Chronic inflammatory demyelinating polyneuropathy (CIDP)‎ : Correlation of clinical profile and distribution pattern of electro-diagnostic tests with tumor necrosis factor-alpha

Abstract EN

Background : chronic inflammatory demyelinating polyneuropathy (CIDP) is a term applied to a spectrum of immune– mediated demyelinating neuropathy that are heterogeneous in clinical manifestations and probably pathogenesis.

Objectives : to investigate whether an increased cytokine expression is correlated with clinical profile and quantitative electro diagnostic criteria for primary demyelination in CIDP patients.

Methodology : motor nerve conduction studies were carried out on 25 patients with clinically definite CIDP.

The patients were evaluated clinically using Hughes functional scale, MRC muscle scale and sensory scale.

Control data were obtained from 20 age and sex matched healthy subjects.

According to neurophysiological criteria for demyelination, patients were classified into distal, intermediate, and diffuse patterns.

The serum concentration of TNF-α was measured by immunoassay in both patients and control.

Results : patients were classified as having distal (n = 8), intermediate (n = 6), diffuse (n = 11) patterns.

A number of clinical profiles were significantly different among the patients groups.

Duration of illness was significantly longer for patients with intermediate pattern, whereas the diffuse group showed significantly higher Hughes functional and sensory scales and non-significantly lower muscle scale.

Abnormal median- normal sural sensory response (AMNSSR) was detected in 11 (44 %) CIDP patients.

Serum level of TNF-α was elevated in 10 (40%) of 25 CIDP patients, most of them were of diffuse pattern (90 %).

The serum level of TNF-αwas correlated with Hughes and MRC scales.

The best response to steroid therapy was achieved in patients with diffuse (90.9 %) and distal (87.5 %) patterns, whereas patients with intermediate pattern were relatively refractory to treatment (33.3 %).

Patients with shorter duration, more severe clinical scales, and lower TNF-α serum level responded better to steroid therapy.

Conclusions : there are different distribution patterns of conduction abnormalities in CIDP patients correlated with clinical profiles and serum level of TNF-α.

This may be useful in prediction of response of CIDP patients to therapy.