Abstract
IntroductionWe aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) with and without diabetes.MethodsCIDP patients with diabetes (CIDP+DM) (n = 67) and without diabetes (CIDP-DM) (n = 67) underwent clinical examination and nerve conduction studies (NCS). CIDP-DM patients were selected using age and gender matching with the existing CIDP+DM cohort. Patients treated with immunotherapies were classified as responders (R) (n = 46) or non-responders (NR) (n = 54) based on clinical response to treatment. The groups were compared using analysis of variance, contingency tables and Kruskal-Wallis analyses.ResultsCIDP+DM subjects had more severe neuropathy based on higher lower limb vibration potential thresholds (VPT)(p = 0.004), higher Toronto Clinical Neuropathy Score (TCNS) (p = 0.0009), more proximal weakness (p = 0.03), more gait abnormality (p = 0.03) and more abnormal NCS. CIDP+DM subjects had more abnormal sural NCS with lower sural sensory nerve action potential amplitudes (2.4±3.0 µV, 6.6±6.0 µV, p<0.0001) and slower sural nerve conduction velocities (38.6±5.4 m/s, 41.0±5.3 m/s, p = 0.04). CIDP-DM subjects were more likely to receive immune therapies (93% vs 57%, p = <0.0001), despite no significant differences in treatment responder rates (p = 0.71). Patients who responded to therapy had shorter duration of CIDP than non-responders (8.0±6.0 y vs 11.9±7.6 y, p = 0.004).DiscussionThe clinical phenotype and electrophysiological profile of CIDP patients differs according to the presence or absence of diabetes. Despite CIDP+DM patients having more severe clinical and electrophysiological neuropathy, they are less likely to receive disease-modifying/specific therapy, yet have similar response rates to treatment as those without diabetes. Specifically, the duration of neuropathy - not diabetes status - was associated with treatment response.
Highlights
We aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) with and without diabetes
We examined a matched cohort of CIDP patients with and without diabetes to compare their clinical characteristics, electrodiagnostic classification of nerve injury and response to treatment
CIDP+diabetes mellitus (DM) patients had more severe neuropathy and a similar treatment response rate compared with CIDP-DM patients as observed by Gorson [5], but were less likely to receive treatment
Summary
We aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) with and without diabetes. CIDP results in demyelination of peripheral nerves, as indicated by a significant reduction of motor conduction velocity and prolonged distal motor latencies [1,2]. Others have suggested that in diabetes patients, the presence of two electrodiagnostic abnormalities consistent with demyelination supports a diagnosis of CIDP, compared to only one such abnormality for non-diabetes patients [12]. This diagnostic challenge underscores the importance of distinguishing CIDP from DSP in diabetes patients, as the former may be amenable to treatment with immunomodulatory therapies, including intravenous immunoglobulin (IVIg), corticosteroids and plasma exchange, even in the presence of an underlying DSP [3,12]
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