A Comparison of Clinically Atypical with Typical Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract

Background: There is a paucity of prospective studies evaluating the atypical features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Aims: To compare clinical and electrodiagnostic features of clinically typical and atypical CIDP patients. Methods: The patients with typical and atypical CIDP diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society were included. The disability was graded on a 0–10 scale. Neurophysiological study included motor and sensory nerve conduction and F wave studies of both upper and lower limbs. The patients were treated with prednisolone with or without azathioprine. The outcome was evaluated at 6 months and improvement was defined as at least 2 grades improvement. Results: Eight out of 37 CIDP patients had atypical features, which included asymmetry in 2, dysautonomia in 3 and pure motor weakness, amyotrophic-lateral-sclerosis-like syndrome and distal weakness in 1 patient each. Tendon reflexes were retained in 3 patients. The mean duration of symptoms was 43 weeks in the typical and 30 weeks in the atypical group. The age, sex, disability, therapeutic response and neurophysiological features were similar in the 2 groups. Conclusion: About one fifth of CIDP patients may have atypical clinical features; however, their electrodiagnostic features and response to treatment are no different from typical CIDP.

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

“…Two studies found a higher level of disability and impairment in patients with typical CIDP than in patients with LSS and DADS 6 30. Another study did not find a difference in terms of disability between typical and atypical CIDP 31. In our series, patients with typical CIDP had a worse MRC score, higher disability and worse QoL than patients with DADS, confirming that DADS is clinically less disabling than typical CIDP.…”

Motor-dominant chronic inflammatory demyelinating polyneuropathy

“…2 Hand dynamometer values of patients with motordominant CIDP before and after IVIg therapy 2.8 ± 1.5 years, range 1-5 years), the average number of relapses was 5.8 ± 2.2 (n = 5, range 4-9). Within the first year after the disease onset, the average number of relapses was 3.8 ± 1.1 (n = 5, range [2][3][4][5]. From the first to the second year after the disease onset, the average number of relapses decreased to 2.3 ± 1.9 (n = 4, range 1-5).…”

“…The subtypes of CIDP differ in clinical presentation, electrophysiological and laboratory features, and response to treatment [1]. There are several reports on the cases of patients with motor-dominant CIDP who showed selective motor involvement upon clinical and electrophysiological examination [2][3][4]. Furthermore, the clinical, pathological, electrophysiological, and prognostic features of patients with motor-dominant CIDP are poorly understood.…”

Demyelinating Findings in Typical and Atypical Chronic Inflammatory Demyelinating Polyneuropathy: Sensitivity and Specificity

“…8,13 In our study, we further classified a-CIDP in to distal CIDP (d-CIDP) with distal symmetric involvement without proximal weakness 14 and multifocal CIDP (m-CIDP) with asymmetric weakness. 15,16 Distal CIDP is also referred to by some authors as distal acquired demyelinating symmetrical neuropathy without a monoclonal protein-I.…”