The claustrum sign is a distinctive neuroimaging finding characterized by bilateral T2/FLAIR hyperintensity of the claustrum, one of the most interconnected regions of the human brain. It was first described in new‐onset refractory status epilepticus (NORSE) and febrile infection–related epilepsy syndrome (FIRES). A systematic search was conducted according to PRISMA guidelines from 2018 (formal definition of NORSE) to February 2026 (PROSPERO 2026 CRD420261307306). We included peer‐reviewed original studies reporting MRI findings and patient‐level data, and compared clinical characteristics, treatments, and outcomes of patients with claustrum alteration versus NORSE/FIRES patients without this finding. Of 785 records identified, 65 studies met inclusion criteria; 41 provided individual patient data, yielding 206 NORSE cases (72 with the claustrum sign, 134 without). All but one patient with the claustrum sign met criteria for cryptogenic NORSE. Patients were predominantly young (mean age 24.9 years) and had a febrile prodrome in 93% of cases. Convulsive status epilepticus (SE) occurred in 98%, progressing to super‐refractory SE (SRSE) in more than half reported cases. Bilateral claustrum hyperintensity was detected in 68/71 patients during the acute phase, typically emerging 7 days after SE onset. Immunotherapy was administered in 92% of patients. In‐hospital mortality was 9%, while 74% of patients developed chronic epilepsy. Compared with patients without claustrum involvement, those with the claustrum sign more often had febrile onset (93% vs. 51%, p < 0.001) and convulsive SE (98% vs. 81.5%, p = 0.008), lower rates of SRSE (51% vs. 96.5%, p < 0.001), and ICU admission (71% vs. 99%, p < 0.001), and a trend toward lower mortality (9% vs. 16%, p = 0.240). Recognition of the claustrum sign may support early diagnosis and targeted treatment.
Margherita Burani, L. Muccioli, G. Giovannini et al.· Annals of Clinical and Trans...· 0 citations
This study explored the association between etiology of status epilepticus (SE) and treatment responsiveness. Consecutive episodes of nonhypoxic SE in patients ≥14 years old were included. Etiology was classified into acute, remote, progressive, defined electroclinical syndromes, and unknown. Four subcategories of acute etiologies were then considered based on the recent proposal: (1) triggering factors in patients with pre-existing epilepsy; (2) acute insults to central nervous system (CNS; "acute-primary CNS"); (3) CNS pathology secondary to metabolic disturbances, systemic infection, or fever ("acute-secondary CNS"); and (4) drug/alcohol intoxication or withdrawal ("acute-toxic"). Multinomial logistic regression models were fitted using a backward stepwise selection. Age, sex, consciousness before treatment, SE semiology, SE etiology, SE recurrence, level of disability, and year of presentation were independent variables. The etiological categories of acute-primary CNS (p < .001), acute-secondary CNS (p = .002), progressive (p = .010), and unknown (p = .018) SE were associated with increased odds of refractory SE. Acute-primary CNS (p < .001), progressive (p = .009), and unknown (p = .048) SE etiologies were associated with increased odds of superrefractory SE, whereas no association was found with acute-secondary CNS etiology (p = .948). Patients with SE showed different responsiveness to medications according to the underlying cause. Heterogeneity exists within the spectrum of acute symptomatic causes; distinct etiological subcategories may carry different risks of refractoriness and superrefractoriness.
Simona Lattanzi, G. Giovannini, Niccolò Orlandi et al.· Epilepsia· 0 citations
Background and Objectives Late-onset unexplained epilepsy (LOUE) represents a substantial proportion of epilepsies with onset after 50 years and often manifests as temporal lobe epilepsy (LO-TLE). Although a link with Alzheimer disease (AD) has been suggested, only a subset of LO-TLE shows AD-related biomarkers, indicating biological heterogeneity. This study aims to characterize the cognitive and CSF phenotype of LO-TLE and compare it with healthy controls (HCs) and patients with mild cognitive impairment due to AD (MCI-AD). Methods This Italian cross-sectional cohort study included LO-TLE patients with normal CSF β-amyloid (Aβ) biomarkers, MCI-AD, and age-matched and sex-matched HC. Participants underwent structural MRI, neuropsychological assessment, and CSF biomarkers assay, including neurofilament light chain (NfL) and the phosphorylated-to-total tau ratio (p/t-tau). Cortical thickness and subcortical volumes were quantified from structural MRI. Cognitive performance was summarized using principal component analyses. Group differences in imaging, cognition, and CSF biomarkers were assessed, and associations between CSF markers and cognition were examined within groups. Results The study included 18 LO-TLE, 24 MCI-AD, and 17 HC. LO-TLE showed preserved cortical thickness and subcortical volumes comparable with HC, whereas MCI-AD exhibited widespread cortical thinning and medial temporal atrophy. Despite normal imaging, LO-TLE showed lower performance compared with HC in episodic memory (t(53) = −7.79, pFDR < 0.001), short-term memory (t(53) = −2.94, pFDR = 0.007), language (t(53) = 4.12, pFDR < 0.001), and executive functions (t(53) = −3.76, pFDR < 0.001), while attention was preserved. Global cognitive performance further distinguished LO-TLE from MCI-AD, with the former group performing better (t(53) = 4.21, pFDR < 0.001). LO-TLE CSF profiles were characterized by low NfL levels and a p/t-tau ratio below the proposed cutoff of 0.17, whereas MCI-AD showed pathologic Aβ and tau alterations, elevated NfL, and p/t-tau ratio above 0.17. In LO-TLE, a higher p/t-tau ratio was associated with better performance on global cognition (rs = 0.585, pFDR = 0.032) and short-term memory (rs = 0.588, pFDR = 0.032), whereas no associations emerged in MCI-AD. Discussion LO-TLE with normal CSF AD biomarkers is characterized by distinct cognitive and biological features compared with MCI-AD, suggesting a disease process independent of AD. The low p/t-tau ratio may reflect alternative pathophysiologic mechanisms and warrants further investigation in larger longitudinal studies to clarify the underlying pathology and clinical trajectories.
Alessia Casarini, Alice Ballerini, Riccardo Maramotti et al.· Neurology· 0 citations
The ACARD score is a user-friendly tool developed to predict 30-day mortality after nonhypoxic SE and has the potential to identify participants at high risk of short-term mortality and outperform the performance of other available scoring systems.
Simona Lattanzi, E. Trinka, P. Bosque-Varela et al.· Neurology· 0 citations
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