Summary
The number and sizes of myelinated and unmyelinated fibers in biopsied sural nerves in cases with Shy-Drager syndrome were studied in comparison with cases with olivopontocerebellar degeneration not having autonomic dysfunction. In Shy-Drager syndrome, there was a tendency for both small myelinated and unmyelinated fiber densities to be reduced in comparison with cases with olivopontocerebellar degeneration. Unmyelinated fibers more than 0.5 μm in diameter were significantly reduced in Shy-Drager syndrome, a fact suggesting unmyelinated fiber degeneration. Multilamellated Schwann cell processes, isolated Schwann cell processes, and collagen pockets were more numerous and conspicuous in cases with Shy-Drager syndrome. It was concluded that unmyelinated fibers and small myelinated fibers in the peripheral nerves were involved selectively in Shy-Drager syndrome. The significance of the findings was discussed in terms of autonomic dysfunction observed clinically.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Aguayo AJ, Nair CPV, Bray GM (1971) Peripheral nerve abnormalities in the Riley-Day syndrome. Findings in a sural biopsy. Arch Neurol 24:106–116
Aguayo AJ, Peyronnard JM, Bray GM (1973) A quantitative ultrastructural study on regeneration from isolated proximal stumps of transected unmyelinated nerves. J Neuropathol Exp Neurol 32:256–270
Behse F, Buchthal F, Carlsen F, Knappeis GG (1975) Unmyelinated fibers and Schwann cells of sural nerve in neuropathy. Brain 98:493–510
Bannister R, Sever P, Gross M (1977) Cardiovascular reflexes and biochemical responses in progressive autonomic failure. Brain 100:327–344
Davidson C, Morgan DB (1976) Long survival in orthostatic hypotension. Case report and a review of the literature. J Chronic Dis 29:733–742
Dyck PJ, Lambert EH (1969) Dissociated sensation in amyloidosis. Compound action potential, quantitative histologic and teased fiber, and electron microcopic studies of sural nerve biopsies. Arch Neurol 20:490–507
Dyck PJ, Ellefson RD, Lais AC, Smith RC, Taylor WF, Vandyke RA (1970) Histologic and lipid studies of sural nerve in inherited hypertrophic neuropathy: Preliminary report of a lipid abnormality in nerve and liver in Dejerine-Sottas disease. Mayo Clin Proc 45:286–327
Dyck PJ, Johnson WJ, Lambert EH, O'Brien PC (1971) Segmental demyelination secondary to axonal degeneration in uremic neuropathy. Mayo Clin Proc 46:400–431
Dyck PJ, Lambert EH, Nichols PC (1971) Quantitative measurement of sensation related to compound action potential and number and sizes of myelinated and unmyelinated fibers of sural nerve in health, Friedreich's ataxia, hereditary sensory neuropathy, and tabes dorsalis. In: Cobb WA (ed) Handbook of electroencephalography and clinical neurophysiology, vol 9. Elsevier, Amsterdam, pp 83–118
Dyck PJ, Hopkins AP (1972) Electron-microscopic observations on degeneration and regeneration of unmyelinated fibers. Brain 94:223–234
Hillarp NA (1960) Peripheral autonomic mechanisms. In: Field J, Magoun HW, Hall VE (eds) Handbook of physiology, sect 1, vol 2. American Physiological Society, Washington DC, pp 979–1006
Hughes RC, Cartlidge NEF, Millac P (1970) Primary neurogenic orthostatic hypotension. J Neurol Neurosurg Psychiatry 33:363–371
Johnson RH, Lee G de J, Oppenheimer DR, Spalding JMK (1966) Autonomic failure with orthostatic hypotension due to intermediolateral column degeneration. A report of two cases with autopsies. Q J Med, 15:276–292
Ochoa J, Mair WGP (1969) The normal sural nerve in man. I. Ultrastructure and number of fibers and cells. Acta Neuropathol (Berl) 13:197–216
Ochoa J, Mair WGP (1969) The normal sural nerve in man. II. Changes in the axons and Schwann cells due to ageing. Acta Neuropathol (Berl) 13:217–239
Ochoa J (1970) Isoniazid neuropathy in man. Quantitative electron microscope study. Brain 93:831–850
Pearson J, Dancis J, Axelrod F, Grover N (1975) The sural nerve in familial dysautonomia. J Neuropathol Exp Neurol 34:413–424
Petito CK, Black IB (1978) Ultrastructure and biochemistry of sympathetic ganglia in idiopathic orthostatic hypotension. Ann Neurol 4:6–10
Roessmann U, Van Den Noort S, McFarland DE (1971) Idiopathic orthostatic hypotension. Arch Neurol 24:503–510
Roessmann U (1975) Primary orthostatic hypotension. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 21. North-Holland, Amsterdam, pp 231–242
Schröder JM, Gibbels E (1977) Marklose Nervenfasern im Senium und im Spätstadium der Thalidomid-Polyneuropathie: quantitativ-elektronenmikroskopische Untersuchungen. Acta Neuropathol (Berl) 39:271–280
Schwarz GA (1975) Dysautonomic syndrome in adults. In: Winken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 21. North-Holland, Amsterdam, pp 243–280
Shy GM, Drager GA (1960) A neurological syndrome associated with orthostatic hypotension. Arch Neurol 2:511–527
Swanson AG, Buchan GC, Alvord EC (1965) Anatomic changes in congenital insensitivity to pain. Absence of small primary sensory neurons in ganglia, roots, and Lissauer's tract. Arch Neurol 12:12–18
Thapedi IM, Ashenhurst EM, Rozdilsky B (1971) Shy-Drager syndrome. Report of an autopsied case. Neurology 21:26–32
Tredici G, Minazzi M (1975) Alcoholic neuropathy. An electronmicroscopic study. J Neurol Sci 25:333–346
Vanderhaeghen JJ, Perier O, Sternon JE (1970) Pathological findings in idiopathic orthostatic hypotension. Its relationship with Parkinson's disease. Arch Neurol 22:207–214
Ziegler MG, Lake CR, Kopin IJ (1977) The sympathetic-nervous-system defect in primary orthostatic hypotension. New Engl J Med 296:293–297
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Tohgi, H., Tabuchi, M., Tomonaga, M. et al. Selective loss of small myelinated and unmyelinated fibers in Shy-Drager syndrome. Acta Neuropathol 57, 282–286 (1982). https://doi.org/10.1007/BF00692184
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00692184