Review: 1
1: Pediatr Neurol. 2001 Mar;24(3):177-82.
Chronic inflammatory demyelinating polyneuropathy in childhood.
Connolly AM.
Department of Neurology, St. Louis Children's Hospital, Washington University of Medicine, St. Louis, Missouri 63110, USA.

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is relatively rare. However, it has been recognized for many years. In patients presenting with this disease, subacute onset of weakness usually develops over at least 2 months and often progresses to a loss of ambulation. Some children's initial presentations may mimic Guillain-Barre syndrome. Dysasthesias are common. Males are affected more than females, and antecedent illnesses or vaccinations occur in approximately half of patients. Physical examination reveals diffuse, proximal greater than distal weakness, with an absence or depression of muscle stretch reflexes. Electrophysiology confirms demyelination, and spinal fluid examination demonstrates albuminocytologic dissociation. The clinical presentation, diagnosis, and prognosis of childhood CIDP are reviewed. Treatment and immunologic features are also discussed in this article.

PMID: 11301217 [PubMed - indexed for MEDLINE]

2: Neuromuscul Disord. 2000 Aug;10(6):398-406.]

Ryan MM, Grattan-Smith PJ, Procopis PG, Morgan G, Ouvrier RA.
Department of Neurology, The Royal Alexandra Hospital for Children, Sydney, Australia.

We reviewed the clinical history, electrophysiologic and pathologic findings, and response to therapy of 16 children with chronic inflammatory demyelinating polyneuropathy. The majority presented with lower limb weakness. Sensory loss was uncommon. The illness was monophasic in seven children, relapsing in six, and three had a slowly progressive course. All patients were treated with immunosuppressive agents. In 11, the initial treatment was prednisolone. All had at least a short-term response but five went on to develop a relapsing course. Intravenous immunoglobulin was the initial treatment in four patients. Three responded rapidly, with treatment being stopped after a maximum of 5 months. In resistant chronic inflammatory demyelinating neuropathy, in addition to prednisolone and immunoglobulin, plasma exchange, azathioprine, cyclosporine, methotrexate, cyclophosphamide and pulse methylprednisolone were tried at different times in different patients. On serial neurophysiologic testing slowing of nerve conduction persisted for long periods after clinical recovery. Follow-up was for an average of 10 years. When last seen 14 patients were asymptomatic, two having mild residual deficits. Childhood chronic inflammatory demyelinating neuropathy responds to conventional treatment and generally has a favorable long-term outcome.

PMID: 10899445 [PubMed - indexed for MEDLINE]
: Eur J Paediatr Neurol. 1998;2(4):169-77.

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