Summary
Four cases of early infantile polyneuropathy with defective myelination are reported. The peripheral nerve was studied by light and electron microscopy; different morphological characteristics have been noticed in these patients. Case 1 presented aspects of defective myelination with atypical “onion bulb” formation composed of multiple layers of basement membrane. In case 2, defective myelination and atypical “onion bulb” formation were associated with aberrant hypermyelination. Cases 3 and 4 were brothers, who presented axonal damage and atypical “onion bulb” formation.
Similar content being viewed by others
References
Anderson RM, Dennett X, Hopkins IJ, Shield LK (1973) Hypertrophic interstitial polyneuropathy in infancy. Clinical and pathologic features in two cases. J Pediatr 82:619–624
Dyck PJ (1984) Inherited neuronal degeneration and atrophy affecting peripheral motor, sensory, and autonomic neurons. In: Dyck PJ, Thomas PK, Lambert EH, Bunge R (eds) Peripheral neuropathy. Saunders, Philadelphia, pp 1600–1655
Guzzetta F, Ferrière G (1985) Congenital neuropathy with prevailing axonal changes. A clinical and histological report. Acta Neuropathol (Berl) 68:185–190
Hakamada S, Kumagai T, Hara K, Miyazaki S, Miyazaki K, Watanabe K (1983) Congenital hypomyelination neuropathy in a newborn. Neuropediatrics 14:182–183
Harati Y, Butler IJ (1985) Congenital hypomyelinating neuropathy. J Neurol Neurosurg Psychiatry 48:1269–1276
Janzer RC, Spycher MA, Boltshauser E, Cserhati M (1986) A case of congenital non-progressive sensory neuropathy with tonic pupils. Clin Neuropathol 5:209–216
Joosten E, Gabreëls F, Gabrééls-Festen A, Vrensen G, Korten J, Notermans S (1974) Electron-microscopic heterogeneity of onion-bulb neuropathies of the Dejerine-Sottas type. Two patients in one family with the variant described by Lyon (1969). Acta Neuropathol (Berl) 27:105–118
Karch SB, Urich H (1975) Infantile polyneuropathy with defective myelination: an autopsy study. Dev Med Child Neurol 17:504–511
Kasman M, Bernstein L, Schulman S (1976). Chronic polyradiculoneuropathy of infancy. A report of three cases with familial incidence. Neurology 26:565–573
Kennedy WR, Ho Sung J, Berry JF (1977) A case of congenital hypomyelination neuropathy. Clinical, morphological, and chemical studies. Arch Neurol 34:337–345
Lyon G (1969) Ultrastructural study of a nerve biopsy from a case of early infantile chronic neuropathy. Acta Neuropathol (Berl) 13:131–142
Nordborg C, Conradi N, Sourander P, Hagberg B, Westerberg B (1984) Hereditary motor and sensory neuropathy of demyelinating and remyelinating type in children. Ultrastructural and morphometric studies on sural nerve biopsy specimens from ten sporadic cases. Acta Neuropathol (Berl) 65:1–9
Ono J, Senba E, Okada S, Abe J, Futagi Y, Shimizu H, Sugita T, Hashimoto S, Yabuuchi H (1982) A case report of congenital hypomyelination. Eur J Pediatr 138:265–270
Palix C, Coignet J (1978) Un cas de polyneuropathie périphérique néo-natale par amyélinisation. Pédiatrie 33:201–207
Schröder JM, Bohl J (1978) Altered ratio between axon caliber and myelin thickness in sural nerves of children In: Canal N, Pozza G (eds) Peripheral neuropathies. Elsevier, North-Holland Biomedical Press, Amsterdam, pp 49–62
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Vital, A., Vital, C., Riviere, J.P. et al. Variability of morphological features in early infantile polyneuropathy with defective myelination. Acta Neuropathol 73, 295–300 (1987). https://doi.org/10.1007/BF00686625
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00686625