The peripheral hypoglossal nerves in 13 cases of amyotrophic lateral sclerosis (ALS) and five control cases were examined using morphometrical methods to demonstrate the degenerative process of motor nerve degeneration. The total number of myelinated fibers and their histograms were analyzed according to the degree of severity of the degeneration. Reduction of the total number of myelinated fibers in ALS hypoglossal nerves were graded in three groups: mild 65%–75%, moderate 50%–65% and severe 30%–50% of the myelinated fibers in controls. Each histogram of the remaining myelinated fibers showed different patterns corresponding to the degree of the degeneration and disclosed that the progressive reduction of large myelinated fibers was the fundamental change. Small myelinated fibers were not reduced, but increased, especially in the group with a moderate grade of degeneration. In plastic section, there were clusters of regenerated myelinated fibers. The transient increase of small myelinated fibers may be a reflexion of myelinated fiber regeneration during the progressive degenerative process of the motor neurons. The correlation between the degree of severity of the hypoglossal nerve degeneration and the atrophy of the tongue muscle and the duration of bulbar symptoms was examined and discussed.
This is a preview of subscription content, log in to check access.
Buy single article
Instant access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Bradley WG, Good P, Rassel CG, Adelman LS (1983) Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral screlosis. Ann Neurol 14:267–277
Davis JN, Thomas PK, Spolding JMK, Harrison SP (1975) Diseases of the 9th, 10th, 11th, and 12th cranial nerves. In: Dick PJ, Thomas PK, Lambert EH, Bunge R (eds) Peripheral neuropathy. W. B. Saunders, Philadelphia, pp 614–627
Hanyu N, Oguchi K, Yanagisawa N, Tsukagoshi H (1982) Degeneration and regeneration of ventral root motorfibers in amyotrophic lateral sclerosis. J Neurol Sci 55:99–115
Inoue K, Hirano A (1979) Early pathological changes of amyotrophic lateral sclerosis. Neurol Med (Tokyo) 11:448–455
Kawamura Y, Dyck PJ, Shimono M, Okazaki H, Tateishi J, Doi H (1981) Morphometric comparison of the vulneability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 40:667–675
Kurisaki H, Takatsu, Toyokura Y (1980) Hypoglossal nerve roots of amyotrophic lateral sclerosis. In: Research group of degenerative neurological disease. Annual reprot. Tokyo. Ministry of Health and Welfare, pp 65–70
Murakami N, Nakano I, Hirano A (1983) A neuropathological study of the hypoglossal nucleus in amyotrophic lateral sclerosis. Neurology (Tokyo) 23:131–139
Ohnishi Y, Makifuchi T, Ikuta F (1980) Morphometric investigation of the myelinated fibers of the anterior spinal roots in amyotrophic lateral sclerosis and Shy-Drager syndrome. Clin Neurol 20:809–815
Rexed B, Therman P (1948) Calibre spectra of motor and sensory nerve fibres to flexor and extensor muscles. J Neurophysiol 11:133–140
Sobue G, Matsuoka Y, Mukai E, takayanagi T, Sobue I, Hashizume Y (1981) Spinal and cranial motor nerve roots in amyotrophic lateral sclerosis and X-linked nessesive bulbospinal muscular atrophy. Acta Neuropathol (Berl) 55:227–235
Tsukagoshi H, Yanagisawa N, Oguchi K, Nagashima K, Murakami T (1979) Morphometric quantification of the cervical limb moter cells in controls and in amyotrophic lateral sclerosis. J Neurol Sci 41:287–297
Wohlfart G, Swank RL (1941) Pathology of amyotrophic lateral sclerosis. Arch Neurol Psychiatry 46:783–799
Wozniak W, Young PA (1969) Further observations on human hypoglossal nerve. Anat Anz [Suppl] 125:203–205
Supported by grant from the Ministry of Health and Welfare of Japan (Research project of progressive degenerative neurological disease)
About this article
Cite this article
Atsumi, T., Miyatake, T. Morphometry of the degenerative process in the hypoglossal nerves in amyotrophic lateral sclerosis. Acta Neuropathol 73, 25–31 (1987). https://doi.org/10.1007/BF00695498
- Amyotrophic lateral sclerosis
- Hypoglossal nerve
- Morphometrical analysis