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Sensory Neuropathy With Onion-Bulb Formation: Report of a Case With Onset in Infancy

Sensory Neuropathy With Onion-Bulb Formation: Report of a Case With Onset in Infancy Abstract • A 27-month-old girl suffered from severe sensory neuropathy with minimal motor dysfunction. The CSF protein level was increased and nerve conduction was severely impaired. Sural nerve biopsy specimen showed increased endoneurial connective tissue. An onion-bulb pattern with concentric interdigitations of Schwann cell cytoplasmic processes and redundant basal laminae were prominent features under electron microscopy. Degree of myelination in individual fiber was far less than expected. Although the clinical manifestations of onion-bulb neuropathy with onset in infancy have been reported to resemble infantile progressive spinal muscular atrophy, the present case demonstrates that the condition can also appear as severe sensory ataxia. (Am J Dis Child 132:379-381, 1978) References 1. Anderson RM, Dennett X, Hopkins IJ, et al: Hypertrophic interstitial polyneuropathy in infancy . J Pediatr 82:619-624, 1973.Crossref 2. Karch SB, Urich H: Infantile polyneuropathy with defective myelination: An autopsy study . Dev Med Child Neurol 17:504-511, 1975.Crossref 3. Kasman M, Bernstein L, Schulman S: Chronic polyradiculoneuropathy of infancy: A report of three cases with familial incidence . Neurology 26:565-573, 1976.Crossref 4. Lyon G: Ultrastructural study of a nerve biopsy from a case of early infantile chronic neuropathy . Acta Neuropathol 13:131-142, 1969.Crossref 5. Kennedy WR, Sung JH, Berry JF: A case of congenital hypomyelination neuropathy: Clinical, morphological, and chemical studies . Arch Neurol 34:337-345, 1977.Crossref 6. Kalyanaraman K, Cancilla PA, Munsat T, et al: Hereditary hypertrophic neuropathy: Report of two cases of an autosomal recessive variant . Bull Los Angeles Neurol Soc 35:58-68, 1970. 7. Thomas PK, Lascelles RG, Stewart G: Hypertrophic neuropathy , in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology . Amsterdam, North-Holland Publishing Co, 1975, vol 21, pp 145-170. 8. Dyck PJ: Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons , in Dyck PJ, Thomas PK, Lambert EH (eds): Peripheral Neuropathy . Philadelphia, WB Saunders & Co., 1975, vol 2, pp 825-867. 9. Pleasure DE, Towfighi J: Onion bulb neuropathies . Arch Neurol 26:289-301, 1972.Crossref 10. Raine CS: Schwann cell responses during recurrent demyelination and their relevance to onion bulb formation . Neuropathol Appl Neurobiol , to be published. 11. Aguayo AJ, Attiwell M, Trecarten J, et al: Abnormal myelination in transplanted Trembler mouse Schwann cells . Nature 265:73-75, 1977.Crossref 12. Koeppen AH, Messmore H, Stehbens WE: Interstitial hypertrophic neuropathy: Biochemical study of the peripheral nervous system . Arch Neurol 24:340-352, 1971.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Sensory Neuropathy With Onion-Bulb Formation: Report of a Case With Onset in Infancy

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120290051009
Publisher site
See Article on Publisher Site

Abstract

Abstract • A 27-month-old girl suffered from severe sensory neuropathy with minimal motor dysfunction. The CSF protein level was increased and nerve conduction was severely impaired. Sural nerve biopsy specimen showed increased endoneurial connective tissue. An onion-bulb pattern with concentric interdigitations of Schwann cell cytoplasmic processes and redundant basal laminae were prominent features under electron microscopy. Degree of myelination in individual fiber was far less than expected. Although the clinical manifestations of onion-bulb neuropathy with onset in infancy have been reported to resemble infantile progressive spinal muscular atrophy, the present case demonstrates that the condition can also appear as severe sensory ataxia. (Am J Dis Child 132:379-381, 1978) References 1. Anderson RM, Dennett X, Hopkins IJ, et al: Hypertrophic interstitial polyneuropathy in infancy . J Pediatr 82:619-624, 1973.Crossref 2. Karch SB, Urich H: Infantile polyneuropathy with defective myelination: An autopsy study . Dev Med Child Neurol 17:504-511, 1975.Crossref 3. Kasman M, Bernstein L, Schulman S: Chronic polyradiculoneuropathy of infancy: A report of three cases with familial incidence . Neurology 26:565-573, 1976.Crossref 4. Lyon G: Ultrastructural study of a nerve biopsy from a case of early infantile chronic neuropathy . Acta Neuropathol 13:131-142, 1969.Crossref 5. Kennedy WR, Sung JH, Berry JF: A case of congenital hypomyelination neuropathy: Clinical, morphological, and chemical studies . Arch Neurol 34:337-345, 1977.Crossref 6. Kalyanaraman K, Cancilla PA, Munsat T, et al: Hereditary hypertrophic neuropathy: Report of two cases of an autosomal recessive variant . Bull Los Angeles Neurol Soc 35:58-68, 1970. 7. Thomas PK, Lascelles RG, Stewart G: Hypertrophic neuropathy , in Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology . Amsterdam, North-Holland Publishing Co, 1975, vol 21, pp 145-170. 8. Dyck PJ: Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons , in Dyck PJ, Thomas PK, Lambert EH (eds): Peripheral Neuropathy . Philadelphia, WB Saunders & Co., 1975, vol 2, pp 825-867. 9. Pleasure DE, Towfighi J: Onion bulb neuropathies . Arch Neurol 26:289-301, 1972.Crossref 10. Raine CS: Schwann cell responses during recurrent demyelination and their relevance to onion bulb formation . Neuropathol Appl Neurobiol , to be published. 11. Aguayo AJ, Attiwell M, Trecarten J, et al: Abnormal myelination in transplanted Trembler mouse Schwann cells . Nature 265:73-75, 1977.Crossref 12. Koeppen AH, Messmore H, Stehbens WE: Interstitial hypertrophic neuropathy: Biochemical study of the peripheral nervous system . Arch Neurol 24:340-352, 1971.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Apr 1, 1978

References