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Benign epilepsy with centrotemporal spikes (BECTS) is associated with a low risk of sudden unexpected death in epilepsy (SUDEP), according to a study published in the June 1 JAMA Neurology. Clinicians should consider discussing this rare outcome with patients and their families and factoring this risk into treatment decisions, according to the authors.
“Our data suggest that even children with BECTS who have only focal motor or infrequent seizures are at risk for SUDEP,” said Kyra Doumlele, of New York University School of Medicine’s Department of Neurology, and colleagues.
BECTS is the most common focal epilepsy syndrome among children. This form of epilepsy typically has a good prognosis and tends to resolve before age 16. As a result, many neurologists do not prescribe antiseizure medications for patients with BECTS. However, patients with this disease have predominantly nocturnal seizures and some generalized tonic-clonic seizures, which could put them at risk for SUDEP.
SUDEP in well-characterized patients with BECTS has not been documented. Ms. Doumlele and colleagues sought to determine whether cases of BECTS were present in the North American SUDEP Registry (NASR), a clinical and biospecimen repository established to investigate the risk factors and mechanisms for SUDEP.
Researchers searched records of 189 decedents enrolled in the NASR from June 3, 2011, to June 3, 2016. Investigators identified cases based on a diagnosis of BECTS by parental report during the intake interview and confirmation from treating physicians. Diagnostic criteria for BECTS included the onset of epilepsy at age 3 to age 13, normal cognition and development before onset of seizures, no symptomatic cause of epilepsy, and EEG results reporting discharges consistent with BECTS.
Investigators identified three boys who had received a diagnosis of BECTS. The first patient and third patient met all clinical and EEG features for a diagnosis of BECTS. The second patient had limited EEG data, but had classic clinical features of BECTS.
The first patient (age 9) had seizure onset at age 5. He experienced an increase in focal motor and secondary generalized tonic-clonic seizures in the six months before his death.
The second patient (age 12) developed epilepsy at age 11. Four months prior to death, he experienced at least three nocturnal generalized tonic-clonic seizures. The autopsy report listed his cause of death as “cardiac dysrhythmia associated with lymphocytic myocarditis, left anterior descending coronary artery intramuscular tunneling, and possible hypertrophic cardiomyopathy.”
The third patient (age 13) developed epilepsy at age 3. His severe postictal state indicated that he might have had two generalized tonic-clonic seizures in the year before he died. His death was unwitnessed, and no autopsy was performed. None of these patients had received antiseizure medications or counseling with their families about the risk of SUDEP.
“These cases illustrate that SUDEP is a rare complication of BECTS, and this information should be shared with families at or soon after diagnosis,” said Ms. Doumlele and colleagues.
—Erica Tricarico
Suggested Reading
Doumlele K, Friedman D, Buchhalter J, et al. Sudden unexpected death in epilepsy among patients with benign childhood epilepsy with centrotemporal spikes. JAMA Neurol. 2017;74(6):645-649.
Benign epilepsy with centrotemporal spikes (BECTS) is associated with a low risk of sudden unexpected death in epilepsy (SUDEP), according to a study published in the June 1 JAMA Neurology. Clinicians should consider discussing this rare outcome with patients and their families and factoring this risk into treatment decisions, according to the authors.
“Our data suggest that even children with BECTS who have only focal motor or infrequent seizures are at risk for SUDEP,” said Kyra Doumlele, of New York University School of Medicine’s Department of Neurology, and colleagues.
BECTS is the most common focal epilepsy syndrome among children. This form of epilepsy typically has a good prognosis and tends to resolve before age 16. As a result, many neurologists do not prescribe antiseizure medications for patients with BECTS. However, patients with this disease have predominantly nocturnal seizures and some generalized tonic-clonic seizures, which could put them at risk for SUDEP.
SUDEP in well-characterized patients with BECTS has not been documented. Ms. Doumlele and colleagues sought to determine whether cases of BECTS were present in the North American SUDEP Registry (NASR), a clinical and biospecimen repository established to investigate the risk factors and mechanisms for SUDEP.
Researchers searched records of 189 decedents enrolled in the NASR from June 3, 2011, to June 3, 2016. Investigators identified cases based on a diagnosis of BECTS by parental report during the intake interview and confirmation from treating physicians. Diagnostic criteria for BECTS included the onset of epilepsy at age 3 to age 13, normal cognition and development before onset of seizures, no symptomatic cause of epilepsy, and EEG results reporting discharges consistent with BECTS.
Investigators identified three boys who had received a diagnosis of BECTS. The first patient and third patient met all clinical and EEG features for a diagnosis of BECTS. The second patient had limited EEG data, but had classic clinical features of BECTS.
The first patient (age 9) had seizure onset at age 5. He experienced an increase in focal motor and secondary generalized tonic-clonic seizures in the six months before his death.
The second patient (age 12) developed epilepsy at age 11. Four months prior to death, he experienced at least three nocturnal generalized tonic-clonic seizures. The autopsy report listed his cause of death as “cardiac dysrhythmia associated with lymphocytic myocarditis, left anterior descending coronary artery intramuscular tunneling, and possible hypertrophic cardiomyopathy.”
The third patient (age 13) developed epilepsy at age 3. His severe postictal state indicated that he might have had two generalized tonic-clonic seizures in the year before he died. His death was unwitnessed, and no autopsy was performed. None of these patients had received antiseizure medications or counseling with their families about the risk of SUDEP.
“These cases illustrate that SUDEP is a rare complication of BECTS, and this information should be shared with families at or soon after diagnosis,” said Ms. Doumlele and colleagues.
—Erica Tricarico
Suggested Reading
Doumlele K, Friedman D, Buchhalter J, et al. Sudden unexpected death in epilepsy among patients with benign childhood epilepsy with centrotemporal spikes. JAMA Neurol. 2017;74(6):645-649.
Benign epilepsy with centrotemporal spikes (BECTS) is associated with a low risk of sudden unexpected death in epilepsy (SUDEP), according to a study published in the June 1 JAMA Neurology. Clinicians should consider discussing this rare outcome with patients and their families and factoring this risk into treatment decisions, according to the authors.
“Our data suggest that even children with BECTS who have only focal motor or infrequent seizures are at risk for SUDEP,” said Kyra Doumlele, of New York University School of Medicine’s Department of Neurology, and colleagues.
BECTS is the most common focal epilepsy syndrome among children. This form of epilepsy typically has a good prognosis and tends to resolve before age 16. As a result, many neurologists do not prescribe antiseizure medications for patients with BECTS. However, patients with this disease have predominantly nocturnal seizures and some generalized tonic-clonic seizures, which could put them at risk for SUDEP.
SUDEP in well-characterized patients with BECTS has not been documented. Ms. Doumlele and colleagues sought to determine whether cases of BECTS were present in the North American SUDEP Registry (NASR), a clinical and biospecimen repository established to investigate the risk factors and mechanisms for SUDEP.
Researchers searched records of 189 decedents enrolled in the NASR from June 3, 2011, to June 3, 2016. Investigators identified cases based on a diagnosis of BECTS by parental report during the intake interview and confirmation from treating physicians. Diagnostic criteria for BECTS included the onset of epilepsy at age 3 to age 13, normal cognition and development before onset of seizures, no symptomatic cause of epilepsy, and EEG results reporting discharges consistent with BECTS.
Investigators identified three boys who had received a diagnosis of BECTS. The first patient and third patient met all clinical and EEG features for a diagnosis of BECTS. The second patient had limited EEG data, but had classic clinical features of BECTS.
The first patient (age 9) had seizure onset at age 5. He experienced an increase in focal motor and secondary generalized tonic-clonic seizures in the six months before his death.
The second patient (age 12) developed epilepsy at age 11. Four months prior to death, he experienced at least three nocturnal generalized tonic-clonic seizures. The autopsy report listed his cause of death as “cardiac dysrhythmia associated with lymphocytic myocarditis, left anterior descending coronary artery intramuscular tunneling, and possible hypertrophic cardiomyopathy.”
The third patient (age 13) developed epilepsy at age 3. His severe postictal state indicated that he might have had two generalized tonic-clonic seizures in the year before he died. His death was unwitnessed, and no autopsy was performed. None of these patients had received antiseizure medications or counseling with their families about the risk of SUDEP.
“These cases illustrate that SUDEP is a rare complication of BECTS, and this information should be shared with families at or soon after diagnosis,” said Ms. Doumlele and colleagues.
—Erica Tricarico
Suggested Reading
Doumlele K, Friedman D, Buchhalter J, et al. Sudden unexpected death in epilepsy among patients with benign childhood epilepsy with centrotemporal spikes. JAMA Neurol. 2017;74(6):645-649.