Epilepsy & behavior : E&B · 2025
Electroclinical features of myoclonic absence epilepsy: A single-center cohort analysis in Southwest China.
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Hu Qiao, Luo Yuanyuan, Hong Siqi, Yuan Ping, Jiang Li
Аннотация
BACKGROUND AND OBJECTIVE: To date, few analyses of the electroclinical features and prognosis of patients with epilepsy with myoclonic absences (EMA) have been performed. We reviewed the clinical and electroencephalography (EEG) features, response to antiseizure medications, and long-term prognosis of patients with EMA in a single center in Southwest China, with the goal of increasing the understanding of the disease, strengthening early clinical diagnosis and treatment, and improving prognosis.
METHODS: We retrospectively analyzed the data of 11 children with EMA at the Epilepsy Center of Children's Hospital of Chongqing Medical University. The patients' history of EEG (including ictal EEG), medical history, head magnetic resonance imaging (MRI), medication, cognition and development were retrospectively analyzed.
RESULTS: Eleven patients (7:4 male:female) with an onset age of 7.18 ± 3.72 years had myoclonic-absence (MA) seizures, and four (36.36 %) had asymmetrical features. Nine (75 %) had multiple seizure types, including MA, myoclonic, absence, and generalized tonic-clonic seizures. During the interictal period, generalized 3 Hz spike-slow wave complexes were rhythmically emitted, with two having a small amount of focal discharge. In eight patients, bilateral symmetrically synchronized 3 Hz rhythmic spike-slow wave complex bursts, which showed a lock-in relationship with myoclonic, were recorded. All patients were treated with new or adjusted antiseizure medications and were followed up for 15-44 months. Five patients (45.45 %) had no seizures and no cognitive impairment; and 4/5 (80 %) were treated with valproic acid alone. During follow-up, the EEG results of four patients showed normal. The remaining six patients had drug-resistant epilepsy. Five had daily MA seizures, and one had daily myoclonic seizures. Among these six patients, four (66.67 %) exhibited developmental delay before disease onset, three experienced language delay, one experienced motor delay at follow-up.
CONCLUSION: The clinical manifestations of EMA are highly heterogeneous. Some patients experience seizures with atypical characteristics and exhibit significant clinical heterogeneity in response to antiseizure medications and cognitive impairment. Some patients have a better prognosis, and developmental delay before disease onset may be associated with a poor prognosis.
METHODS: We retrospectively analyzed the data of 11 children with EMA at the Epilepsy Center of Children's Hospital of Chongqing Medical University. The patients' history of EEG (including ictal EEG), medical history, head magnetic resonance imaging (MRI), medication, cognition and development were retrospectively analyzed.
RESULTS: Eleven patients (7:4 male:female) with an onset age of 7.18 ± 3.72 years had myoclonic-absence (MA) seizures, and four (36.36 %) had asymmetrical features. Nine (75 %) had multiple seizure types, including MA, myoclonic, absence, and generalized tonic-clonic seizures. During the interictal period, generalized 3 Hz spike-slow wave complexes were rhythmically emitted, with two having a small amount of focal discharge. In eight patients, bilateral symmetrically synchronized 3 Hz rhythmic spike-slow wave complex bursts, which showed a lock-in relationship with myoclonic, were recorded. All patients were treated with new or adjusted antiseizure medications and were followed up for 15-44 months. Five patients (45.45 %) had no seizures and no cognitive impairment; and 4/5 (80 %) were treated with valproic acid alone. During follow-up, the EEG results of four patients showed normal. The remaining six patients had drug-resistant epilepsy. Five had daily MA seizures, and one had daily myoclonic seizures. Among these six patients, four (66.67 %) exhibited developmental delay before disease onset, three experienced language delay, one experienced motor delay at follow-up.
CONCLUSION: The clinical manifestations of EMA are highly heterogeneous. Some patients experience seizures with atypical characteristics and exhibit significant clinical heterogeneity in response to antiseizure medications and cognitive impairment. Some patients have a better prognosis, and developmental delay before disease onset may be associated with a poor prognosis.