CLINICAL CHARACTERISTICS OF PATIENTS WITH CHRONIC ACQUIRED DEMYELINATING POLYNEUROPATHY

Abstract

Chronic acquired demyelinating neuropathy (CADP) is heterogeneous ill both clinical and laboratory features. This study was performed to define the clinical. clccuodiagnostic and histological findings. course and response 10 therapy in patients with CADI'. Thirty patients (20 men and 10 women) with CADI' were studied. Diagnostic criteria were based on clinical presentation. clcctrophysiolcgical studies. cerebrospinal fluid (CSF) protein level and sural nerve biopsy findings. Response 10 treatment was assessed by changes in average muscle score (A:vlS). Twenty-one patients conformed to the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (Cf Dl") and 9 to distal acquired demyelinating symmetric neuropathy (DADS). The course was monophasic in Cl (23~/~-)), relapsing in I0 (40(~/;1) and chronic progressive in 8 (30':••;,): 4( 13°•'()) had ucutc presentation with subsequent progression or relapsing course. Motor nerve conduction velocity (i"--INCV) of less than 70°,-() and greater than 70'~;(, of normal were seen in 18 (60'~';)) and 12 (40{~-;1) patients. respectively. Conduction block was observed in 14 (47(~/o) and CSF protein levels WCl"C elevaled in 19 patients (66':--;)). Demyelination was reported in 61(;--() and 58%) of the biopsies performed in patients with MNCV <: 70'~";l and> 70'}'() of normal. respectively. The association between "•lNCV and histologic findings was no! significant. Twenty-one patients were treated with intravenous immunoglobulin (lVlg). Fifteen patients  83(;-{1) with ClDP had significant improvement in AfvlS following the iuitial fVlg treatment (P n.ol). This study highlights the heterogeneity of clinical and laboratory findings in C:"I)P and the importance of early treatment.