Contributions
Abstract: EP1785
Type: ePoster
Abstract Category: Therapy - disease modifying - 32 Others
Background: Marburg´s variant of MS (MVMS) is characterized by acute and extensive demyelination, with a typical monophasic fulminant course usually leading to death within few weeks. There is no standard treatment strategy for MVMS. Usually high dose intravenous steroids represent the first line option followed by plasma exchange or intravenous immunoglobulins. The only data regarding the use of immunosuppressants refer to mitoxantrone and cyclophosphamide, which may slow or even stop the course of the disease.
Objectives: We describe the case of a woman who came to our attention for a severe neurological presentation characterized by very rapidly worsening left hemiplegia, vision loss and cognitive impairment. The rapid clinical deterioration, the extensive involvement of periventricular white matter and brainstem and the laboratory data were highly suggestive of an active inflammatory demyelinating disease. A wide spectrum of differential diagnosis were investigated, including acute disseminated encephalomyelitis (ADEM), Balo's concentric sclerosis, neuromyelitis optica spectrum disorders (NMOSD) and others infectious diseases.
Results: Following exclusion of other possible etiologies, a diagnosis of Marburg type multiple sclerosis was made. The patient was treated with high dose steroids iv without improvement and then with plasma exchange but she became poorly responsive to verbal and pain stimulation, and developed right hemiplegia associated to focal motor seizures. Considering the high efficacy on relapse rate and disability accumulation in relapsing-remitting MS, treatment with alemtuzumab was started. In the following weeks an improvement of the clinical and MRI picture was observed: the patient became awake, responsive to external stimuli, provided verbal responses and showed initial motor recruitment of the right arm.
Conclusions: To the best of our knowledge, this is the first reported case of Marburg type multiple sclerosis treated with alemtuzumab. Although a delayed effect of immunotherapies administered prior to alemtuzumab could not be ruled out, this hypothesis seems unlikely considering the continuous worsening of clinical and MRI picture after more than two weeks from steroids and plasma exchange initiation.
Disclosure:
Massimiliano Calabrese received payment for development of educational presentations including service on speakers bureaus by Biogen-Elan, Genzyme, TEVA, Bayer-Schering, he received travel/accomodation expenses by Novartis Pharma, Genzyme, Biogen idec, Merck Serono, Bayer-Schering, TEVA, and Advisory Board membership by Bayer-Shering, Genzyme, Biogen Idec.
Alberto Gajofatto received payment for participating in advisory board and travel support from Merck.
Abstract: EP1785
Type: ePoster
Abstract Category: Therapy - disease modifying - 32 Others
Background: Marburg´s variant of MS (MVMS) is characterized by acute and extensive demyelination, with a typical monophasic fulminant course usually leading to death within few weeks. There is no standard treatment strategy for MVMS. Usually high dose intravenous steroids represent the first line option followed by plasma exchange or intravenous immunoglobulins. The only data regarding the use of immunosuppressants refer to mitoxantrone and cyclophosphamide, which may slow or even stop the course of the disease.
Objectives: We describe the case of a woman who came to our attention for a severe neurological presentation characterized by very rapidly worsening left hemiplegia, vision loss and cognitive impairment. The rapid clinical deterioration, the extensive involvement of periventricular white matter and brainstem and the laboratory data were highly suggestive of an active inflammatory demyelinating disease. A wide spectrum of differential diagnosis were investigated, including acute disseminated encephalomyelitis (ADEM), Balo's concentric sclerosis, neuromyelitis optica spectrum disorders (NMOSD) and others infectious diseases.
Results: Following exclusion of other possible etiologies, a diagnosis of Marburg type multiple sclerosis was made. The patient was treated with high dose steroids iv without improvement and then with plasma exchange but she became poorly responsive to verbal and pain stimulation, and developed right hemiplegia associated to focal motor seizures. Considering the high efficacy on relapse rate and disability accumulation in relapsing-remitting MS, treatment with alemtuzumab was started. In the following weeks an improvement of the clinical and MRI picture was observed: the patient became awake, responsive to external stimuli, provided verbal responses and showed initial motor recruitment of the right arm.
Conclusions: To the best of our knowledge, this is the first reported case of Marburg type multiple sclerosis treated with alemtuzumab. Although a delayed effect of immunotherapies administered prior to alemtuzumab could not be ruled out, this hypothesis seems unlikely considering the continuous worsening of clinical and MRI picture after more than two weeks from steroids and plasma exchange initiation.
Disclosure:
Massimiliano Calabrese received payment for development of educational presentations including service on speakers bureaus by Biogen-Elan, Genzyme, TEVA, Bayer-Schering, he received travel/accomodation expenses by Novartis Pharma, Genzyme, Biogen idec, Merck Serono, Bayer-Schering, TEVA, and Advisory Board membership by Bayer-Shering, Genzyme, Biogen Idec.
Alberto Gajofatto received payment for participating in advisory board and travel support from Merck.