Clinical and neuroimaging features of patients with claustrum sign.
Frontiers in neurology January 1, 2025 Chunyan Zhao, Meijiao Zhang, Qingping Zhang et al. 2 citations
Among 20 patients with the claustrum sign on brain MRI, febrile infection-related epilepsy syndrome (FIRES) was the most common diagnosis (60%), followed by antibody-negative autoimmune encephalitis (20%), myelin oligodendrocyte glycoprotein antibody-associated disease (15%), and Wilson's disease (5%). Seizures occurred in 85% and impaired consciousness in 70%. The claustrum sign appeared a median of 11.5 days after symptom onset, was transient in most cases, and resolved by a median of 53 days. Patients with FIRES had the worst outcomes—all developed chronic epilepsy, 75% had poor memory and calculation—while those with autoimmune encephalitis or MOGAD had favorable outcomes. The claustrum sign likely represents a transient neuroinflammatory lesion and may be an imaging marker of neuroinflammation, with lesions potentially contributing to seizures and impaired consciousness through disrupted connectivity.