Schilder’s disease

Magriço, Marta; Lorga, Tiago; Serrazina, Filipa; Salavisa, Manuel

doi:10.1136/pn-2023-003736

Cited by 3 publications

(1 citation statement)

References 4 publications

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“…TDL were originally associated with presentations in patients with multiple sclerosis 3 . However, various etiologies underlying TDL formation have been identified, encompassing conditions such as acute disseminated encephalomyelitis 4 , 5 , neuromyelitis optica spectrum disorder (NMOSD) 6 , 7 , myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) 8 , MS variants (namely Baló concentric sclerosis 9 , 10 , myelinoclastic diffuse sclerosis or Schilder’s disease 11 , and Marburg’s acute MS 12 ), and autoimmune neurological disorders such as Behçet disease 13 and neurosarcoidosis 14 . Moreover, TDL may result from infectious processes 15 , 16 , certain medications 17 , 18 , or occur in isolation, devoid of concomitant demyelinating pathologies.…”

Section: Introductionmentioning

confidence: 99%

Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Ongphichetmetha,

Aungsumart,

Siritho

et al. 2024

Sci Rep

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Tumefactive demyelinating lesions (TDL), characterized by large (≥ 2 cm) demyelinating lesions mimicking tumors, are a rare manifestation of the central nervous system inflammatory demyelinating diseases (CNS-IDD). Distinguishing TDL from other brain lesions can be challenging, often necessitating biopsy or advanced diagnostics. The natural history of TDL varies among races. This study aimed to assess demographics, clinical and radiological features, laboratory findings, management, and outcomes of Thai patients with TDL. We retrospectively reviewed records of twenty-six patients with TDL from the Multiple Sclerosis and Related Disorders registry from two tertiary medical centers. Among 1102 CNS-IDD patients, 26 (2.4%) had TDL. The median age at TDLs onset was 34.5 years (range 17–75); 69.2% were female. Over 70% manifested TDL as their initial CNS-IDD presentation. Common presenting symptoms included motor deficits, sensory disturbances, and cognitive problems. About two-fifths exhibited multiple lesions, most frequently in the frontoparietal region (46.2%). Half of the patients showed an incomplete ring on post-contrast T1-weighted imaging, with peripheral diffusion-weighted imaging restriction in twenty-one patients. T2-hypointense rims were present in thirteen (56.5%) patients. Brain biopsy was performed in 12 cases (46.1%). Serum aquaporin-4 immunoglobulin was positive in 16.7% of tested (4/24) cases. Serum myelin oligodendrocyte glycoprotein immunoglobulin was negative in all thirteen patients tested. Twenty patients (76.9%) received intravenous corticosteroids for TDL attacks. After the median follow-up period of 48 months (range 6–300), 23.1% experienced CNS-IDD relapses. Median Expanded Disability Status Scale at TDL diagnosis was 4.3 (range 0.0–9.5), and improved to 3.0 (range 0.0–10.0) at the last follow-up. This study suggested that TDL were rare among Thai CNS-IDD patients, frequently presenting as a monophasic condition with a favorable outcome.

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