Machteld Hillen

A second case of Marburg’s variant of multiple sclerosis with vasculitis and extensive demyelination

Marburg’s variant of multiple sclerosis is a rapidly progressive and malignant form of multiple sclerosis (MS) that usually leads to severe disability or death within weeks to months without remission. Few cases have been described in the literature since the original description by Marburg. The classic pathological findings usually include highly destructive zones of extensive demyelination, necrosis with dense cellular infiltrate, and giant reactive astrocytes. We report a case of a 31-year-old woman with Marburg’s variant of MS who, over a period of eight months, became totally disabled, blind, and quadriplegic, with vocal cord paralysis, requiring a tracheostomy. The patient underwent diagnostic stereotactic brain biopsy. Clinical findings, magnetic resonance imaging (MRI), serologic and cerebrospinal fluid (CSF) findings, and neuropathology are discussed. MRI showed extensive white matter involvement in the brain and spinal cord that continuously progressed over time. A diagnostic stereotactic brain biopsy revealed extensive active demyelination with unexpected finding of active vasculitis and fibrinoid necrosis with a vascular inflammatory cell infiltrate, including polymorphonuclear neutrophils and rare eosinophils. Serologic work-up for vasculitis and neuromyelitis optica was unremarkable and the CSF showed only one oligoclonal band (OCB) not present in serum. This is the second case of Marburg’s variant of MS that demonstrated both demyelination and vasculitis. In our case these features were demonstrated simultaneously, even though the demyelination was the predominant pathological finding. Since vasculitis is not a feature of classic MS, these findings pose the question as to whether Marburg’s variant of MS is a true variant or different entity altogether.

Clinical Reasoning: Corpus callosum lesion with multiple strokes

A 71-year-old woman with hypertension, diabetes, and recent stroke presented with acute left hemiparesis, which started 3.5 hours earlier but improved by the time of emergency department evaluation. Examination showed mild left-sided pronator drift and sensory extinction. Her history was significant for resection of a right frontoparietal meningioma for which she had received adjuvant radiation therapy 3 years previously.

Acute retinal necrosis in multiple sclerosis: A neuroimmunologic challenge!

We report a case of a 32-year-old woman with a 2-year history of multiple sclerosis (MS) who presented to the ophthalmology clinic 1 week after the completion of pulse steroids with complaints of blurred vision associated with pain in the left eye. She was treated with 3 courses of high-dose methylprednisolone (1,000 mg a day for 3 days) for recurrent relapses in the 5 months before presentation (brain MRI, figure, A). Her symptoms during these relapses were left hemiparesis, right hemiparesis, and worsening right hemiparesis, in that order. She was started on glatiramer acetate 1 month before presentation. Given the frequent relapses, changing her disease-modifying therapy to natalizumab was being considered.