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Chronic inflammatory demyelinating neuropathy(CIDP)is a rare disease in childhood.We reviewed the clinical characteristics,response to therapy,and long-term prognosis in 13 children(1.5 to 16 years of age)diagnosed with CIdP at Washington University Medical Center,St.Louis,and the Royal children's Hospital,Melbourne,Australia,between 1979 and 1994.The most common presenting symptom(in 11/13[85%])was lower extremity weakness associated with difficulty in walking.Preceding events within 1 month of onset,mostly intercurrent infections or vaccinations,occurred in seven children(54%).The disease was monophasic in three children(23%).One relapse occurred in four(30%)and multiple relapses in six(46%).All patients had at least short-term response to steroids.Three children(23%) recovered completely during the first year.Ten children(77%)had residual weakness after an average follow-up of 6 years.there seems to be two populations of children with CIDP.One subgroup,with a favorable prognosis, progressed to peak disability over less than 3 months;these children often have a monophasic course with complete resolution of symptoms and signs and withdrawal from all medications by 1 year after onset.A second subgroup progressed for 3 months or longer;these children all required substantial doses of prednisone for prolonged periods and had considerable long-term morbidity with persistent weakness. |
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children
leg weakness,bilateral
paraparesis
polyneuropathy,chronic inflammatory demyelinating
polyneuropathy,chronic inflammatory demyelinating,childhood
prognosis
treatment of neurologic disorder
walking,difficulty with
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