Citation, DOI, disclosures and article data
Citation:
Lorente E, Sharma R, Haouimi A, et al. Claustrum sign. Reference article, Radiopaedia.org (Accessed on 28 Mar 2025) https://doi.org/10.53347/rID-82681
The claustrum sign refers to the bilateral claustral involvement classically seen in febrile infection-related epilepsy syndrome (FIRES).
This sign is described on T2 and FLAIR MRI sequences, 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 1-3. In the context of febrile infection-related epilepsy syndrome (FIRES), the sign is usually apparent ten days after onset of status epilepticus 2.
While a classic sign of febrile infection-related epilepsy syndrome (FIRES), the claustrum sign has also been reported to occur in other cytokine-mediated diseases of the central nervous system, including 1-4:
Additionally, the same sign is also reported in Wilson disease, albeit rarely, as the 'bright claustrum sign' 4,5.
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1. Muccioli L, Pensato U, Di Vito L, Messia M, Nicodemo M, Tinuper P. Teaching NeuroImage: Claustrum Sign in Febrile Infection–Related Epilepsy Syndrome. Neurology. 2022;98(10):e1090-1. doi:10.1212/wnl.0000000000013261 - Pubmed
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2. Guo K & Hong Z. Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome (FIRES). Neurol Sci. 2023;44(9):3357-9. doi:10.1007/s10072-023-06887-6 - Pubmed
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3. Di Dier K, Dekesel L, Dekeyzer S. The Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome (FIRES). J Belg Soc Radiol. 2023;107(1):45. doi:10.5334/jbsr.3142 - Pubmed
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4. Ma X, Fan L, Jin P, Lin K, Tong G, Wang G. Clinical and Neuroimaging Features in Neurological Wilson’s Disease with Claustrum Lesions. Sci Rep. 2024;14(1):22266. doi:10.1038/s41598-024-73475-9 - Pubmed
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5. Rędzia-Ogrodnik B, Członkowska A, Antos A et al. Pathognomonic Neuroradiological Signs in Wilson's Disease – Truth or Myth? Parkinsonism & Related Disorders. 2023;107:105247. doi:10.1016/j.parkreldis.2022.105247 - Pubmed
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