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Journal of Child Neurology
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Congenital Cervical Spinal Cord Lesions: Pathogenesis, Management, and Outcome

Rajeshree Govender, FCP

Department of Paediatric Neurology School of Child and Adolescent Health, Red Cross Children's Hospital Univ. of Cape Town ZA

Nicky A. Wieselthaler, FCRad

Department of Radiology and School of Child and Adolescent Health, Red Cross Children's Hospital, Univ. of Cape Town ZA

Veruschka Ramanjam, FCP

Child Development School of Child and Adolescent Health, Red Cross Children's Hospital, University of Cape Town, Cape Town, South Africa

Alvin Ndondo, FCP

Department of Paediatric Neurology School of Child and Adolescent Health, Red Cross Children's Hospital Univ. of Cape Town ZA

Jo M. Wilmshurst, FCP

Department of Paediatric Neurology School of Child and Adolescent Health, Red Cross Children's Hospital Univ. of Cape Town ZA, wilmshur{at}ich.uct.ac.za

Three patients (2 boys) presented with nontraumatic congenital lesions of the spinal cord resulting in paralysis and contractures of their upper limbs from birth. Limited improvement occurred in all. Two survived. One patient required ventilation support after birth; his upper limbs had lower motor neuron flaccid paralysis, and his lower limbs evolved to pyramidal tract impairment. He died at 9 months of age with an intercurrent chest infection. The other 2 patients had lower motor neuron pathology in their upper limbs and normal lower limb function. One of these patients attained ambulation. All 3 patients retained normal higher mental function. Neuroimaging of the spinal cord from the most affected patient demonstrated atrophy of the cervical and high thoracic regions (C4-T3). Spinal neuroimaging results from the less affected patient were normal. Multidisciplinary management assisted these children to reach their full potential in a resource-poor setting. The etiology of focal pathology to the cervical region in these infants with congenital nontraumatic insults remains undefined, similar to the few cases in the literature. The diverse pathogeneses are hypothesized and the literature reviewed.

Key Words: cervical cord • atraumatic lesion • arthrogryposis

Journal of Child Neurology, Vol. 22, No. 7, 874-879 (2007)
DOI: 10.1177/0883073807304205


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