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Ultrastructural Observations on Spinal Ganglion Biopsy in Friedreich’s Ataxia: A Preliminary Report

Published online by Cambridge University Press:  18 September 2015

J. Lamarche ,
C. Luneau  and
B. Lemieux
J. Lamarche
Affiliation:
Departments of Pathology, Neurosurgery and Pediatrics, Centre Hospitalier Universitaire, Sherbrooke, Quebec
C. Luneau
Affiliation:
Departments of Pathology, Neurosurgery and Pediatrics, Centre Hospitalier Universitaire, Sherbrooke, Quebec
B. Lemieux
Affiliation:
Departments of Pathology, Neurosurgery and Pediatrics, Centre Hospitalier Universitaire, Sherbrooke, Quebec
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Abstract:

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We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich’s ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 9 , Issue 2 , May 1982 , pp. 137 - 139
Copyright
Copyright © Canadian Neurological Sciences Federation 1982

References

REFERENCES

Allard, P., Duhaime, M., Raso, J.V., Thiry, P.S., Drouin, G., and Geoffroy, G. (1980). Pathomechanics and Management of Scoliosis in Friedreich’s ataxia patients.’ Can. J. Neurol. Sci, 7: 383–388.CrossRefGoogle Scholar
Cavanagh, J.B.: The “dying back” process (1979). A common denominator in many naturally occurring and toxic neuropathies. Arch. Pathol. Lab. Med. 103: 569–664.Google Scholar
Côté, M., Davignon, A., Peckodrouin, K., Solignac, , Geoffroy, G., Lemieux, B., and Barbeau, A. (1976). Cardiological signs and symptoms in Friedreich’s ataxia. Can. J. Neurol. Sci, 3: 319–321.CrossRefGoogle ScholarPubMed
Dyck, P.J. and Lais, A.C. (1973). Evidence for segmental demyelination secondary to axonal degeneration in Friedreich’s ataxia. In: Kakulas, B. (ed) Clinical Studies in Myology. Int. Congr. Series. Amsterdam, K., Excerpta Medica: 252–263.Google Scholar
Elias, E., Muller, D.P.R., and Scott, J. (1981). Association of spino-cerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E. The Lancet: 1319–1321.Google Scholar
Geoffroy, G., Barbeau, A., Breton, G., Lemieux, B., Aube, M., Léger, C., and Bouchard, J.B. (1976). Clinical description and roentgenologic evaluation of patients with Friedreich’s ataxia. Can. J. Neurol. Sci, 3: 279–286.CrossRefGoogle ScholarPubMed
Hughes, J.T., Brownell, B., and Herwer, R.L. (1968). The peripheral sensory pathway in Friedreich’s Ataxia. An examination by light and electron microscopy of the posterior nerve roots, posterior root ganglia and peripheral sensory nerves in cases of Friedreich’s ataxia. Brain 91: 803–818.CrossRefGoogle ScholarPubMed
Inoue, K., Hirano, A., and Hassin, J. (1979). Friedreich’s ataxia selectively involves the large neurons of the dorsal root ganglia (abstract). Annals of Neurol: 6, 150.Google Scholar
Jellinger, J. (1973). Neuroaxonal dystrophy: its natural history and related disorders, in: Zimmerman, H.M. (ed). Progress in neuropathology. New York, Grune and Stratton, vol. II: 129–180.Google Scholar
Mcleod, J.G. (1971). An electrophysiological and pathological study of peripheral nerves in Friedreich’s ataxia. J. Neurol. Sci. 12: 333–349.CrossRefGoogle ScholarPubMed
Peyronnard, J.M., Bouchard, J.P., Lapointe, L., Lamontagne, A., Lemieux, B., and Barbeau, A. (1976): Nerve conduction studies and electromyography in Friedreich’s ataxia. Can. J. Neurol. Sci, 3:313–317.Google Scholar
Spencer, P.S., Schaumburg, H.H. (1976). Central-peripheral-distal-axonopathy: The pathology of “dying back” neuropathies. in: Zimmerman, H.M. (ed) Progress in Neuropathology. New York, Grune and Stratton, Vol. 3: 253–295.Google Scholar
Spencer, P.S., Sabri, M.I., Schaumburg, H.H. and Moore, C.L. (1979). Does a defect of energy metabolism in the nerve fiber underlie axonal degeneration in polyneuropathies. Ann. Neurol. 5: 501–507.CrossRefGoogle ScholarPubMed