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Abstract Details

Rapidly Progressive Demyelinating Disease Secondary to the Marburg Variant of Multiple Sclerosis: A Case Report
Multiple Sclerosis
P9 - Poster Session 9 (5:30 PM-6:30 PM)
12-004
The Marburg variant is a rare manifestation of multiple sclerosis characterized by a rapid course typically resulting in severe debility or death in weeks to months. Although initially described by Otto Marburg in 1906, available medical literature remains limited.
To report a case of rapidly progressive neurological deterioration due to the Marburg variant of multiple sclerosis.
NA
A 55-year-old female with history of hypertension, hyperlipidemia, type 2 diabetes mellitus, cervical cancer, and subdural hematoma presented with 10-day history of right-sided facial numbness, ophthalmoplegia, and bilateral blurred vision. Exam revealed bilaterally impaired visual acuity to finger counting, left conjugate horizontal gaze palsy, right internuclear ophthalmoplegia (one-and-a-half syndrome), right peripheral facial nerve paresis, and diffuse hyperreflexia. LP showed a lymphocytic pleocytosis with elevated oligoclonal bands and kappa free light chains. MRI revealed multifocal contrast-enhancing periventricular, brainstem, and spinal cord lesions. MOG and aquaporin-4 antibodies were negative. She was initiated on high-dose IV steroids and an oral taper regimen with significant improvement.

One week after discharge she was readmitted for worsening visual acuity, ophthalmoplegia, dysarthria, dysphagia, and gait instability. MRI revealed progression of the prior lesions with new contrast-enhancing brainstem and periventricular lesions. Serial LPs showed persistent lymphocytic pleocytosis without evidence of malignancy. She underwent a second course of high-dose IV steroids, followed by plasmapheresis without improvement. Stereotactic brain biopsy was inconclusive. Repeat brain biopsy of a large periventricular lesion demonstrated a demyelinating lesion with relative axonal preservation and no evidence of malignancy. Based on her clinical course and pathologic findings, she was diagnosed with Marburg variant of multiple sclerosis. Monthly cyclophosphamide was initiated with gradual improvement.
The Marburg variant of multiple sclerosis is an aggressive, demyelinating disease with a poor prognosis. Unfortunately the medical literature regarding this disease remains sparse. Early tissue biopsy is key to exclude alternative diagnoses.
Authors/Disclosures
Natalie Bartnik, DO (University of Kansas Medical Center)
PRESENTER
Dr. Bartnik has nothing to disclose.
David M. Anson, MD (University of Kansas Medical Center Department of Neurology) Dr. Anson has nothing to disclose.
Daniah Shamim, MD (University of Kansas Medical Center) Dr. Shamim has nothing to disclose.
Yunxia Wang, MD, FAAN (KUMC) Dr. Wang has nothing to disclose.