Claustrum damage and refractory status epilepticus following febrile illness.
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.
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.
Stefano
S
Meletti
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.
Jana
J
Slonkova
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.
Iva
I
Mareckova
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.
Giulia
G
Monti
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.
Nicola
N
Specchio
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.
Petr
P
Hon
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.
Giada
G
Giovannini
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.
Vaclav
V
Marcian
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.
Annalisa
A
Chiari
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.
Petr
P
Krupa
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.
Nicola
N
Pietrafusa
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.
Dagmar
D
Berankova
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.
Michal
M
Bar
D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D001479:Basal Ganglia / Q000473:pathology*; D001930:Brain Injuries; D004569:Electroencephalography / Q000379:methods; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D012189:Retrospective Studies; D012640:Seizures / Q000188:drug therapy / Q000473:pathology*; D013226:Status Epilepticus / Q000188:drug therapy / Q000473:pathology*; D055815:Young Adult
D000927:Anticonvulsants