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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in children
Abstract & Commentary
By Michael Rubin, MD, Professor of Clinical Neurology, Weill Cornell Medical College. Dr. Rubin reports no financial relationships relevant to this field of study.
Synopsis: In children, CIDP is less common than in adults, but has a better long-tern prognosis.
Source: Jo HY, et al. Chronic inflammatory demyelinating polyradiculoneuropathy in children: Characterized by subacute, predominantly motor dominant polyneuropathy with a favorable response to the treatment. Acta Neurol Scand 2010:121: 342–347 DOI: 10.1111/j.1600-0404.2009.01222.x.
With an estimated prevalence of approximately 0.5/100,000, compared to 1-2/100,000 in adults, childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare and remains poorly understood. To further our appreciation of this disease, retrospective record review was undertaken of all CIDP patients seen in Pusan National University Hospital, Yangsan City, South Korea, between 1996-2008, and divided into childhood (age < 16 years) and adult cases. Clinical diagnostic criteria comprised more than four weeks of progressive weakness or sensory impairment with hyporeflexia, or a relapsing course of same, lasting beyond 12 months. Laboratory investigations included routine blood studies, cerebrospinal fluid analysis, and electrodiagnostic testing, with criteria for CIDP being those established by the European Neuromuscular Center International Workshop (Neuromusc Disord 2002;12:195–200). Patients with HIV or other infections, monoclonal gammopathy, malignancy, or specific anti-ganglioside antibodies were excluded. All patients received immunomodulatory therapy including intravenous immunoglobulin (IVIG), intravenous or oral prednisone, or azathioprine. Statistical analysis encompassed the Fisher exact test or Pearson's chi-square test, with P < 0.5 considered statistically significant.
Among 28 CIDP patients seen over the study period, seven were children (< 16 years) and 21 were adult. Mean age of onset was 9.7 years in the former vs. 51.8 in the latter, and females predominated in both groups, 57.1% and 71.4%, respectively. Two children had a relapsing course with 1 and 3 episodes, but the remaining five had a monophasic course. Four had motor predominant CIDP, two a sensorimotor type, and 1 a sensory dominant form, with the legs affected in 5, and both arms and legs in two. Prior upper respiratory infection was seen in only two. Cranial nerve involvement was not present in any patient, child or adult. IVIG was used in six children resulting in excellent improvement in all, with two children having a relapse over the 1- to 8-year follow-up period, responding to subsequent steroid therapy with complete, or almost complete, resolution of symptoms. Compared to adults, the children demonstrated more predominant motor involvement of the legs, subacute progression within the first eight weeks, and excellent response to treatment.
Though usually treatment-responsive, not all childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) responds to intravenous immunoglobulin (IVIG), steroid therapy, or plasma exchange (Eur J Ped Neurol 2009;13;209-218). When these modalities prove ineffective, azathioprine, methotrexate, or cyclosporine may be beneficial. Anecdotal case reports using interferon α or β, and monoclonal antibodies against specific B-cell antigens including rituximab and alemtuzumab, have also described efficacy in limited instances. Other potential therapies include mycophenolate mofetil, etanercept (tumour necrosis factor alpha antagonist) and tacrolimus (FK-506). Ironically, aside from treating the disorder, interferon-α, β, TNF-α antagonists, and tacrolimus may also cause a demyelinating polyradiculoneuropathy. Nevertheless, CIDP in children has an overall 70%–100% remission rate, as compared to 65%–70% in adults, with a very good outcome in 83%–85%.