Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP), which can occur t hrough life from childhood to old age, presents a wide variety of clinical phenotypes. We investigated the relationship between age of onset and pheno type in 124 CIDP patients. Clinical symptoms, pathologic findings and elect rophysiologic features were assessed according to age at onset: juvenile, y ounger than 20-years-old; adult, 20 to 64; and elderly, older than 64 (tota l n=124). Half of the juvenile group showed subacute progression initially, while mast patients in the elderly group showed chronic insidious progress ion (chi (2)=23.2, P<0.0001). Motor dominant neuropathy was prominent in ju veniles, while sensorimotor neuropathy was frequent in the elderly group (< chi>(2)=27.0, P<0.0001). A relapsing and remitting course predominated in t he juvenile group (<chi>(2)=8.50, P=0.0143). Demyelinating and axonal degen erating features in sural nerve biopsy and in nerve conduction studies were common to three age groups. The subperineurial edema was more prominent in the juvenile and adult groups (P=0.006). Functional recovery was common in all three age groups, but was least apparent in the elderly group (P=0.000 62). Demyelinating features in studies of nerve conduction and biopsy speci mens was common to all three age groups, and was a useful diagnostic featur e. Clinical features of CIDP differ by age of onset, which is a factor to c onsider in diagnosis, therapy, and prognosis. (C) 2001 Elsevier Science B.V . All rights reserved.