Tumefactive demyelinating lesions as a first clinical event: Clinical, imaging, and follow-up observations

Natalizumab is Effective for the Treatment of Relapsing-remitting Tumefactive Multiple Sclerosis

“…Altintas et al stated that only 16.7% of patients developed new lesions exceeding 2 cm in diameter, with a median follow-up of 38 months after the first attack with TDL (3). In addition, Jeong et al reported that only 16.1% of 31 patients with initially diagnosed TDL developed new lesions exceeding 2 cm in diameter over a median follow-up period of about 38 months (7). Our patient had a third large demyelinating attack during the first year of follow-up.…”

Commentary: Tumefactive Demyelinating Lesions as a First Clinical Event: Clinical, Imaging, and Follow-up Observations

“…They found that most of TDLs evolve into multiple sclerosis (MS) or neuromyelitis optica spectrum disorders (NMOSD), albeit a minority of patients remains without a definite diagnosis, even after a careful and extensive diagnostic workup (1). The authors underlined that the current understanding of the etiology of TDLs should be revaluated upon appraisal of NMOSD because extensive brain lesions have been frequently reported as a first manifestation in NMOSD, especially in Asian populations (1). …”

“…Although some radiological characteristics on routine magnetic resonance imaging (MRI) such as an open-ring enhancement, T2-hypointense rim, peripheral restriction on diffusion-weighted imaging, and venular enhancement are considered typical for TDLs, they were not found in all cases (1–5). Even the advanced MRI techniques, such as MR spectroscopy, led to equivocal results because both normalized choline increase and N -acetyl-aspartate decrease were found in variable proportion in both TDLs (3, 4) and gliomas (3) with a broad overlap between these diseases.…”

“…Enlarged blood vessels with surrounding edema and relative axonal preservation were observed also at histological examination (3, 5). Moreover, some clinical symptoms, more frequent in TDLs than in MS, such as encephalopathy, confusion, rapid memory dysfunction, seizures, stupor/coma (4, 5), and even increased intracranial pressure symptoms (1), are more compatible with venous stasis than with localized and well-defined demyelinating lesions.…”

“…Indeed, fibrin deposition precedes and regulates the inflammatory demyelination in both experimental allergic encephalomyelitis and MS (9). Antiphospholipid positivity is even greater in NMOSD than in MS (11), and it should be noted that NMOSD usually represent a more severe disease as compared to MS and at the same time frequently associate with extensive brain lesions similar to TDLs (1). The increased antiphospholipid positivity in NMOSD indicates a raised likelihood of thrombotic phenomena in this disease, and thus, presumably in TDLs.…”