Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2015; 3(6): 525-532
Published online Jun 16, 2015. doi: 10.12998/wjcc.v3.i6.525
Variant of multiple sclerosis with dementia and tumefactive demyelinating brain lesions
Sherifa A Hamed
Sherifa A Hamed, Department of Neurology and Psychiatry, Hospital of Neurology and Psychiatry, Assiut University Hospital, Assiut 71516, Egypt
Author contributions: Hamed SA solely contributed to this work.
Ethics approval: The study was reviewed by and received exemption from the Assiut University Hospital Institutional Review Board.
Informed consent: The study participant provided written informed consent for inclusion in this case report.
Conflict-of-interest: The author declared no potential conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Sherifa A Hamed, MD, Consultant Neurologist, Professor, Department of Neurology and Psychiatry, Hospital of Neurology and Psychiatry, Assiut University Hospital, Assiut 71516, Egypt.
Telephone: +2-88-2333327 Fax: +2-88-2333327
Received: September 16, 2014
Peer-review started: September 16, 2014
First decision: December 17, 2014
Revised: December 26, 2014
Accepted: March 16, 2015
Article in press: March 18, 2015
Published online: June 16, 2015
Processing time: 276 Days and 11.9 Hours

We describe an unusual clinical and diagnostic feature of a patient with multiple sclerosis (MS). A 25-year-old woman was admitted to the Neurology department (December 2009) with one month history of rapid cognitive deterioration. She had poor cognition, dysphasia, reduction in visual acuity and temporal pallor of the optic discs. She had prolonged latencies of P100 component of visual evoked potentials (VEPs). Magnetic resonance imaging (MRI)-brain showed multifocal large (≥ 3 cm) white-matter hypointense lesions in T1W and hyperintense in T2W and fluid-attenuated inversion recovery images and patchy enhancement. A diagnosis of tumefactive MS was given. She received two consecutive 5-d courses of 1 g daily intravenous methylprednisolone for 2 mo and oral prednisolone in dose of 80 mg twice/daily in between. At the 3rd month, Mini Mental State Examination and VEPs returned to normal but not the MRI. Patient continued oral steroids after hospital discharge (March 2010) for 9 mo with significant MRI improvement after which tapering of steroids started for a year. The patient refused immunomodulation therapy due to her low socioeconomic status. Neither clinical relapse nor new MRI lesions were observed throughout the next 4 years. In spite of the aggressive course of tumefactive MS variant, good prognosis may be seen in some patients.

Keywords: Tumefactive multiple sclerosis, Acute dementia

Core tip: Multiple sclerosis (MS) is the most common cause of progressive neurologic handicap in young adults. MS is typically presented by sensory, motor and visual dysfunctions, abnormal visual, auditory brainstem, somatosensory and motor evoked potentials, elevated cerebrospinal fluid proteins and oligoclonal bands, and abnormal neuroimaging of the brain and spinal cord. In the literature, atypical clinical and radiological presentations or variants have been described in adults with MS which may pose diagnostic difficulties. However and in spite of the aggressive course of its tumefactive variant, good prognosis may be seen in some patients on corticosteroids.