Chronic Inflammatory Demyelinating Polyradiculoneuropathy, in an 8-Year-Old Girl, Complicated by Deafness and Kidney Fibrosis

Abstract

Based on case history and clinical and electrophysiological examinations, the authors report on a case of an 8-year-old girl who was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy. The disease was complicated by deafness and kidney fibrosis. During treatment with methylprednisolone and intravenous immunoglobulin, followed by mycophenolate mofetil, prompt improvement of neurological findings occurred. The improvement of hearing was poor. Because the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy has still not been clear, and on the grounds of several cases of chronic inflammatory demyelinating polyradiculoneuropathy conjoined with the kidney disease described in literature (glomerulopathy, interstitial nephritis), every patient with chronic inflammatory demyelinating polyradiculoneuropathy needs to undergo the urinalyses.

Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges, controversies, and questions

“…[ 12 13 ] Data for efficacy and tolerability of other immunomodulating agents such as azathioprine, cyclosporine A, mycophenolate mofetil, methotrexate, cyclophosphamide, and rituximab are anecdotal and mixed. [ 2 5 13 14 15 16 17 ]…”

Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

“…Other oculomotor symptoms comprised afferent pupillary defect and partial right VIth nerve palsy, ptosis, abduction deficit and unilateral tonic pupillary dilatation (20,33). Bulbar disorders resulted in dysphagia, and mastication, hypophonia and lingual fasciculations (31,34,35). Generalized facial weakness and dysarthria have also been shown (30)(31)(32)34).…”

“…The most frequently used dosage is a course of 0.4 g/kg/day for five days, either a single treatment or repeated every three to four weeks (19,20,22,(54)(55)(56). Some groups prefer to administer 2 g/kg over two (25,57) or three days (58) or 1 g/kg during two days per month (35,59). Others prefer 0.4 g/kg/alternate day (combined with plasma exchange) or weekly infusions of 0.8 g/kg (60).…”

“…Its adverse effects included electrolyte disorders, crises of hypertension, vomiting, edema, renal failure, and hirsutism (71). Mycophenolate mofetil was designated to result in a partial response in one patient; in association with corticosteroids, it improved motor power in an eight-year-old girl (25,35). Methotrexate, a folic acid analogue blocking DNA creation, showed notable response in a one-year-old boy for two years after discontinuing azathioprine due to vomiting and no benefit from cyclosporine (14).…”

Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

“…Generalized facial weakness and dysarthria have also been shown (30)(31)(32)34). Sensorineural deafness was defined in one patient (35).…”