Childhood chronic inflammatory demyelinating polyneuroradiculopathy--three cases and a review of the literature.
BACKGROUND
Chronic inflammatory demyelinating polyneuroradiculopathy (CIDP) is an autoimmune disease of the peripheral nervous system, causing demyelination and even axonal degeneration. In children, abnormal gait as a first sign of muscle weakness is a frequent reason to seek medical attention. Diagnosis is made on the basis of clinical characteristics, electromyography and nerve conduction studies, and elevated protein in cerebrospinal fluid.


OBJECTIVE
We present three new cases of CIDP. The literature was reviewed in order to obtain more information on presentation, outcome and treatment strategies world-wide.


RESULTS
The course of disease can be relapsing-remitting or chronic-progressive. From case series it is known that first-line immunotherapy (intravenously administered immunoglobulin, corticosteroids or plasmapheresis) is initially of benefit in most children with CIDP. There is little evidence, however, on second-line therapies as azathioprine, cyclosporine A, mycophenolate mofetil, methothrexate, cyclophosphamide and IFN alpha. Although the outcome of children with CIDP is generally regarded to be good, disease related disability can be severe.


CONCLUSIONS
Childhood CIDP is rare. In general and in comparison to adults, children tend to have a more acute progressive onset, with more severe symptoms. Showing a higher tendency towards a relapsing-remitting course, children often show a better and faster improvement after therapy, and a more favorable outcome. Swift recognition of CIDP and empiric start of treatment are considered important to avoid potentially irreversible axonal damage and associated disability. Response to first-line therapies is usually favorable, however recommendations regarding the choice of second-line therapy can only be made on the basis of current practice described in case reports. Safety and efficacy data are insufficient. The cases described show that trial and error are often involved in finding an optimal treatment strategy, especially in those patients refractory to first-line treatment or with a prolonged course. Clinical experience with immunomodulatory treatment is paramount when treating children with CIDP.
Department of Neurology - Child Neurology, Antwerp University Hospital, University of Antwerp, Wilrijkstraat 10, 2650 Edegem, Antwerp, Belgium. netke.riekhoff@uza.be
Antoinetta G M
AG
Riekhoff
Chris
C
Jadoul
Rudy
R
Mercelis
Patrick
P
Cras
Berten P G M
BP
Ceulemans
D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D003907:Dexamethasone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D005528:Foot; D020233:Gait Disorders, Neurologic / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000209:etiology; D009460:Neurologic Examination; D009989:Orthotic Devices; D010951:Plasma Exchange; D010956:Plasmapheresis; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D012008:Recurrence; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D003907:Dexamethasone