Cyclophosphamide as initial treatment of aggressive MS (Marburg variant) in resource limited settings – A case report

Abstract

The Marburg variant of multiple sclerosis (MS), first described in 1906 by Otto Marburg, an Austrian neurologist, is characterized by a fulminant monophasic course with rapid disease progression, often leading to death within weeks or months. Due to its rarity, no established treatment guidelines exist. A 34-year-old man was diagnosed with MS after presenting with acute-onset numbness and weakness in the right lower limb. Magnetic resonance imaging (MRI) revealed a significant burden of numerous supra- and infratentorial white matter plaques. The patient was treated with steroid pulse therapy. However, the patient’s condition continued to deteriorate clinically and radiologically despite receiving intravenous immunoglobulin and plasma exchange. An extensive workup was performed to exclude other differential diagnoses that were significant for the presence of oligoclonal bands (OCBs) in the cerebrospinal fluid (CSF). A diagnosis of Marburg variant MS was considered, and high-dose cyclophosphamide (HiCy) (50 mg/kg/d IV) was initiated on alternate days for 4 days. Twenty-six days after treatment completion, the patient exhibited significant clinical improvement, with an Expanded Disability Status Scale (EDSS) score of 5. This case contributes to the growing evidence that cyclophosphamide may be an effective therapeutic option for patients with Marburg variants of MS who do not achieve satisfactory clinical improvement with conventional acute treatments.