Abstract
Marburg’s variant of multiple sclerosis (MS) is a malignant and rapidly progressive form of MS that typically leads to deterioration or death within weeks to months. Here we present a case involving a 25-year-old woman who presented with fluctuating mild encephalopathy of one-week duration who progressed to coma and ultimately died on day seven after being admitted to our universitybased neurosciences intensive care unit (NSICU). During her stay, numerous studies were completed including a brain MRI with gadolinium which revealed multiple diffuse ring-enhancing lesions consistent with a fulminant demyelinating process, which was later supported by autopsy findings. CSF studies and serologies were essentially unremarkable. The rarity of Marburg’s variant of MS has resulted in a scarcity of literature and lack of clinical recommendations regarding management. After review of literature and with consideration of our case report, even when marked improvement following IV methylprednisolone is observed, adjunct therapy with a second modality should be considered to improve the chance of survival.
Highlights
Marburg’s variant of multiple sclerosis (MS) can be considered the most fulminant, and often fatal form of MS variant that typically leads to deterioration or death within weeks to months [1]
After review of literature and with consideration of our case report, even when marked improvement following IV methylprednisolone is observed, adjunct therapy with a second modality should be considered to improve the chance of survival
Marburg first described a malignant form of MS in 1906 with three cases of fulminant demyelinating disease.The first patient he described was a previously healthy, 30-year-old woman who suddenly developed confusion associated with headaches, vomiting, gait abnormality, left hemiparesis, and hyperreflexia, and died within one year [2]
Summary
Marburg’s variant of MS can be considered the most fulminant, and often fatal form of MS variant that typically leads to deterioration or death within weeks to months [1]. MRI brain revealed numerous T2/ FLAIR hyper intense lesions in the bilateral cerebral white and gray matter, brain stem, and cerebellum (Figure 1). These lesions demonstrated incomplete ring-enhancement on T1 weighted imaging with gadolinium and peripheral restricted diffusion on DWI (Figure 2). On hospital day four she started showing signs of herniation She was intubated and despite maximal medical management which included placement of an external ventricular drain and hyperosmolar therapy, she died on hospital day seven. Gross examination of the formalin-fixed brain showed marked and diffuse edema (brain weight – 1518g), with numerous white matter lesions involving all lobes of the cerebrum (Figure 3).
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