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A case of severe congenital chronic inflammatory demyelinating polyneuropathy with complete spontaneous remission

Majumdar, A; Hartley, L; Manzur, AY; King, RHM; Orrell, RW; Muntoni, F; (2004) A case of severe congenital chronic inflammatory demyelinating polyneuropathy with complete spontaneous remission. NEUROMUSCULAR DISORD , 14 (12) 818 - 821. 10.1016/j.nmd.2004.09.003.

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Abstract

Chronic inflammatory demyelinating polyneuropathy (CIPD) is characterised by progressive weakness, hyporeflexia and electrophysiological evidence of demyelination with maximal neurological deficit reached after at least 8 weeks progression. CIPD rarely affects children. We present a neonate with clinical features compatible with congenital CIPD. A term male infant of non-consanguineous parents was referred to us at birth with weakness and contractures affecting his leas, suggesting a prenatal onset of immobility. He also had evidence of bulbar dysfunction with poor suck, recurrent aspiration and requiring nasogastric feeding. He had no antigravity movements in the legs, bilateral wrist drop, distal joint contractures and absent deep tendon reflexes. Electromyography showed neurogenic changes, with nerve conduction velocities markedly reduced, increased distal motor latency and dispersed compound muscle action potentials. Cerebrospinal fluid protein was raised. Sural nerve biopsy demonstrated decreased numbers of myelinated fibres and inflammatory cell infiltrates. Muscle biopsy showed denervation. He only received supportive treatment and by 6 months he had fully recovered, and all electrophysiological parameters had normalised. (C) 2004 Elsevier B.V. All rights reserved.

Type: Article
Title: A case of severe congenital chronic inflammatory demyelinating polyneuropathy with complete spontaneous remission
DOI: 10.1016/j.nmd.2004.09.003
Keywords: inflammatory neuropathy, floppy infant, congenital, newborn, GUILLAIN-BARRE-SYNDROME, CLINICAL COURSE, CHILDHOOD, CONDUCTION, INFANCY, POLYRADICULONEUROPATHY, FEATURES
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health > ICH Developmental Neurosciences Prog
URI: http://discovery.ucl.ac.uk/id/eprint/122561
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