Evolution and long term outcome in patients presenting with large demyelinating lesions as their first clinical event

Abstract

Objectives: To describe the long term evolution and outcome of a homogeneous cohort of patients (n=14) with large demyelinating lesions (LDLs) as the first clinical event from a group of idiopathic inflammatory demyelinating diseases of central nervous system.

Methods: Detailed review of LDLs from December 2002-January 2007 was made. Patients had at least two magnetic resonance imaging (MRI) and minimum follow-up of 2years. The disability was assessed using Estimated Expanded Disability Status Scale (EDSS) and Rappaport Disability Rating Scale (DRS) at onset and last follow-up.

Results: Fourteen consecutive LDL patients (male=7), with mean age 32.7years and mean follow-up of 45.5months were included. Motor deficits (79%) and cognitive symptoms (43%) marked the onset, none had optic neuritis. All except two responded to steroids. Follow-up MRI showed complete resolution in 43%, 57% showing marked reduction in size. On follow-up, 2 relapsed. Mean EDSS and DRS at presentation were 5.93 and 9.07 and at last follow-up were 1.75 and 2.25 (p<0.001).

Conclusions: Our data suggests that, patients presenting with LDLs as their first clinical event behaves distinctly in their presentation, imaging characteristics, prognosis and long term outcome as compared to MS and ADEM. Albeit significant disability at the onset, these patients show an excellent response to treatment with good functional recovery in long term and rare relapses.