Clinical and Radiologic Features, Pathology, and Treatment of Baló Concentric Sclerosis

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012230
Author(s):  
Evan A. Jolliffe ◽  
Yong Guo ◽  
Todd A Hardy ◽  
P. Pearse Morris ◽  
Eoin P. Flanagan ◽  
...  

Objective:To describe clinical, radiological and pathological features of patients with Baló’s concentric sclerosis (BCS) and assess overlap between BCS and other central nervous system inflammatory demyelinating diseases.Methods:Retrospective review of BCS cases from US and Australian tertiary care centers.Results:We identified 40 BCS cases with 38 available MRIs. Solitary MRI lesions were present in 26% (10/38). We saw >1 active concurrent BCS lesion in 45% (17/38). A third (13/38) had multiple sclerosis-suggestive lesions on the index MRI, of which 10 fulfilled Barkhof criteria. In patients with serial MRI performed within one month of the index MRI, lesions expanded radially with sequentially increased numbers of T2 hyperintense rings 52% (14/27). Initially non-enhancing or centrally enhancing lesions subsequently developed single or multiple enhancing rings (41%; 9/22) and incomplete enhancing rings (14%; 3/22). Discordance between rings as they appear on ADC, DWI, and gadolinium-enhanced imaging was observed in 67% (22/33). AQP4-IgG (n=26) and MOG-IgG (n=21) were negative in all patients with serum available. Clinical response to steroid treatment was seen in 46% (13/28). A monophasic clinical course was present in 56% (18/32) at last follow-up (median 27.5 months; range 3-100 months). The initial attack was fatal in 10% (4/40). Median time from symptom onset to death was 23 days (range 19-49 days). All 17 patients with pathology available demonstrated typical findings of multiple sclerosis. Patients with active demyelinating lesions all demonstrated oligodendrocytopathy (pattern III).Conclusions:BCS may be a distinct subtype of multiple sclerosis characterized by pattern III immunopathology.

2009 ◽  
Vol 11 (3) ◽  
pp. 122-126 ◽  
Author(s):  
Sarah A. Morrow ◽  
Marcelo Kremenchutzky

Multiple sclerosis (MS) is a common disabling neurologic disease with an overall prevalence in Canada of 240 in 100,000. Multiple sclerosis clinics are located at tertiary-care centers that may be difficult for a patient to access during an acute relapse. Many relapses are evaluated by primary-care physicians in private clinics or emergency departments, but these physicians' familiarity with MS is not known. Therefore, a survey was undertaken to determine the knowledge and experience of primary-care physicians regarding the diagnosis and treatment of MS relapses. A total of 1282 licensed primary-care physicians in the catchment area of the London (Ontario, Canada) Multiple Sclerosis Clinic were identified and mailed a two-page anonymous survey. A total of 237 (18.5%) responses were obtained, but only 216 (16.8%) of these respondents were still in active practice. Of these 216 physicians, only 9% reported having no MS patients in their practice, while 70% had one to five patients, 16.7% had six to ten, and 1.9% had more than ten (3.7% did not respond to this question). Corticosteroids were recognized as an MS treatment by 49.5% of the respondents, but only 43.1% identified them as a treatment for acute relapses. In addition, 31% did not know how to diagnose a relapse, and only 37% identified new signs or symptoms of neurologic dysfunction as indicating a potential relapse. Despite the high prevalence of MS in Canada, primary-care physicians require more education and support from specialists in MS care regarding the diagnosis and treatment of MS relapses.


Author(s):  
L.F. Kastrukoff ◽  
D.R. McLean ◽  
T.A. McPherson

SUMMARY:Multiple sclerosis patients treated with antithymocyte globulin (ATG) were re-evaluated after five years. No long term benefit was found. Notably, the group of patients with an elevated gamma globulin to total protein ration in their C.S.F. and who did particularly well after treatment with ATG also failed to show any long term benefit. Few long term detrimental effects of ATG immunosuppression were identified. The implications of the results are discussed as they relate to the use of immunosuppression in multiple sclerosis.


2016 ◽  
Vol 365 ◽  
pp. 143-146 ◽  
Author(s):  
R. Iodice ◽  
A. Carotenuto ◽  
R. Dubbioso ◽  
I. Cerillo ◽  
L. Santoro ◽  
...  

Author(s):  
Ilkka Rauma ◽  
Tiina Mustonen ◽  
Juha Matti Seppä ◽  
Maritta Ukkonen ◽  
Marianne Männikkö ◽  
...  

Abstract Background Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice. Objectives To evaluate the safety of alemtuzumab in a nationwide cohort of Finnish MS patients. Methods In this retrospective case series study, we analyzed the data of all but two MS patients who had received alemtuzumab in Finland until 2019. Data were systematically collected from patient files. Results Altogether 121 patients were identified, most of whom had received previous DMTs (82.6%). Median follow-up time after treatment initiation was 30.3 months and exceeded 24 months in 78 patients. Infusion-associated reactions (IARs) were observed in 84.3%, 57.3%, and 57.1% of patients during alemtuzumab courses 1–3, respectively. Serious adverse events (SAEs) were observed in 32.2% of patients, serious IARs in 12.4% of patients, and SAEs other than IARs in 23.1% of patients. Autoimmune adverse events were observed in 30.6% of patients. One patient died of hemophagocytic lymphohistiocytosis, and one patient died of pneumonia. A previously unreported case of thrombotic thrombocytopenic purpura was documented. Conclusions SAEs were more frequent in the present cohort than in previous studies. Even though alemtuzumab is a highly effective therapy for MS, vigorous monitoring with a long enough follow-up time is advised.


2010 ◽  
Vol 16 (4) ◽  
pp. 480-490 ◽  
Author(s):  
U. Dalgas ◽  
E. Stenager ◽  
J. Jakobsen ◽  
T. Petersen ◽  
HJ Hansen ◽  
...  

Fatigue occurs in the majority of multiple sclerosis patients and therapeutic possibilities are few. Fatigue, mood and quality of life were studied in patients with multiple sclerosis following progressive resistance training leading to improvement of muscular strength and functional capacity. Fatigue (Fatigue Severity Scale, FSS), mood (Major Depression Inventory, MDI) and quality of life (physical and mental component scores, PCS and MCS, of SF36) were scored at start, end and follow-up of a randomized controlled clinical trial of 12 weeks of progressive resistance training in moderately disabled (Expanded Disability Status Scale, EDSS: 3—5.5) multiple sclerosis patients including a Control group ( n = 15) and an Exercise group ( n = 16). Fatigue (FSS > 4) was present in all patients. Scores of FSS, MDI, PCS—SF36 and MCS—SF36 were comparable at start of study in the two groups. Fatigue improved during exercise by —0.6 (95% confidence interval (CI) —1.4 to 0.4) a.u. vs. 0.1 (95% CI —0.4 to 0.6) a.u. in controls ( p = 0.04), mood improved by —2.4 (95% CI —4.1 to 0.7) a.u. vs. 1.1 (—1.2 to 3.4) a.u. in controls ( p = 0.01) and quality of life (PCS—SF36) improved by 3.5 (95% CI 1.4—5.7) a.u. vs. —1.0 (95% CI —3.4—1.4) a.u. in controls ( p = 0.01). The beneficial effect of progressive resistance training on all scores was maintained at follow-up after further 12 weeks. Fatigue, mood and quality of life all improved following progressive resistance training, the beneficial effect being maintained for at least 12 weeks after end of intervention.


2014 ◽  
Vol 3 (1) ◽  
pp. 67-71 ◽  
Author(s):  
K.S. Pandey ◽  
S.C. Krieger ◽  
C. Farrell ◽  
C. Hannigan ◽  
T. DeAngelis ◽  
...  

2014 ◽  
Vol 85 (10) ◽  
pp. 1109-1115 ◽  
Author(s):  
Cecilie Jacobsen ◽  
Jesper Hagemeier ◽  
Kjell-Morten Myhr ◽  
Harald Nyland ◽  
Kirsten Lode ◽  
...  

2007 ◽  
Vol 13 (8) ◽  
pp. 1011-1019 ◽  
Author(s):  
M. Younes ◽  
J. Hill ◽  
J. Quinless ◽  
M. Kilduff ◽  
B. Peng ◽  
...  

Cognitive impairment in multiple sclerosis is difficult to study because of the heterogeneity and variability of this disease. The gold standard for measurement of cognitive function in multiple sclerosis is a full battery of neurocognitive tests, which is time consuming and expensive. Some cognitive tests like the PASAT, a measure of working verbal memory and processing speed, have been proposed for screening and follow-up of cognitive function in clinical trials. We studied whether we could measure cognitive function in multiple sclerosis over the Internet. For this we used the Cognitive Stability Index (CSI)™, developed for persons with known or suspected primary central nervous system illness. The CSI was compared with formal neurocognitive testing (NPsych) and the PASAT in a cross-sectional study of 40 consecutive multiple sclerosis patients with subjective cognitive complaints. NPsych revealed that only 18 of the 40 patients (46%) were cognitively impaired. Although both the CSI and the PASAT were equalivalent in their specificity (86%), the CSI was significantly more sensitive than the PASAT (83% versus 28%). We conclude that the CSI, because of its availability over the Internet, has great potential as a tool for screening and follow up of cognitive function in multiple sclerosis. Multiple Sclerosis 2007; 13: 1011—1019. http://msj.sagepub.com


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