title: New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features.
abstract: 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 ofde novoSE is mandatory for the search of specific etiologies.
authors.affiliation: Department of Biomedical, Metabolic, and Neural Sciences, Center for Neurosciences and Neurotechnology, University of Modena and Reggio Emilia, Modena, Italy; Neurology Unit, NOCSAE Hospital, AOU Modena, Modena, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Meletti
authors.affiliation: Department of Biomedical, Metabolic, and Neural Sciences, Center for Neurosciences and Neurotechnology, University of Modena and Reggio Emilia, Modena, Italy; Neurology Unit, NOCSAE Hospital, AOU Modena, Modena, Italy.
authors.forename: Giada
authors.initials: G
authors.lastname: Giovannini
authors.affiliation: Clinic of Nervous System Diseases, University of Foggia, Riuniti Hospital , Foggia , Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: d'Orsi
authors.affiliation: Department of Neurology, University of Virginia , Charlottesville, VA , USA.
authors.forename: Lisa
authors.initials: L
authors.lastname: Toran
authors.affiliation: Department of Biomedical, Metabolic, and Neural Sciences, Center for Neurosciences and Neurotechnology, University of Modena and Reggio Emilia, Modena, Italy; Neurology Unit, NOCSAE Hospital, AOU Modena, Modena, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Monti
authors.affiliation: Department of Neurology, University of Virginia , Charlottesville, VA , USA.
authors.forename: Rahul
authors.initials: R
authors.lastname: Guha
authors.affiliation: 1st Department of Neurology, Aristotle University of Thessaloniki, AHEPA Hospital , Thessaloniki , Greece.
authors.forename: Andreas
authors.initials: A
authors.lastname: Kiryttopoulos
authors.affiliation: Clinic of Nervous System Diseases, University of Foggia, Riuniti Hospital , Foggia , Italy.
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Pascarella
authors.affiliation: Clinic of Nervous System Diseases, University of Foggia, Riuniti Hospital , Foggia , Italy.
authors.forename: Tommaso
authors.initials: T
authors.lastname: Martino
authors.affiliation: Department of Pathophysiology, Medical School, University of Athens, Neuroimmunology Unit , Athens , Greece.
authors.forename: Haris
authors.initials: H
authors.lastname: Alexopoulos
authors.affiliation: 1st Department of Neurology, Aristotle University of Thessaloniki, AHEPA Hospital , Thessaloniki , Greece.
authors.forename: Martha
authors.initials: M
authors.lastname: Spilioti
authors.affiliation: Clinic of Neurology, University Hospital Ostrava , Ostrava , Czech Republic.
authors.forename: Jana
authors.initials: J
authors.lastname: Slonkova
keywords: claustrum; epilepsy; fever; new-onset refractory status epilepticus; refractory status epilepticus; status epilepticus