Topic
Malignant multiple sclerosis
About: Malignant multiple sclerosis is a research topic. Over the lifetime, 12 publications have been published within this topic receiving 281 citations.
Papers
TL;DR: MS in patients who reach a high EDSS based on disease progression is more likely to remain malignant, and some demographic and clinical factors are found to predict a malignant outcome.
Abstract: Objective: Multiple sclerosis (MS) that causes patients to require assistance for ambulation (Expanded Disability Status Scale [EDSS] ≥6) within 5 years from symptom onset is generally termed malignant. Malignant status can be transient (TM) or sustained until year 5 (SM). We studied the incidence, predictors, and demographic and clinical characteristics of malignant MS. Methods: Patients with symptom onset in 2002–2005 and 5-year follow-up were selected from the Partners Multiple Sclerosis Center database. Patients with TM were further grouped into TM and SM. The mechanism of reaching EDSS 6 (relapse- vs progression-related) was determined. Results: A total of 487 patients were included (17 TM, 42 SM). The incidence proportion of ever malignant (EM = SM+TM) was estimated as 12.11% and SM as 8.62%. Patients with older age at onset, male gender, and positive smoking history were more likely to become SM. Compared to nonmalignant patients, the proportion of progressive-onset MS in the SM group was significantly higher, but not different in TM. Within relapsing-onset patients, most of TM, and a smaller proportion of the SM group had a relapse-related as opposed to progression-related mechanism. The final model predictors for EM vs nonmalignant were older age at onset, motor symptoms at onset, and progressive disease onset. Within the malignant patients, predictors of TM vs SM were younger age and brainstem symptoms at onset. Conclusions: Over 10% of patients with MS experience a malignant course as defined above. Some demographic and clinical factors are found to predict a malignant outcome. MS in patients who reach a high EDSS based on disease progression is more likely to remain malignant.
92 citations
TL;DR: It is suggested that a rapid decline in the level of maintained body weight in a patient without pituitary disease or general organic disorder, or distinct emotional disorder, may represent a clinical manifestation of tissue injury of the lateral hypothalamus.
Abstract: A 41-year-old woman had profound weight loss and cachexia as a manifestation of rapidly fatal multiple sclerosis. Demyelinating lesions were present in the lateral hypothalamus. Data from animal experiments have indicated that lateral hypothalamic lesions cause a weight loss associated with a lowering of the regulation level or “set-point” for body weight. This case suggests, therefore, that a rapid decline in the level of maintained body weight in a patient without pituitary disease or general organic disorder, or distinct emotional disorder, may represent a clinical manifestation of tissue injury of the lateral hypothalamus.
58 citations
TL;DR: It is concluded that intensive immunosuppression followed by autologous stem cell transplantation is a viable therapeutic option in patients with malignant MS unresponsive to conventional forms of treatment.
Abstract: Malignant multiple sclerosis (MS) is a rare but clinically important subtype of MS characterized by the rapid development of significant disability in the early stages of the disease process. These patients are refractory to conventional immunomodulatory agents and the mainstay of their treatment is plasmapheresis or immunosuppression with mitoxantrone, cyclophosphamide, cladribine or, lately, bone marrow transplantation. We report on the case of a 17-year old patient with malignant MS who was treated with high-dose chemotherapy plus anti-thymocyte globulin followed by autologous stem cell transplantation. This intervention resulted in an impressive and long-lasting clinical and radiological response. It is concluded that intensive immunosuppression followed by autologous stem cell transplantation is a viable therapeutic option in patients with malignant MS unresponsive to conventional forms of treatment.
51 citations
TL;DR: In this article, a review of benign and malignant multiple sclerosis, remitting and progressive forms, are studied from epidemiological and clinical points of view in the context of large populations of patients with multiple sclerosis on longterm follow up at a specialized centre, with the objective of finding, if possible, the patterns of the natural history of the disease.
Abstract: INTRODUCTION Multiple sclerosis is one of the commonest idiopathic inflammatory diseases of the central nervous system in young adults. The study of the natural history of multiple sclerosis implies in the first place, establishing precise limits for the diagnosis of the disease, which is still of unknown origin. It is also necessary to analyze the wide range of varieties, which as different categories or subgroups and with different degrees of severity, make up the clinical spectrum of this entity. DEVELOPMENT AND CONCLUSIONS Throughout this review benign and malignant multiple sclerosis, remitting and progressive forms, are studied from epidemiological and clinical points of view in the context of large populations of patients with multiple sclerosis on long-term follow up at a specialized centre, with the objective of finding, if possible, the patterns of the natural history of the disease.
30 citations
TL;DR: It is believed that IA may be useful, either as a coadyuvant or alternative treatment in severe relapses in MS patients that do not respond to high-dose intravenous corticosteroid therapy.
Abstract: Introduction The treatment of prolonged, severe relapses in multiple sclerosis (MS) patients who respond poorly to high-dose intravenous corticosteroids is not yet established. Plasma immunoadsortion (IA) removes immunoglobulins (IgG), immune complexes, and complement from plasma. It may bear some advantages compared to plasmapheresis, a nonselective procedure that requires substitution of patient plasma by colloids solutions or plasma, which may carry a potential risk for viral infections. Patients and methods Three relapsing-remitting MS patients with a malignant course received IA. All they were experiencing a prolonged relapse unresponsive to high-dose intravenous corticosteroids, causing a locked-in state in two of the patients and severe pseudobulbar impairment in the third one. Five to six IA consecutive sessions were administered along a 7-10 days course. Results IA was followed by a prompt and unequivocal clinical response in all three patients, which paralleled a decrease in IgG, fibrinogen, and C3 complement serum levels. IA administration was followed by immunosuppressor therapy, either with cyclophosphamide and intravenous ACTH (2 cases) or mitoxantrone (1 case). Improvement has been sustained along a mean follow-up of 7.6 years (range: 7-8.5 years), only one of the patients suffering two mild clinical relapses. Conclusion We believe that IA may be useful, either as a coadyuvant or alternative treatment in severe relapses in MS patients that do not respond to high-dose intravenous corticosteroid therapy.
23 citations
Related Topics (5)
Performance Metrics
| Year | Papers |
|---|---|
| 2019 | 1 |
| 2017 | 1 |
| 2011 | 1 |
| 2008 | 1 |
| 2005 | 1 |
| 2000 | 2 |