Andrew J.M.

Boulton, MD, FRCP

Chronic Inflammatory Demyelinating Polyneuropathy and Diabetes

Haq RU, Pendlebury WW, Fries TJ, Tandan R: Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients. Muscle Nerve 2003, 27:465470.
Rating: Of importance. Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) has been increasingly recognized in recent years and comprises a clinical picture of a symmetrical, predominantly motor polyneuropathy with proximal and distal weakness in the lower limbs, with reduced reflexes, that has a relapsing or progressive course. Electrophysiologic (EP), clinical, cerebrospinal fluid, and histologic criteria for CIDP have been published and it seems to occur more commonly in diabetic patients [1]. There is also a suggestion that diabetic patients with CIDP may respond to immunomodulatory treatment [2]. Aims: The aim of the present study was, in a retrospective review, to determine whether a subset of diabetic patients with demyelinating polyneuropathy was similar in clinical, EP, and histologic findings to those with CIDP. Responses to immunomodulatory therapy were also compared. Methods: The authors undertook a medical record review of all patients with a clinical and EP diagnosis of polyneuropathy seen at their center over a 15-year period. They identified 10 patients who met clinical criteria and at least one EP criterion for the diagnosis of CIDP (Group A): all had undergone a sural nerve biopsy. Group B comprised a group of nine diabetic patients with a diagnosis of polyneuropathy who had also 1) undergone a sural nerve biopsy, and 2) had at least one of the EP criteria for the diagnosis of CIDP. Comparisons were made between the two groups. Results: Nondiabetic patients with CIDP were younger than those diabetic patients with CIDP (41.3 vs 55.3 years), but there were no other differences between the groups who had similar clinical features and functional disabilities. With minor exceptions, EP and histologic findings were similar between the two groups. A majority of patients in each group received immunomodulating treatment for the CIDP comprising steroids alone or in combination with plasmapheresis, intravenous
immunoglobulin, or azathioprine. Those with mild functional disabilities did not receive such treatment. A favorable response to treatment was reported in all treated patients.

Conclusions: The authors concluded that an immunemediated demyelinating polyneuropathy is a well-recognized entity in diabetic patients, and for those with the clinical and EP features and significant functional deficits, immunomodulating therapy should be considered.

Editors comments
Although not strictly a clinical trial, I elected to include this paper to raise awareness among endocrinologists that within the collective condition known as diabetic somatic polyneuropathy, there may be a minority of patients who may benefit from specific treatments. CIDP should be suspected in neuropathic diabetic patients if 1) there is a prominence of motor symptoms and signs involving distal or proximal lower limb muscles or upper limbs; 2) after some years of a predominant sensory neuropathy, a motor neuropathy develops with progressive symptoms or signs; or 3) patients are diagnosed with amyotrophy (proximal motor neuropathy). However, the diagnosis should not be made on clinical grounds alone: there are characteristic EP criteria and some patients may require examination of nerve histology or cerebrospinal fluid [1,3,4]. Referral to a neurologist with an interest in peripheral nerve disorders is indicated together with a detailed EP examination. The reason to consider a diagnosis of CIDP in neuropathic diabetic patients with these atypical features is becoming increasingly clear: a response to specific immunomodulatory therapy (usually intravenous immunoglobulin) may, in some cases, be dramatic in terms of clinical improvement [2,5]. There is now an urgent need for good qualitycontrolled clinical trials of immunosuppressive agents in CIDP [6], especially in diabetic patients.

References
1. 2. Sharma KR, Cross J, Farronay O, et al.: Demyelinating neuropathy in diabetes mellitus. Arch Neurol 2002, 59:758765. Sharma KR, Cross J, Ayyar DR, et al.: Diabetic demyelinating polyneuropathy responsive to intravenous immunoglobulin therapy. Arch Neurol 2002, 59:751757.

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Nicolas G, Maisonobe T, Le Forestier N, et al.: Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2002, 25:2630. Rotta FT, Sussman AT, Bradley WG, et al.: The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2000, 173:129139.

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Cocito D, Ciaramitaro P, Isoardo G, et al.: Intravenous immunoglobulin as first treatment in diabetics with concomitant distal symmetric axonal polyneuropathy and CIDP. J Neurol 2002, 249:719722. Hughes RA: Systematic reviews of treatment for inflammatory demyelinating neuropathy. J Anat 2002, 200:331339.