Features of tumefactive demyelinating lesion (TDL) on magnetic resonance imaging (MRI)can facilitate the differential diagnosis of TDL and neoplastic lesions, but vary considerably among patients. The larger TDL grows, the more difficult it becomes to differentiate TDL from neoplastic lesions. The purpose of this study was to elucidate typical MRI features in 12 patients with large TDL (>30 mm in diameter).
METHODSWe identified 12 patients with large TDL (six men, six women; age range 17-64 years, median age 27 years) and studied the clinical histories and the results of laboratory and various radiological studies in these patients. All cases of clinically definite multiple sclerosis were diagnosed in accordance with McDonald's revised criteria.
RESULTS
Common MRI features of large TDLs included variable degrees of mass effect (71%) and edema (100%), a T2 hypointense rim (79%), venular enhancement (57%), and peripheral restriction on diffusion-weighted images (50%). Ring enhancement (38%), open-ring enhancement (31%), or decreased N-acetylaspartate ratios on magnetic resonance spectroscopy (22%) were less frequently observed. Brain angiography demonstrated venous dilatations on and around the TDL.
CONCLUSIONSThe diagnosis of large TDL is challenging. Our findings suggest that multiple venous dilatations on and around TDLs on angiography can facilitate diagnosis.
We describe a patient with dermatomyositis who presented with rapidly developing severe muscle weakness complicated by massive pleural effusion with interstitial lung disease. Myopathological analysis was suggestive of dermatomyositis. This patient showed both anti-Jo-1 and anti-SRP antibodies in serum. To our knowledge, the coexistence of these two myositis-specific autoantibodies (MSA) is considered extremely rare and is clearly an exception to the rule of having only one MSA. Our findings provide compelling evidence that the coexistence of these two MSAs may lead to more severe clinical symptoms, interacting in a complex fashion, thus expanding the clinical spectrum of idiopathic inflammatory myopathy.
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