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Vol. 24. Num. 5.September - October 2013
Pages 191-236
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Vol. 24. Num. 5.September - October 2013
Pages 191-236
Case report
DOI: 10.1016/j.neucir.2013.02.008
Tumefactive multiple sclerosis requiring emergency craniotomy: Case report and literature review
Esclerosis múltiple pseudotumoral que precisa craneotomía urgente: caso clínico y revisión de la literatura
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Pablo M. Munarriza,??
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pablommunarriz@gmail.com

Corresponding author.
, Ana M. Castaño-Leona, Rafael Martinez-Pereza, Aurelio Hernandez-Lainb, Ana Ramosc, Alfonso Lagaresa
a Department of Neurosurgery, University Hospital 12 de Octubre, Complutense University of Madrid, School of Medicine, Madrid, Spain
b Department of Neuropathology, University Hospital 12 de Octubre, Complutense University of Madrid, School of Medicine, Madrid, Spain
c Department of Neuroradiology, University Hospital 12 de Octubre, Complutense University of Madrid, School of Medicine, Madrid, Spain
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Abstract

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system, characterized by focal neurological dysfunction with a relapsing and remitting course. Tumor-like presentation of MS (or “tumefactive”/“pseudotumoral” presentation) has been described before with a certain frequency; it consists of a large single plaque (>2cm) with presence of edema and mass effect and it is hard to distinguish from a brain tumor. However, we present a very rare case of a 53-year-old woman with a right temporal mass that turned out to be a MS plaque, who deteriorated within hours (brain herniation with loss of consciousness and unilateral mydriasis) and required an emergency craniotomy. We also present a review of the literature. It appears that only 4 cases of emergency craniotomy/craniectomy required in a patient with a tumor-like MS plaque have been reported before.

Keywords:
Multiple sclerosis
Tumefactive multiple sclerosis
Pseudotumoral multiple sclerosis
Resumen

La Esclerosis Múltiple (EM) es una enfermedad desmielinizante del sistema nervioso central, que se caracteriza por déficits neurológicos con un curso recurrente-remitente. La presentación pseudotumoral de la esclerosis múltiple ha sido descrita en la literatura con cierta frecuencia, se define como la presencia de una única placa desmielinizante grande (>2cm) con edema asociado y efecto de masa, que puede confundirse fácilmente con un tumor cerebral entre otros diagnósticos alternativos. Presentamos el caso de una mujer de 53 años con una lesión en el lóbulo temporal derecho que resultó ser una placa de desmielinización aguda, la singularidad del caso reside en que la paciente deteriora neurológicamente en pocas horas (coma y midriasis ipsilateral) requiriendo una intervención quirúrgica urgente. En la revisión de la literatura que hemos llevado a cabo, solo hemos encontrado publicados otros cuatro casos en los que un paciente con una placa pseudotumoral de EM precisase una craneotomía/craniectomía urgente.

Palabras clave:
Esclerosis múltiple
Esclerosis múltiple tumefacta
Esclerosis múltiple pseudotumoral

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