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.