[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.