The claustrum sign refers to the bilateral claustral involvement seen in febrile infection-related epilepsy syndrome (FIRES).
This sign is described on T2 and FLAIR sequences, seen as hyperintensity of both claustra during the acute phase and posterior hypointensity during the chronic phase if a complete recovery is not achieved and atrophy has been established.
The combination of status epilepticus and pleocytosis in the CSF are highly specific for febrile infection-related epilepsy syndrome (FIRES) 1,2.
- 1. Makar AB, McMartin KE, Palese M, Tephly TR. Formate assay in body fluids: application in methanol poisoning. (1975) Biochemical medicine. 13 (2): 117-26. doi:10.1016/0006-2944(75)90147-7 - Pubmed
- 2. Stefano Meletti, Jana Slonkova, Iva Mareckova, Giulia Monti, Nicola Specchio, Petr Hon, Giada Giovannini, Vaclav Marcian, Annalisa Chiari, Petr Krupa, Nicola Pietrafusa, Dagmar Berankova, Michal Bar. Claustrum damage and refractory status epilepticus following febrile illness. (2015) Neurology. 85 (14): 1224. doi:10.1212/WNL.0000000000001996 - Pubmed