Matthew A Tremblay1, Javier E Villanueva-Meyer2, Soonmee Cha3, Tarik Tihan4, Jeffrey M Gelfand5. 1. MS Center, Department of Neurology, University of California, San Francisco, Box 3014, 1500 Owens St, Ste 320, San Francisco, CA 94158, United States. Electronic address: mtremblay@uchc.edu. 2. Neuroradiology Division, Department of Radiology, University of California, San Francisco, 350 Parnassus Ave, Box 0336, Ste 307H, San Francisco, CA 94143-0628, United States. Electronic address: Javier.Villanueva-Meyer@ucsf.edu. 3. Neuroradiology Division, Department of Radiology, University of California, San Francisco, 350 Parnassus Ave, Box 0336, Ste 307H, San Francisco, CA 94143-0628, United States. Electronic address: Soonmee.Cha@ucsf.edu. 4. Department of Pathology, University of California, San Francisco, 505 Parnassus Avenue, Box 0102, San Francisco, CA 94143-0102, United States. Electronic address: Tarik.Tihan@ucsf.edu. 5. MS Center, Department of Neurology, University of California, San Francisco, Box 3014, 1500 Owens St, Ste 320, San Francisco, CA 94158, United States. Electronic address: Jeffrey.Gelfand@ucsf.edu.
Abstract
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 >15years 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 42years (range 16 to 69years) and 55% were female. All but one of the biopsied lesions were considered tumefactive demyelinating lesions (TDLs) by imaging criteria, measuring >2cm 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 12months 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.
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 >15years 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 42years (range 16 to 69years) and 55% were female. All but one of the biopsied lesions were considered tumefactive demyelinating lesions (TDLs) by imaging criteria, measuring >2cm 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 12months 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.
Authors: M Nagappa; A B Taly; S Sinha; R D Bharath; A Mahadevan; P S Bindu; J S Saini; C Prasad; S K Shankar Journal: Acta Neurol Scand Date: 2012-12-31 Impact factor: 3.209
Authors: Maciej Jurynczyk; Ruth Geraldes; Fay Probert; Mark R Woodhall; Patrick Waters; George Tackley; Gabriele DeLuca; Saleel Chandratre; Maria I Leite; Angela Vincent; Jacqueline Palace Journal: Brain Date: 2017-03-01 Impact factor: 13.501
Authors: Bogdan F G Popescu; Yong Guo; Mark E Jentoft; Joseph E Parisi; Vanda A Lennon; Sean J Pittock; Brian G Weinshenker; Dean M Wingerchuk; Caterina Giannini; Imke Metz; Wolfgang Brück; Elizabeth A Shuster; Jonathan Carter; Clara D Boyd; Stacey Lynn Clardy; Bruce A Cohen; Claudia F Lucchinetti Journal: Neurology Date: 2014-12-10 Impact factor: 9.910
Authors: C F Lucchinetti; R H Gavrilova; I Metz; J E Parisi; B W Scheithauer; S Weigand; K Thomsen; J Mandrekar; A Altintas; B J Erickson; F König; C Giannini; H Lassmann; L Linbo; S J Pittock; W Brück Journal: Brain Date: 2008-06-05 Impact factor: 13.501