Clinical and imaging correlation in patients with pathologically confirmed tumefactive demyelinating lesions

摘要

Objectives: To characterize clinical and imaging features in patients with pathologically confirmed demyelinating lesions. Methods: In this retrospective chart review, we analyzed clinical-radiological-pathological correlations in patients >15 years old who underwent brain biopsy at our institution between 2000 and 2015 and had inflammatory demyelination on neuropathology. Results: Of 31 patients, the mean age was 42 years (range 16 to 69 years) and 55% were female. All but one of the biopsied lesions were considered tumefactive demyelinating lesions (TDLs) by imaging criteria, measuring > 2 cm on contrast-enhanced brain MRI. On clinical follow-up, the final diagnosis was a CNS malignancy in 2 patients (6.5%). In patients without malignant tumor, the TDL was solitary in 12 (41%) and multifocal in 17 (59%), with contrast enhancement in all but one case, primarily in an incomplete rim enhancement pattern (75.9%). Of 16 patients with at least 12 months of clinical follow-up, 7 (43.8%) had a clinical relapse. Of patients without a prior neurologic history, relapse occurred in 2/7 (29%) in solitary TDL and 2/6 (33%) in multifocal lesions at initial presentation. Recurrent TDLs occurred in 3 patients, all with initially solitary TDLs. Stratifying by CSF analysis, 4 of 6 patients (67%) with either an elevated IgG Index or >2 oligoclonal bands suffered a clinical relapse compared to 2/8 (25%) with non-inflammatory CSF. Conclusions: Pathologically confirmed TDLs call for careful clinical correlation, clinical follow-up and imaging surveillance. Although sometimes clinically monophasic, tumefactive demyelinating lesions carried nearly a 45% risk of near-term clinical relapse in our study, even when presenting initially as a solitary mass lesion.