Chronic inflammatory demyelinating polyneuropathy : clinical, electrophysiological and morphological study

Twelve patients (M:F 9:3) who fulfilled diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP) were seen at NIMHANS over a period of three years (1987-1990). Their ages ranged from 20 yrs to 71 yrs and the mean duration of symptoms was 30 months (range 3 months to 6 yrs). Symptoms at the onset were dependent on the duration of disease. These included paraesthesia (7), weakness (4) and ataxia in lower limbs (1). Salient features on examintion were: distal weakness (10), proximal weakness (6), impaired touch and pain (12), impaired joint position and vibration sense (6), distal areflexia (12), bilateral impaired hearing (2) and thickened nerves (4). Electrophysiological evidence of demyelination was present in all and albumino cytological dissociation in CSF was noted in 55 of the patients. Sural nerve biopsy revealed significant loss of myelinated fibres in all the five patients studied. Increase in endo and perineural collagen, remyelination and schwann cell proliferation were also seen. Inflammatory infiltrates were conspicuously absent. Steroids were given in 10 patients. The therapeutic response was good in 5 and moderate in 5. Two patients had remitting relapsing course. Response to steroids could not be predicted on the basis of clinical or laboratory features. The recent diagnostic criteria and their therapeutic relevance are discussed.