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Meletti S, Giovannini G, d'Orsi G, et al. New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features. Front Neurol. 2017;8:111doi: 10.3389/fneur.2017.00111.
Meletti, S., Giovannini, G., d'Orsi, G., Toran, L., Monti, G., Guha, R., Kiryttopoulos, A., Pascarella, M. G., Martino, T., Alexopoulos, H., Spilioti, M., & Slonkova, J. (2017). New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features. Frontiers in neurology, 8111. https://doi.org/10.3389/fneur.2017.00111
Meletti, Stefano, et al. "New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features." Frontiers in neurology vol. 8 (2017): 111. doi: https://doi.org/10.3389/fneur.2017.00111
Meletti S, Giovannini G, d'Orsi G, Toran L, Monti G, Guha R, Kiryttopoulos A, Pascarella MG, Martino T, Alexopoulos H, Spilioti M, Slonkova J. New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features. Front Neurol. 2017 Mar 27;8:111. doi: 10.3389/fneur.2017.00111. eCollection 2017. PMID: 28396650; PMCID: PMC5366956.
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Front Neurol. 2017 Mar 27;8:111. doi: 10.3389/fneur.2017.00111. eCollection 2017.

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

Frontiers in neurology

Stefano Meletti, Giada Giovannini, Giuseppe d'Orsi, Lisa Toran, Giulia Monti, Rahul Guha, Andreas Kiryttopoulos, Maria Grazia Pascarella, Tommaso Martino, Haris Alexopoulos, Martha Spilioti, Jana Slonkova

Affiliations

  1. 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.
  2. Clinic of Nervous System Diseases, University of Foggia, Riuniti Hospital , Foggia , Italy.
  3. Department of Neurology, University of Virginia , Charlottesville, VA , USA.
  4. 1st Department of Neurology, Aristotle University of Thessaloniki, AHEPA Hospital , Thessaloniki , Greece.
  5. Department of Pathophysiology, Medical School, University of Athens, Neuroimmunology Unit , Athens , Greece.
  6. Clinic of Neurology, University Hospital Ostrava , Ostrava , Czech Republic.

PMID: 28396650 PMCID: PMC5366956 DOI: 10.3389/fneur.2017.00111

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 of

Keywords: claustrum; epilepsy; fever; new-onset refractory status epilepticus; refractory status epilepticus; status epilepticus

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