| Abstract [eng] |
Tumefactive multiple sclerosis is a rare variant of multiple sclerosis, whose differential diagnosis often poses a challenge to clinicians because of its unusual manifestation, resembling primary central nervous system tumors, metastases, cerebral infarction, and other demyelinating disorders. The clinical manifestation is heterogeneous and depends on the size and location of the lesions, but the most common symptoms are hemiparesis or hemiplegia and hemisensory disturbances. The best imaging modality for visualizing tumefactive demyelinating lesions is magnetic resonance imaging. The lesions are usually larger than 2 cm, enhancing with contrast, accompanied by varying degrees of perifocal edema and mass effect. One of the most characteristic features is the accumulation of contrast in an open-ring pattern. Although the diagnosis of tumefactive multiple sclerosis mainly relies on clinical manifestations and magnetic resonance imaging findings, biopsy is often required to confirm the diagnosis. Corticosteroid pulse therapy is the first-line treatment of acute symptomatic tumefactive multiple sclerosis, and if the response is inadequate, plasmapheresis is administered. Disease-modifying therapy is usually initiated upon establishing the diagnosis of multiple sclerosis based on the 2017 revised McDonald criteria. Tumefactive multiple sclerosis is typically treated with higher efficacy drugs (natalizumab, alemtuzumab, ocrelizumab) compared to typical multiple sclerosis. Most patients develop a relapsing-remitting disease course. Although it has been observed that aggressive treatment of tumefactive multiple sclerosis is associated with longer time periods between the first and second relapses, long-term prognosis does not significantly differ from typical multiple sclerosis. This paper presents clinical cases reflecting the challenging differential diagnosis of tumefactive multiple sclerosis. |
