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Heterogeneity of congenital motor and sensory neuropathies.
Neuropediatrics. 1985 Feb; 16(1):33-8.N

Abstract

Six children suffering from a congenital motor and sensory neuropathy (CMSN) are described. Severe muscle hypotonia, areflexia and a delay of motor development are detectable in all of them. Sural nerve biopsies exhibited an almost complete absence of myelinated fibres and a correspondingly slow nerve conduction velocity (NCV) of less than 10 m/s was detectable in four patients. A few segments with hypermyelination adjacent to gross hypomyelination were seen in the fifth patient, and the NCV was 15 m/s. The sural nerve of the sixth patient showed a loss of thick myelinated nerve fibres, and his NCV was 25 m/s. These results demonstrate the histological heterogeneity of CMSN which was already detected by the NCV. The relation of our findings to the classification of HMSN by Dyck and Lambert (1968) is discussed.

Authors

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Pub Type(s)

Journal Article

Language

eng

PubMed ID

2983258

Citation

Lütschg, J, et al. "Heterogeneity of Congenital Motor and Sensory Neuropathies." Neuropediatrics, vol. 16, no. 1, 1985, pp. 33-8.
Lütschg J, Vassella F, Boltshauser E, et al. Heterogeneity of congenital motor and sensory neuropathies. Neuropediatrics. 1985;16(1):33-8.
Lütschg, J., Vassella, F., Boltshauser, E., Dias, K., & Meier, C. (1985). Heterogeneity of congenital motor and sensory neuropathies. Neuropediatrics, 16(1), 33-8.
Lütschg J, et al. Heterogeneity of Congenital Motor and Sensory Neuropathies. Neuropediatrics. 1985;16(1):33-8. PubMed PMID: 2983258.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Heterogeneity of congenital motor and sensory neuropathies. AU - Lütschg,J, AU - Vassella,F, AU - Boltshauser,E, AU - Dias,K, AU - Meier,C, PY - 1985/2/1/pubmed PY - 1985/2/1/medline PY - 1985/2/1/entrez SP - 33 EP - 8 JF - Neuropediatrics JO - Neuropediatrics VL - 16 IS - 1 N2 - Six children suffering from a congenital motor and sensory neuropathy (CMSN) are described. Severe muscle hypotonia, areflexia and a delay of motor development are detectable in all of them. Sural nerve biopsies exhibited an almost complete absence of myelinated fibres and a correspondingly slow nerve conduction velocity (NCV) of less than 10 m/s was detectable in four patients. A few segments with hypermyelination adjacent to gross hypomyelination were seen in the fifth patient, and the NCV was 15 m/s. The sural nerve of the sixth patient showed a loss of thick myelinated nerve fibres, and his NCV was 25 m/s. These results demonstrate the histological heterogeneity of CMSN which was already detected by the NCV. The relation of our findings to the classification of HMSN by Dyck and Lambert (1968) is discussed. SN - 0174-304X UR - https://www.unboundmedicine.com/medline/citation/2983258/Heterogeneity_of_congenital_motor_and_sensory_neuropathies_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1052541 DB - PRIME DP - Unbound Medicine ER -