CLINICAL CHARACTERISTICS OF PATIENTS WITH CHRONIC ACQUIRED DEMYELINATING POLYNEUROPATHY

Chronic acquired demyelinating neuropathy (CADP) is heterogeneous in both clinical and laboratory features. This study was performed to define the clinical, electrodiagnostic and histological findings, course and response to therapy in patients with CADP. Thirty patients (20 men and 10 women) with CADP were studied. Diagnostic criteria were based on clinical presentation, clectrophysiological studies, cerebrospinal fluid (CSF) protein level and sural nerve biopsy findings. Response to treatment was assessed by changes in average muscle score (AMS). Twenty-one patients conformed to the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP) and 9 to distal acquired demyelinating symmetric neuropathy (DADS). The course was monophasic in 6 (23%), relapsing in 10 (40%) and chronic progressive in 8 (30%); 4(13%) had acute presentation with subsequent progression or relapsing course. Motor nerve conduction velocity (MNCV) of less than 70% and greater than 70% of normal were seen in 18 (60%) and 12 (40%) patients, respectively. Conduction block was observed in 14 (47%) and CSF protein levels were elevated in 19 patients (66%). Demyelination was reported in 61% and 58% of the biopsies performed in patients with MNCV 70% and 70% of normal, respectively. The association between MNCV and histologic findings was not significant. Twenty-one patients were treated with intravenous immunoglobulin (IVIg). Fifteen patients (83%) with CIDP had significant improvement in AMS following the initial IVIg treatment (P = 0.01). This study highlights the heterogeneity of clinical and laboratory findings in CADP and the importance of early treatment