Introduction: Tumefactive demyelination (TD) can be a challenging scenario for clinicians due to difficulties distinguishing it from other conditions, such as neoplasm or infection; or with managing the consequences of acute lesions, and then deciding upon the most appropriate longer term treatment strategy.
Areas covered: The authors review the literature regarding TD covering its clinic-radiological features, association with multiple sclerosis (MS), and its differential diagnosis with other neuroinflammatory and non-inflammatory mimicking disorders with an emphasis on atypical forms of demyelination including acute disseminated encephalomyelitis (ADEM), MOG antibody-associated demyelination (MOGAD) and neuromyelitis spectrum disorders (NMOSD). We also review the latest in the acute and long-term treatment of TD.
Expert opinion: It is important that the underlying cause of TD be determined whenever possible to guide the management approach which differs between different demyelinating and other inflammatory conditions. Improved neuroimaging and advances in serum and CSF biomarkers should one day allow early and accurate diagnosis of TD leading to better outcomes for patients.
Keywords: ADEM; Balo; MOGAD; MRI; Marburg; Multiple sclerosis; NMOSD; Schilder; atypical; differential diagnosis; prognosis; pseudotumour; treatment.