New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features

• Published in: Frontiers in neurology Vol. 8; p. 111
• Main Authors: Meletti, Stefano, Giovannini, Giada, d’Orsi, Giuseppe, Toran, Lisa, Monti, Giulia, Guha, Rahul, Kiryttopoulos, Andreas, Pascarella, Maria Grazia, Martino, Tommaso, Alexopoulos, Haris, Spilioti, Martha, Slonkova, Jana
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Media S.A 27.03.2017
• Subjects: Neuroscience; epilepsy; claustrum; status epilepticus; fever; refractory status epilepticus; new-onset refractory status epilepticus
• ISSN: 1664-2295; 1664-2295
• DOI: 10.3389/fneur.2017.00111

New-onset refractory status epilepticus (NORSE) is a rare but challenging condition occurring in a previously healthy patient, often with no identifiable cause. We describe the electro-clinical features and outcomes in a group of patients with NORSE who all demonstrated a typical magnetic resonance imaging (MRI) sign characterized by bilateral lesions of the claustrum. The group includes 31 patients (12 personal and 19 previously published cases; 17 females; mean age of 25 years). Fever preceded status epilepticus (SE) in 28 patients, by a mean of 6 days. SE was refractory/super-refractory in 74% of the patients, requiring third-line agents and a median of 15 days staying in an intensive care unit. Focal motor and tonic-clonic seizures were observed in 90%, complex partial seizures in 14%, and myoclonic seizures in 14% of the cases. All patients showed T2/FLAIR hyperintense foci in bilateral claustrum, appearing on average 10 days after SE onset. Other limbic (hippocampus, insular) alterations were present in 53% of patients. Within the personal cases, extensive search for known autoantibodies was inconclusive, though 7 of 11 patients had cerebrospinal fluid lymphocytic pleocytosis and 3 cases had oligoclonal bands. Two subjects died during the acute phase, one in the chronic phase (probable sudden unexplained death in epilepsy), and one developed a persistent vegetative state. Among survivors, 80% developed drug-resistant epilepsy. Febrile illness-related SE associated with bilateral claustrum hyperintensity on MRI represents a condition with defined clinical features and a presumed but unidentified autoimmune etiology. A better characterization of SE is mandatory for the search of specific etiologies.