High-dose cyclophosphamide as a rescue treatment for fulminant Marburg multiple sclerosis: A case report (P12-3.014)

Abstract

<h3>Objective:</h3> To describe the presentation of a life-threatening multiple sclerosis variant and the positive response to cyclophosphamide induction. <h3>Background:</h3> The rare Marburg variant of MS is characterized by life-threatening severity and a fulminant disease course. Many treatment regimens have been proposed to combat this disease process, but the most promising results have been demonstrated with high-dose induction cyclophosphamide 50 mg/kg/day for four days (HiCy). <h3>Design/Methods:</h3> N/a <h3>Results:</h3> A previously healthy 39-year-old woman presented with confusion and aphasia. She rapidly became minimally conscious, only intermittently withdrawing to pain. CSF was unrevealing. MRI brain showed widespread subcortical and infratentorial T2 hyperintense lesions. After diagnosis of acute encephalitis, she was treated with IV steroids, 11 sessions of plasma exchange, and 5 sessions of IVIG with little to no clinical response. Further MR imaging revealed spinal cord and open ring-enhancing juxtacortical lesions. After ruling out alternative causes, she was diagnosed with Marburg variant MS and started on HiCy. As early as day two of treatment, she became alert and oriented and was consistently able to follow commands for the first time. She demonstrated steady clinical improvement thereafter, and was discharged with an EDSS of 1. At three-month follow up, she had ongoing disability-free stability and started ocrelizumab for maintenance therapy. <h3>Conclusions:</h3> Though rare and diagnostically elusive, early recognition of Marburg and initiation of rescue treatment with cyclophosphamide can be lifesaving. Given its many potential adverse reactions, the decision to use of HiCy should not be taken lightly and must be weighed against other treatment modalities. We propose that despite these risks, the risk of not treating or delaying treatment in the face of fulminant MS is far greater. This case represents the swiftest and most significant clinical recovery (EDSS 9 to 0) in a patient with Marburg MS treated with HiCy. <b>Disclosure:</b> Dr. Roberts has received intellectual property interests from a discovery or technology relating to health care. Dr. Abdullah has nothing to disclose. Miss Shoga has nothing to disclose. Dr. Reyna has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Reyna has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Reyna has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Reyna has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Medtronic. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Celgene. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Sanofi. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Novartis. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Serono. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion. Dr. Reyna has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Bristol Myers Squibb. An immediate family member of Dr. Parker has received personal compensation for serving as an employee of Rochal Industries, Rochal Parners. An immediate family member of Dr. Parker has received personal compensation in the range of $5,000-$9,999 for serving as an officer or member of the Board of Directors for Sanara Med Tech. An immediate family member of Dr. Parker has stock in Sanara Med Tech. An immediate family member of Dr. Parker has stock in Rochal Industries. An immediate family member of Dr. Parker has stock in Rochal Parnters. The institution of Dr. Parker has received research support from Texas Alzheimer’s Research and Care Consortium. The institution of an immediate family member of Dr. Parker has received research support from Rochal Industries. An immediate family member of Dr. Parker has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Parker has received publishing royalties from a publication relating to health care. Dr. Parker has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Speaker with Univ of Texas Tyler. An immediate family member of Dr. Parker has received personal compensation in the range of $0-$499 for serving as a NIH Review Committees Member with NIH. An immediate family member of Dr. Parker has a non-compensated relationship as a Chairwoman of the Board - Ann Salamone with BioMed SA that is relevant to AAN interests or activities.