Stefano Meletti1, Jana Slonkova2, Iva Mareckova2, Giulia Monti2, Nicola Specchio2, Petr Hon2, Giada Giovannini2, Vaclav Marcian2, Annalisa Chiari2, Petr Krupa2, Nicola Pietrafusa2, Dagmar Berankova2, Michal Bar2. 1. From the Department of Biomedical, Metabolic, and Neural Science (S.M., G.M., G.G.), University of Modena and Reggio Emilia, Modena; NOCSAE Hospital (S.M., G.M., G.G., A.C.), ASL Modena, Italy; Clinic of Neurology (J.S., P.H., V.M., D.B., M.B.) and Radiodiagnostic Institute (P.K.), University Hospital Ostrava; the Neurological Department of Na Homolce Hospital (I.M.), Prague, Czech Republic; and the Division of Neurology (N.S., N.P.), Bambino Gesù Children's Hospital, IRCCS, Rome Italy. stefano.meletti@unimore.it. 2. From the Department of Biomedical, Metabolic, and Neural Science (S.M., G.M., G.G.), University of Modena and Reggio Emilia, Modena; NOCSAE Hospital (S.M., G.M., G.G., A.C.), ASL Modena, Italy; Clinic of Neurology (J.S., P.H., V.M., D.B., M.B.) and Radiodiagnostic Institute (P.K.), University Hospital Ostrava; the Neurological Department of Na Homolce Hospital (I.M.), Prague, Czech Republic; and the Division of Neurology (N.S., N.P.), Bambino Gesù Children's Hospital, IRCCS, Rome Italy.
Abstract
OBJECTIVE: To characterize the clinical, EEG, and brain imaging findings in an adult case series of patients with de novo refractory status epilepticus (SE) occurring after a febrile illness. METHODS: A retrospective study (2010-2013) was undertaken with the following inclusion criteria: (1) previously healthy adults with refractory SE; (2) seizure onset 0-21 days after a febrile illness; (3) lacking evidence of infectious agents in CSF; (4) no history of seizures (febrile or afebrile) or previous or concomitant neurologic disorder. RESULTS: Among 155 refractory SE cases observed in the study period, 6 patients (17-35 years old) fulfilled the inclusion criteria. Confusion and stupor were the most common symptoms at disease onset, followed after a few days by acute repeated seizures that were uncountable in all but one. Seizures consisted of focal motor/myoclonic phenomena with subsequent generalization. Antiepileptic drugs failed in every patient to control seizures, with all participants requiring intensive care unit admission. Barbiturate coma with burst-suppression pattern was applied in 4 out of 6 patients for 5-14 days. One participant died in the acute phase. In each patient, we observed a reversible bilateral claustrum MRI hyperintensity on T2-weighted sequences, without restricted diffusion, time-related with SE. All patients had negative multiple neural antibodies testing. Four out of 5 surviving patients developed chronic epilepsy. CONCLUSIONS: This is a hypothesis-generating study of a preliminary nature supporting the role of the claustrum in postfebrile de novo SE; future prospective studies are needed to delineate the specificity of this condition, its pathogenesis, and the etiology.
OBJECTIVE: To characterize the clinical, EEG, and brain imaging findings in an adult case series of patients with de novo refractory status epilepticus (SE) occurring after a febrile illness. METHODS: A retrospective study (2010-2013) was undertaken with the following inclusion criteria: (1) previously healthy adults with refractory SE; (2) seizure onset 0-21 days after a febrile illness; (3) lacking evidence of infectious agents in CSF; (4) no history of seizures (febrile or afebrile) or previous or concomitant neurologic disorder. RESULTS: Among 155 refractory SE cases observed in the study period, 6 patients (17-35 years old) fulfilled the inclusion criteria. Confusion and stupor were the most common symptoms at disease onset, followed after a few days by acute repeated seizures that were uncountable in all but one. Seizures consisted of focal motor/myoclonic phenomena with subsequent generalization. Antiepileptic drugs failed in every patient to control seizures, with all participants requiring intensive care unit admission. Barbituratecoma with burst-suppression pattern was applied in 4 out of 6 patients for 5-14 days. One participant died in the acute phase. In each patient, we observed a reversible bilateral claustrum MRI hyperintensity on T2-weighted sequences, without restricted diffusion, time-related with SE. All patients had negative multiple neural antibodies testing. Four out of 5 surviving patients developed chronic epilepsy. CONCLUSIONS: This is a hypothesis-generating study of a preliminary nature supporting the role of the claustrum in postfebrile de novo SE; future prospective studies are needed to delineate the specificity of this condition, its pathogenesis, and the etiology.
Authors: Peter Baxter; Antonia Clarke; Helen Cross; Brian Harding; Elaine Hicks; John Livingston; Robert Surtees Journal: Seizure Date: 2003-09 Impact factor: 3.184
Authors: 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 Journal: Front Neurol Date: 2017-03-27 Impact factor: 4.003
Authors: Benjamin P Whatley; Joel S Winston; Luke A Allen; Sjoerd B Vos; Ashwani Jha; Catherine A Scott; April-Louise Smith; Fahmida A Chowdhury; Jamshed B Bomanji; Samden D Lhatoo; Ronald M Harper; Beate Diehl Journal: Front Neurol Date: 2021-11-24 Impact factor: 4.003