Tumefactive demyelinating lesions spectrum disorders and the potential role of contemporary disease modifying treatments: a case report

Maia, Carolina; Novo, Ana; Sousa, Mário; Bras, P Garcia; Brito, Olga Martins de; Rebelo, Olinda; Batista, Sónia; Sousa, Lívia

doi:10.1016/j.msard.2020.102669

Cited by 3 publications

(1 citation statement)

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“…Adverse prognostic factors include lesion size >5 cm, infiltrating lesions and older age (10,11,51). Patients with established MS who develop TDLs, or who subsequently meet criteria should receive conventional recommended MS treatment but with caution when switching from natalizumab or fingolimod and where the pathogenesis is uncertain, B-lymphocyte suppression may have broader efficacy (35). Those with TDLs who do not meet criteria for MS should be treated according to the severity of the disease and treatment response.…”

Section: Discussionmentioning

confidence: 99%

Phenotyping variants of tumefactive demyelinating lesions according to clinical and radiological features—A case series

Boyle

Fernando²,

Drummond³

et al. 2023

Front. Neurol.

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BackgroundTumefactive demyelinating lesions (TDLs) are defined as lesions >2 cm on MRI of the brain. They are identified in a range of demyelinating diseases including massive demyelination due to Marburg's acute MS, Schilder's Disease, Balo's concentric sclerosis, and Tumefactive MS. Apart from the rare demyelinating variants which are often diagnosed histologically, there are no detailed data to phenotype TDLs.MethodsWe describe the clinical and radiological features of four similar patients with very large TDLs (>4 cm), that are not consistent with the rare demyelinating variants and may represent a distinct phenotype.ResultsAll patients presented with hemiplegia and apraxia. The mean age at onset was 37 years with an equal sex distribution. All patients were diagnosed with Tumefactive demyelination based on MRI and CSF analysis, precluding the need for brain biopsy. All responded to potent immunotherapy (including high dose corticosteroids, plasma exchange, rituximab, and/or cyclophosphamide). The mean lag from diagnosis to treatment was 1 day. The median EDSS at presentation was six and recovery to a median EDSS of two occurred over 6 months.ConclusionWe propose that Tumefactive lesions larger than 4 cm are termed “Giant demyelinating lesions” (GDLs) not only on the basis of size, but a rapid and fulminant demyelinating presentation leading to acute, severe neurological disability that is, nonetheless, responsive to immunotherapy. Further clinical studies are required to ratify this proposed phenotype, establish the immunological profile and best treatment for such patients.

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Section: Discussionmentioning

confidence: 99%