Article (Scientific journals)
Congenital cervical spinal muscular atrophy: a non-familial, non progressive condition of the upper limbs.
Hageman, G.; RAMAEKERS, Vincent; Hilhorst, B. G. et al.
1993In Journal of Neurology, Neurosurgery and Psychiatry, 56 (4), p. 365-8
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Keywords :
Adult; Arthrogryposis/diagnosis/genetics; Biopsy; Diagnosis, Differential; Female; Functional Laterality/physiology; Humans; Infant; Magnetic Resonance Imaging; Male; Muscles/innervation; Neurologic Examination; Radial Nerve/pathology; Spinal Cord/pathology; Spinal Muscular Atrophies of Childhood/diagnosis/genetics
Abstract :
[en] Two patients with congenital cervical spinal muscular atrophy had symmetrical severe muscle weakness and wasting confined to the upper limbs, areflexia and congenital contractures. The shoulders were internally rotated, elbows extended and wrists flexed. There were no sensory or bulbar symptoms, scoliosis, long tract signs or lower limb involvement. This condition should be regarded as a neurogenic type of arthrogryposis, limited to the upper limbs.
Disciplines :
Pediatrics
Neurology
Author, co-author :
Hageman, G.;  Department of Neurology, Medical Spectrum Twente Hospital Enschede, The Netherlands
RAMAEKERS, Vincent ;  Department of Pediatrics University Hospital, RWTH, Aachen, Germany
Hilhorst, B. G.;  Department of Clinical Neurophysiology, Medical Spectrum Twente Hospital Enschede, The Netherlands
Rozeboom, A. R.;  Department of Radiology, Medical Spectrum Twente Hospital Enschede, The Netherlands
Language :
English
Title :
Congenital cervical spinal muscular atrophy: a non-familial, non progressive condition of the upper limbs.
Publication date :
April 1993
Journal title :
Journal of Neurology, Neurosurgery and Psychiatry
ISSN :
0022-3050
eISSN :
1468-330X
Publisher :
BMJ Publishing Group, United Kingdom
Volume :
56
Issue :
4
Pages :
365-8
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 07 June 2014

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