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Article (Scientific journals)
Subtotal aplasia of myelinated nerve fibers in the sural nerve.
Schroder, J. M.; Heide, G.; RAMAEKERS, Vincent et al.
1993In Neuropediatrics, 24 (5), p. 286-91
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Keywords :
Adolescent; Axons/pathology; Biopsy; Brain/pathology; Child; Child, Preschool; Female; Humans; Magnetic Resonance Imaging; Male; Nerve Fibers, Myelinated/pathology; Neurologic Examination; Peripheral Nerves/abnormalities/pathology; Polyneuropathies/pathology; Reference Values; Spinocerebellar Degenerations/pathology; Sural Nerve/abnormalities/pathology
Abstract :
[en] Thus far, only very few cases with neuronal maldevelopment in the peripheral nervous system have been reported (Table 1). The present sporadic case manifested itself with peripheral sensorimotor polyneuropathy in early infancy. Clinical findings included pareses and hypaesthesia of distal extremities and severely reduced nerve conduction velocities. During adolescence, cerebellar ataxia developed. Sural nerve biopsy taken at the age of 14.5 years showed severe fascicular hypoplasia, aplasia of large myelinated nerve fibers, and subtotal deficiency of small myelinated nerve fibers without numerical reduction of unmyelinated axons. There was no structural evidence of a progression of myelinated fiber breakdown although some collagen pockets and empty Schwann cell processes among preserved unmyelinated axons indicated some loss of unmyelinated fibers. These findings are interpreted as representing maldevelopment of the myelinated fibers in the peripheral nervous system. Appropriate classification of this unique disease among the known developmental disorders of peripheral nerves is discussed.
Disciplines :
Neurology
Pediatrics
Author, co-author :
Schroder, J. M.;  Institute of Neuropathology, Faculty of the Rheinisch-Westfälische Technische Hochschule Aachen, Germany
Heide, G.;  Institute of Neuropathology, Faculty of the Rheinisch-Westfälische Technische Hochschule Aachen, Germany
RAMAEKERS, Vincent ;  Clinic of Pediatrics, Faculty of the Rheinisch-Westfälische Technische Hochschule Aachen, Germany
Mortier, W.;  Clinic of Pediatrics, Klinikum Barmen, Wuppertal, Germany
Language :
English
Title :
Subtotal aplasia of myelinated nerve fibers in the sural nerve.
Publication date :
October 1993
Journal title :
Neuropediatrics
ISSN :
0174-304X
eISSN :
1439-1899
Publisher :
Georg Thieme Verlag, Germany
Volume :
24
Issue :
5
Pages :
286-91
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 07 June 2014

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