Tags

Type your tag names separated by a space and hit enter

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. 1984; 65(1):1-9.AN

Abstract

Ten autosomal recessive/sporadic cases of hereditary motor and sensory neuropathy type I (HMSN I), nine of which originated from the northern part of Sweden, were included in the study. Parents were free from neurologic symptoms. Motor and sensory conduction velocity was normal when recorded, i.e., in 19 and 17 parents, respectively. Sural nerve biopsies from the ten cases revealed a varying degree of onion bulb formation. In eight of the cases the onion bulbs consisted of abundant basement membranes, whereas the Schwann cells were few and sometimes lacking. There were in some cases considerable differences between separate fascicles as to the loss of myelinated nerve fibers. In the six biopsies in which teasing was performed signs of present and previous demyelination were noticed. Numerous internodal segments were abnormally thin with reference to their length. In many such segments there were marked local thickenings of the nerve fiber. In cross sections the probable counterparts to these thickenings were nerve fibers with unduly thick myelin sheaths and complex folding of the myelin. Ultrastructural axonal changes were seen in the majority of the cases. The pathogenetic and diagnostic implications of the present findings are discussed.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

6516797

Citation

Nordborg, C, et al. "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 Neuropathologica, vol. 65, no. 1, 1984, pp. 1-9.
Nordborg C, Conradi N, Sourander P, et al. 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. 1984;65(1):1-9.
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 Neuropathologica, 65(1), 1-9.
Nordborg C, et al. 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. 1984;65(1):1-9. PubMed PMID: 6516797.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Nordborg,C, AU - Conradi,N, AU - Sourander,P, AU - Hagberg,B, AU - Westerberg,B, PY - 1984/1/1/pubmed PY - 1984/1/1/medline PY - 1984/1/1/entrez SP - 1 EP - 9 JF - Acta neuropathologica JO - Acta Neuropathol. VL - 65 IS - 1 N2 - Ten autosomal recessive/sporadic cases of hereditary motor and sensory neuropathy type I (HMSN I), nine of which originated from the northern part of Sweden, were included in the study. Parents were free from neurologic symptoms. Motor and sensory conduction velocity was normal when recorded, i.e., in 19 and 17 parents, respectively. Sural nerve biopsies from the ten cases revealed a varying degree of onion bulb formation. In eight of the cases the onion bulbs consisted of abundant basement membranes, whereas the Schwann cells were few and sometimes lacking. There were in some cases considerable differences between separate fascicles as to the loss of myelinated nerve fibers. In the six biopsies in which teasing was performed signs of present and previous demyelination were noticed. Numerous internodal segments were abnormally thin with reference to their length. In many such segments there were marked local thickenings of the nerve fiber. In cross sections the probable counterparts to these thickenings were nerve fibers with unduly thick myelin sheaths and complex folding of the myelin. Ultrastructural axonal changes were seen in the majority of the cases. The pathogenetic and diagnostic implications of the present findings are discussed. SN - 0001-6322 UR - https://www.unboundmedicine.com/medline/citation/6516797/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_ L2 - http://www.diseaseinfosearch.org/result/4890 DB - PRIME DP - Unbound Medicine ER -