Chronic inflammatory demyelinationg polyradiculoneuropathy with and without diabetes mellitus – A retrospective hospital-based study

Abstract

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by relapsing or progressive dysfunction of the motor and/or sensory fibers of the peripheral nervous system due to inflammatory demyelination. Secondary axonal degeneration is important for permanent disability. CIDP is important to recognize as it is potentially treatable. CIDP associated with diabetes mellitus (DM) has been recognized. Aim: To study the clinical, electrophysiological and cerebrospinal fluid (CSF) characteristics of Hong Kong Chinese patients with CIDP; and compare between CIDP patients with and without DM. METHODS: Patients followed in our neurology clinic who fulfilled the diagnostic criteria for CIDP of the Ad Hoc Subcommittee of the American Academy of Neurology AIDS task force and had disease duration of at least 1 year were studied. All patients had standardized electrophysiological studies for diagnosis and progress monitor. A total of 27 CIDP patients were studied. Their mean onset age was 57.9 years (range 23– 80), and 17 were males. Their mean disease duration was 7.1 years (range 1–18 years). Three patients had monoclonal gammopathy. Six patients (22.2%) had relapsing disease. Nine patients (33.3%) had DM (all type 2 DM) with a mean duration of 6.1 years (range 1-20 years) between DM diagnosis and CIDP symptom onset. The mean HbA1c value at CIDP onset was 7.7% (range 6.0–15.1%). Their mean CSF protein level was 1.7 g/L and all had acellular CSF. Their mean onset and worst modified neuropathy impairment score (NIS) were 35.3 and 63.0 respectively. The mean number of conduction blocks (over 4 limbs on standard electrophysiological studies) was 3.8 (range 0–8). 86.7% patients responded to plasmapheresis, 83.3% responded to oral corticosteroids and 83.3% responded to intravenous immunoglobulins (response defined as significant improvement). Three patients (11%) died, 2 of these 3 had monoclonal bands. Among the survivors, clinical outcome was satisfactory in 85% patients who remained ADL independent at latest follow-up, while 15% were ADL partially dependent. 61% of patients required immunosuppressant therapy upon latest follow-up. There is no significant difference in any clinical, electrophysiological and CSF parameters between patients with and without DM. CONCLUSION: One third of our CIDP patients had type 2 DM. There is no difference in clinical, electrophysiological and CSF characterisitics between CIDP patients with and without DM.