Title : Epidemiology, Imageology and treatment strategies in tumefactive mulitple sclerosis: A systematic review of literature
Abstract:
Objective: Tumefactive Multiple Sclerosis (TMS) is a rare subtype of multiple sclerosis (MS) with an incidence of 1-3 per 1000 MS patients. Here, we aimed to review the literature to present data on the epidemiology of TMS.
Background: The annual incidence of tumefactive MS is 0.3/100,000. The overall prevalence of TMS is 1.4 to 8.2% of MS patients. This could be an underestimate due to the unavailability of a global TMS registry and the underreporting of this condition.
Design/Methods: We searched PubMed and Google Scholar for articles published on Tumefactive MS using the search term “Tumefactive MS” and identified 137 case reports and 16 case series. Data from a total of 221 patients reported from various countries were analyzed.
Results: Out of the 221 cases studied, 152 (68.7%) were females and 69 (31.2%) were males. The mean age was 33.17 ± 14.47 years. In 146 cases (66.1%), TMS was the first presentation of MS, while 75 (33.9%) occurred in known cases of MS. The most common clinical presentation of TMS was motor symptoms (65.6%); other common features included sensory disturbances (32.13%), visual defects (24.4%), cerebellar symptoms (23.07%), speech disorders (21.71%), cognitive impairment (8.14%), headache (14.9%), and seizures (8.59%). MRI findings indicated that the frontal lobe (36.2%) was the most affected, followed by the parietal lobe (31.9%), temporal lobe (16.4%), occipital lobe (11.3%), pons (8%), cerebellum (7.5%), spinal cord (4.7%), and thalamus (4.2). Almost all patients were treated with steroids (87.6%), which mostly led to regression of symptoms. Plasma exchange therapy was also effectively used along with steroids in many cases (27.3%). Fingolimod (15.8%) was the most used disease-modifying therapy in TMS. Other disease-modifying drugs included interferon beta (11.5%), cyclophosphamide (7.2%), rituximab (5.3%), IVIG (3.8%), natalizumab (3.3%), and glatiramer acetate (3.3%). The study also revealed notable data on drug-related tumefactive lesions, reporting 29 cases (13.1%) associated with fingolimod, 2 cases with alemtuzumab, 1 case with ocrelizumab, 1 case with natalizumab, and 1 case with rituximab.
Conclusion: Our study revealed data on the demographics, symptomatology, and anatomical distribution of TMS. It also sheds light on the treatment-related TMS phenomenon, particularly with fingolimod. Further trials should be conducted on the use of these medications in multiple sclerosis.
Audience Take Away Notes:
- This review on Tumefactive Multiple Sclerosis (TMS) provides healthcare professionals with valuable insights into clinical practice. The given data sheds light on demographic profiles, common clinical presentations, and lesion distributions in the brain.
- Additionally, this review of treatment options, including steroids, plasma exchange, and disease-modifying therapies, shows comprehensive data on the therapeutic decisions and treatment outcomes. This data may be referred to in developing newer treatment plans.
- Beyond clinical application, the review serves as a valuable educational resource for neurology faculty and researchers of the treatment. It supports further exploration into emerging therapies and the underlying mechanisms of TMS, advancing knowledge and stimulating future research in the field.
