Chronic inflammatory demyelinating polyradiculoneuropathy

Introduction

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the term used to describe chronic progressive and chronic relapsing polyneuropathies associated with inflammation and primary demyelination of the nerves and nerve roots. Austin (1958) gave an early description of recurrent polyneuropathies responsive to corticosteroid treatment. Such responsiveness to corticosteroids is a feature of CIDP. Austin regarded the case described by Targowla (1894) as the first description of relapsing polyneuropathy. Another early description was given by Hinman & Magee (1967): they highlighted the similarity of the chronic disease to the Guillain–Barré syndrome (GBS) and the elevation of cerebrospinal fluid (CSF) protein, which is another typical feature of CIDP. Thomas et al. (1969) and Prineas & McLeod (1976) highlighted the relapsing course of disease and described ‘chronic relapsing polyneuritis’. Later the term ‘chronic inflammatory polyradiculoneuropathy’ was used by Dyck et al. (1975), who also included patients with a progressive course of disease. More recently, the term ‘chronic inflammatory demyelinating polyradiculoneuropathy’ has been accepted. This term is used for patients with relapsing and non-relapsing disease. It is difficult to make a distinction between patients with recurrent attacks of GBS and those with CIDP (Thomas et al., 1969; McCombe, Pollard & McLeod, 1987b).