Progressive Solitary Sclerosis: Insidious Motor Impairment Localized to an Isolated CNS Demyelinating Lesion (P3.008)

Objective: To report patients with progressive solitary sclerosis, a progressive motor impairment resulting from a single central nervous system (CNS) demyelinating lesion. Background: Current diagnostic criteria for multiple sclerosis (MS) require dissemination in time and space with multiple MRI lesions. We previously reported MS patients with progressive impairment consistent with MS from a solitary demyelinating cervicomedullary lesion. Methods: We identified 28 patients (23 prospectively identified from our MS clinic and 5 retrospectively identified from our progressive MS database) who met inclusion criteria of: (1) progressive motor impairment >1 year attributable to a single radiologically identified CNS demyelinating lesion along the corticospinal tracts; (2) absence of other demyelinating CNS lesions; and (3) no history of relapses affecting other CNS segments. Patients were excluded if an alternative etiology for progressive motor impairment was found. Multiple brain and spinal cord MRIs were reviewed by a neuroradiologist blinded to the clinical details. Results: The median age was 48.5 years (range, 23-71) and 16 (57[percnt]) were women. All had insidiously progressive upper motor neuron weakness directly corresponding to the solitary MRI demyelinating lesion. Clinical presentations were: hemiparesis/monoparesis, 22; quadriparesis, 4; paraparesis, 1; and spastic-ataxia, 1. Solitary MRI lesions involved: cervical spinal cord, 18; cervicomedullary/brainstem region 6; thoracic spinal cord, 2; subcortical white matter, 2. Median follow-up from symptom onset was 90 months (range 15-343 mos.) and median expanded disability status scale score (EDSS) at last follow up was 6 (range 2-10). Elevations in cerebrospinal fluid oligoclonal bands or immunoglobulin (Ig) G index were found in 13 of 24 (54[percnt]). Isolated demyelinating etiology was confirmed in two with pathology (biopsy, 1; autopsy, 1). Conclusion: Progressive solitary sclerosis results from an isolated CNS demyelinating lesion arising in the cerebral, brainstem or spinal cord white matter. Disclosure: Dr. Keegan has received research support from Terumo BCT. Dr. Kaufman has received personal compensation for activities with SpineThera as a consultant. Dr. Weinshenker has received personal compensation for activities with Novartis, Biogen Idec, Mitsubishi Pharmaceuticals, MedImmune Pharmaceuticals, Chugai, and Chord as a consultant. Dr. Weinshenker has received royalty payments from RSR Ltd. and Oxford Dr. Kantarci has nothing to disclose. Dr. Schmalstieg has nothing to disclose. Dr. Paz Soldan has nothing to disclose. Dr. Flanagan has nothing to disclose.