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Sensory Neuron Degeneration in Familial Kugelberg-Welander Disease

Published online by Cambridge University Press:  18 September 2015

Toni R. Winder  and
Roland N. Auer
Toni R. Winder
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary, Calgary
Roland N. Auer*
Affiliation:
Departments of Pathology and Clinical Neurosciences, University of Calgary, Calgary
*
Neuropathology, Health Sciences Center, 3330 Hospital Dr. N.W., Calgary, Alberta, Canada T2N 4N1
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Abstract:

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A 53 year old man developed symptoms of motor neuron disease in childhood. There was a family history of a similar disorder and it was felt to represent a form of Kugelberg-Welander disease. In addition to the motor deficits, sensory abnormalities in his legs were documented during life. Autopsy revealed anterior horn cell loss throughout the length of the spinal cord, with preservation of the phrenic nucleus. The lumbar dorsal root ganglia showed active degeneration of sensory neurons, with nuclear changes exceeding cytoplasmic ones. The fasciculus gracilis showed Wallerian degeneration. The findings provide direct evidence that sensory neurons can degenerate in some forms of motor neuron disease, and that the “demyelination” or “degeneration” of posterior columns sometimes seen in the various forms of motor neuron disease may actually be secondary to cell body disease in the dorsal root ganglia.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 1 , February 1989 , pp. 67 - 70
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

References

REFERENCES

1.Luthy, F, Martin, F. Observation de case de sclérose latérale amyotrophique avec considération particulure sur la participation sensitive. Schweiz Med Wchnschr 1947; 25: 694–697.Google Scholar
2.Lawyer, T, Netsky, MD. ALS: a clinicoanatomic study of 53 cases. Arch Neurol Psychiat (Chic) 1953; 69: 171–192.CrossRefGoogle Scholar
3.Kawamura, Y, Dyck, PJ, Shimono, M, et al. Morphometric comparison of the vulnerability of peripheral motor and sensory neurons in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 1981; 40: 667–675.CrossRefGoogle ScholarPubMed
4.Tandan, R, Bradley, WG. Amyotrophic lateral sclerosis: part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol 1985; 18: 271–280.CrossRefGoogle ScholarPubMed
5.Kuzuhara, S, Chou, SM. Preservation of the phrenic motoneurons in Werdnig-Hoffmann disease. Ann Neurol 1981; 9: 506–510.CrossRefGoogle ScholarPubMed
6.Dyck, PJ, Lambert, EH. Lower motor and primary sensory neuron diseases with peroneal muscular atrophy. II. Neurologic, genetic, and electrophysiologic findings in various neuronal degenerations. Arch Neurol 1968; 18: 619–625.CrossRefGoogle ScholarPubMed
7.Hughes, JT, Brownell, B. Pathology of peroneal muscular atrophy (Charcot-Marie-Tooth disease). J Neurol Neurosurg Psychiatry 1972; 35: 648–657.CrossRefGoogle ScholarPubMed
8.Engel, WK, Kurland, LT, Klatzo, I. An inherited disease similar to ALS with a pattern of posterior column involvement. An intermediate form? Brain 1959; 82: 203–219.Google Scholar
9.Radtke, RA, Erwin, A, Erwin, CW. Abnormal sensory evoked potentials in ALS. Neurology 1986; 36: 796–801.CrossRefGoogle Scholar
10.Towfighi, J, Young, RSK, Ward, RM. Is Werdnig-Hoffmann disease a pure lower motor neuron disorder? Acta Neuropathol (Beri) 1985, 65: 270–280.CrossRefGoogle ScholarPubMed
11.Carpenter, S, Karpati, G, Rothman, S, et al. Pathological involvement of primary sensory neurons in Werdnig-Hoffmann disease. Acta Neuropathol (Beri) 1978; 42: 91–97.CrossRefGoogle ScholarPubMed
12.Peress, NS, Stermann, AB, Miller, R, et al. “Chromatolytic” neurons in lateral geniculate body in Werdnig-Hoffmann disease. Clin Neuropathol 1986; 5: 69–72.Google ScholarPubMed
13.Shishikura, K, Hara, M, Sasaki, Y, et al. A neuropathologic study of Werdnig-Hoffmann disease with special reference to the thalamus and posterior roots. Acta Neuropathol (Beri) 1983; 60: 99–106.CrossRefGoogle Scholar
14.Kugelberg, E. Chronic proximal (pseudomyopafhic) spinal muscular atrophy. In: Vinken, PJ, Bruyn, GW, eds. Handbook of clinical neurology. New York: North-Holland Publishing Co. 1975: 67–80Google Scholar
15.Paulson, GW, Liss, L, Sweeney, PJ. Late onset spinal muscle atrophy - a sex linked variant of Kugelberg-Welander. Acta Neurol Scand 1980; 61: 49–55.CrossRefGoogle ScholarPubMed