Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy

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Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP), which can occur through life from childhood to old age, presents a wide variety of clinical phenotypes. We investigated the relationship between age of onset and phenotype in 124 CIDP patients. Clinical symptoms, pathologic findings and electrophysiologic features were assessed according to age at onset: juvenile, younger than 20-years-old; adult, 20 to 64; and elderly, older than 64 (total n=124). Half of the juvenile group showed subacute progression initially, while most patients in the elderly group showed chronic insidious progression (χ2=23.2, P<0.0001). Motor dominant neuropathy was prominent in juveniles, while sensorimotor neuropathy was frequent in the elderly group (χ2=27.0, P<0.0001). A relapsing and remitting course predominated in the juvenile group (χ2=8.50, P=0.0143). Demyelinating and axonal degenerating 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=0.00062). Demyelinating features in studies of nerve conduction and biopsy specimens was common to all three age groups, and was a useful diagnostic feature. Clinical features of CIDP differ by age of onset, which is a factor to consider in diagnosis, therapy, and prognosis.

Introduction

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a polyneuropathy of presumed autoimmune pathogenesis showing insidious development over weeks to years [1], [2], [3], [4]. Clinically, CIDP shows generally symmetrical motor and sensory deficits in the limbs with a limited degree of autonomic involvement, and either a chronic progressive or relapsing course with a variable degree of residual disability [3], [4], [5]. The diagnosis of CIDP usually is confirmed by electrophysiologic studies showing a demyelinating polyneuropathy with variable involvement of different nerves [6]. Several clinical and pathologic descriptions of CIDP [1], [3], [4] have indicated that CIDP has a wide variety of phenotypes that vary as to the degree of axonal involvement, motor and sensory deficits, clinical course, long-term prognostic features, and response to treatment.

CIDP can begin at any age, even in early childhood [7], [8], [9], [10], [11]. Our group and several others [12], [13], [14], [15] have documented that childhood-onset CIDP differs in clinical expression from CIDP beginning later in life, with more rapid initial progression, more favorable response to corticosteroid therapy, and a more favorable prognosis. Although characteristic features of CIDP occurring in the elderly have not been well characterized, age is believed to influence CIDP phenotypic expression in late-onset CIDP. Furthermore, CIDP with onset in old age occasionally is misdiagnosed as an age-related neuropathy such as diabetic or compression neuropathy. We examined clinicopathologic and prognostic features at different ages of onset in a large series of CIDP patients, and found significant age-dependent differences in phenotypic expression.

Section snippets

Patients

Clinical records were reviewed for all patients seen in the Nagoya University Hospital and its affiliated hospitals between 1989 and 1998 who were diagnosed as CIDP. The diagnosis of CIDP was based on the criteria adopted by the Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force in 1991 [6]. The patients numbered 124 (81 males, 43 females) and ranged in age from 2 to 90 years. None had a history of exposure to toxic agents or a family history of neuropathy. Patients with

Results

Clinical details of 124 CIDP patients were summarized in Table 1. The male:female ratio and follow-up period were substantially similar among the three age groups. Motor and sensory impairments were more prominent in lower than upper extremities in all age groups. Muscular atrophy was observed in 17% of patients, slightly more predominant in the elderly group. Patients with objective sensory loss showed impairment of both deep and superficial sensation as well as complaints of numbness.

Discussion

The present study demonstrated significantly different clinical and pathologic manifestations of CIDP according to age at onset. The juvenile-onset group showed initial rapid progression, motor predominance, greater likelihood of relapses, worse functional scores at peak severity, and better recovery. On the other hand, CIDP with onset in elderly individuals was characterized by chronic insidious progression in the early phase, combined motor-sensory or sensory involvement patterns, less

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