136/jnnp.48.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Ingram D. A., Swash M. Central motor conduction is abnormal in motor neuron disease. J Neurol Neurosurg Psychiatry. 1987 Feb;50(2):159–166. doi: 10.1136/jnnp.50.2.159. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Kennedy W. R., Alter M., Sung J. H. Progressive proximal spinal and bulbar muscular atrophy of late onset. A sex-linked recessive trait. Neurology. 1968 Jul;18(7):671–680. doi: 10.1212/wnl.18.7.671. [DOI] [PubMed] [Google Scholar]
  11. MAGEE K. R. Familial progressive bulbar-spinal muscular atrophy. Neurology. 1960 Mar;10:295–305. doi: 10.1212/wnl.10.3.295. [DOI] [PubMed] [Google Scholar]
  12. Maertens de Noordhout A., Rothwell J. C., Thompson P. D., Day B. L., Marsden C. D. Percutaneous electrical stimulation of lumbosacral roots in man. J Neurol Neurosurg Psychiatry. 1988 Feb;51(2):174–181. doi: 10.1136/jnnp.51.2.174. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Merton P. A., Hill D. K., Morton H. B., Marsden C. D. Scope of a technique for electrical stimulation of human brain, spinal cord, and muscle. Lancet. 1982 Sep 11;2(8298):597–600. doi: 10.1016/s0140-6736(82)90670-5. [DOI] [PubMed] [Google Scholar]
  14. Merton P. A., Morton H. B. Stimulation of the cerebral cortex in the intact human subject. Nature. 1980 May 22;285(5762):227–227. doi: 10.1038/285227a0. [DOI] [PubMed] [Google Scholar]
  15. Mills K. R., Murray N. M. Electrical stimulation over the human vertebral column: which neural elements are excited? Electroencephalogr Clin Neurophysiol. 1986 Jun;63(6):582–589. doi: 10.1016/0013-4694(86)90145-8. [DOI] [PubMed] [Google Scholar]
  16. Rothwell J. C., Thompson P. D., Day B. L., Dick J. P., Kachi T., Cowan J. M., Marsden C. D. Motor cortex stimulation in intact man. 1. General characteristics of EMG responses in different muscles. Brain. 1987 Oct;110(Pt 5):1173–1190. doi: 10.1093/brain/110.5.1173. [DOI] [PubMed] [Google Scholar]
  17. Schmid U. D., Walker G., Hess C. W., Schmid J. Magnetic and electrical stimulation of cervical motor roots: technique, site and mechanisms of excitation. J Neurol Neurosurg Psychiatry. 1990 Sep;53(9):770–777. doi: 10.1136/jnnp.53.9.770. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Sobue G., Hashizume Y., Mitsuma T., Takahashi A. Size-dependent myelinated fiber loss in the corticospinal tract in Shy-Drager syndrome and amyotrophic lateral sclerosis. Neurology. 1987 Mar;37(3):529–532. doi: 10.1212/wnl.37.3.529. [DOI] [PubMed] [Google Scholar]
  19. Sobue G., Hashizume Y., Mukai E., Hirayama M., Mitsuma T., Takahashi A. X-linked recessive bulbospinal neuronopathy. A clinicopathological study. Brain. 1989 Feb;112(Pt 1):209–232. doi: 10.1093/brain/112.1.209. [DOI] [PubMed] [Google Scholar]
  20. Thompson P. D., Day B. L., Rothwell J. C., Dick J. P., Cowan J. M., Asselman P., Griffin G. B., Sheehy M. P., Marsden C. D. The interpretation of electromyographic responses to electrical stimulation of the motor cortex in diseases of the upper motor neurone. J Neurol Sci. 1987 Aug;80(1):91–110. doi: 10.1016/0022-510x(87)90224-3. [DOI] [PubMed] [Google Scholar]
  21. Thompson P. D., Dick J. P., Day B. L., Rothwell J. C., Berardelli A., Kachi T., Marsden C. D. Electrophysiology of the corticomotoneurone pathways in patients with movement disorders. Mov Disord. 1986;1(2):113–117. doi: 10.1002/mds.870010205. [DOI] [PubMed] [Google Scholar]
  22. Wilde J., Moss T., Thrush D. X-linked bulbo-spinal neuronopathy: a family study of three patients. J Neurol Neurosurg Psychiatry. 1987 Mar;50(3):279–284. doi: 10.1136/jnnp.50.3.279. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES