Late-onset tumefactive multiple sclerosis
A patient with tumefactive multiple sclerosis (MS) initially presented at the age of 87 years; the revised diagnostic criteria from the International Panel on MS (2001) were fulfilled. Late-onset MS and tumefactive demyelinating lesions are discussed. This case suggests that MS can occur at any age. MS should be borne in mind for differential diagnosis if a brain tumor-like lesion with little mass effect and edema is found in an elderly patient.
- [Show abstract] [Hide abstract] ABSTRACT: Tumefactive demyelinating disease (TDD) is a rare demyelinating disease—probably a variant of multiple sclerosis (MS)—presenting as a focal cerebral mass. Clinically and radiographically, TDD is usually difficult to differentiate from tumor and abscess. Magnetic resonance imaging (MRI) characteristically shows a large lesion—contrast enhancement often like an incomplete ring—with relatively limited mass effect and surrounding edema. Findings on “non-conventional” MRI, multifocality on MRI of the whole central nervous system (CNS), monitoring of visual evoked potentials, and cerebrospinal fluid analysis may all contribute to the differential diagnosis. In analogy with the recommendations for acute MS exacerbations, acute disseminated encephalomyelitis and other forms of acute conditions with severe inflammatory CNS demyelination, high-dose corticosteroid treatment is regarded as first-line therapy. A rapid and pronounced clinical and radiological response to the administration of high-dose corticosteroids is often observed and may alleviate the need for cerebral biopsy. In agreement with the recommendations for other conditions characterized by acute CNS demyelination, plasma exchange is considered to be second-line therapy. In patients who do not respond to non-surgical therapy and who exhibit a pronounced cerebral mass lesion and clinical signs of increased intracranial pressure (ICP), decompressive craniectomy including wide opening of the dura is recommended.0Comments 1Citation
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- [Show abstract] [Hide abstract] ABSTRACT: An 82-year old male, with no significant past medical history, presented with a subacute right foot drop in the setting of a 14-month history of generalised weakness, highly-responsive to steroids. Temporal artery and vastus lateralis biopsies were normal. Vasculitic screen and inflammatory markers were normal. Lumbar puncture revealed elevated cerebrospinal fluid (CSF) protein without oligoclonal bands. Visual evoked response (VER) was normal. Magnetic resonance imaging (MRI) of his lumbar spine showed compression of exiting L5 nerve root. He had three cerebral MRI scans spaced over the 12 month period, which showed a progressive increase of T2 and fluid attenuated inversion recovery (FLAIR) hyperintense lesions consistent with active demyelinating plaques. He was treated with intravenous methylprednisolone 1g daily for three days with a weaning regimen of oral prednisolone, resulting in a full return of power and a resolution of his right foot drop. He was diagnosed with late-onset multiple sclerosis (LOMS), and was treated with monthly natalizumab. A literature review of LOMS is discussed. © 2013 Elsevier B.V. All rights reserved.0Comments 0Citations