Late-onset tumefactive multiple sclerosis

Department of Radiology, Wakayama Medical University, 811-1 Kimiidera, Wakayama, 641-8510, Japan.
Radiation Medicine 12/2008; 26(9):549-52. DOI: 10.1007/s11604-008-0273-4
Source: PubMed


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.

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    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.
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