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J Neurol Neurosurg Psychiatry. Published Online First: 20 March 2008. doi:10.1136/jnnp.2007.142133
Copyright © 2008 by the BMJ Publishing Group Ltd.

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Letters

A case of malignant monophasic multiple sclerosis (Marburg’s disease type) successfully treated with decompressive hemicraniectomy

Jose J González Sánchez 1*, Joaquim Ensenyat Nora 1, Matteo de Notaris 2, Jordi Rumià Arboix 1, Carlos García-Amorena García 1 and Enrique Ferrer Rodríguez 1

1 Hospital Clinic of Barcelone. Clinic Institute of Nervous System Disease. Division of Neurosurgery, Spain
2 Department of Neurological Sciences, División Neurosurgery, Universita Studi di Napoli Federico II, Italy

* To whom correspondence should be addressed. E-mail: jjgcmb{at}hotmail.com.

Accepted 10 March 2008


*  Abstract

Marburg’s disease is a variant of multiple sclerosis which produces a monophasic fulminant course with extended edema and brain herniation refractory to intensive medical treatments and with poor outcome or dead. We report a case of a 31 years old female patient previously healthy who presented an hyperacute onset of pseudotumoral Marburg’s type of Multiple Sclerosis with left hemispheric massive brain swelling. On the seventh day of admission, patient’s intracranial pressure became uncontrolled in spite of medical aggressive treatment. Left decompressive hemicraniectomy allowed intracranial pressure control. Patient had an excellent outcome with an Expanded Disability Status Scale of 2.5.








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