Abstract Objective To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.Methods A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.Results There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Two were diagnosed with Rasmussen encephalitis, one was diagnosed with focal cortical dysplasia, one was diagnosed with Alpers syndrome caused by POLG gene mutation, one was diagnosed with Angelman syndrome, and one was diagnosed with tuberculous meningitis. The latter two children had the predisposing factors for acute encephalopathy induced by status epilepticus and craniocerebral operation during the onset of EPC, while the other four children had natural progression of EPC. All the children had focal seizures except EPC, and symptoms included automatism, bilateral asymmetric tonic seizure, deflection, complex motor, and autonomic symptoms, with disturbance of consciousness in some children. EPC often lasted for several days or even several months. All children had abnormalities on head MRI, including local abnormal signal, cortex swelling, diffusive brain atrophy or brain atrophy at one side, local cortex thickening, and cortical necrosis. Head PET/CT scan was performed for three children and found local hypermetabolism or co-existence of hypermetabolism and hypometabolism. All the children had abnormalities on electroencephalography (EEG), with cerebral, hemispheric, or diffusive distribution of abnormal electrical activities, and during the onset of EPC, some EEG changes were recognizable and some were difficult to identify. All the children with EPC were not sensitive to antiepileptic drugs. EPC was relatively self-limiting in the child with Angelman syndrome. The child with focal cortical dysplasia underwent resection of epileptic foci and had good postoperative control, without neurological dysfunction. The child with Rasmussen encephalitis underwent functional hemispherectomy and had no attack after surgery, with neurological dysfunction. The child with Alpers syndrome had the worst prognosis.Conclusions EPC is a special type of epileptic seizures. Immune inflammation and metabolic etiologies are the main causes of EPC in children, and the selection of treatment regimens, treatment outcome, and prognosis depend on etiology.
ZHANG Mu,TANG Zhen-Li,WU Li-Wen et al. Etiology and clinical features of epilepsia partialis continua: an analysis of six cases[J]. CJCP, 2018, 20(12): 1008-1014.
ZHANG Mu,TANG Zhen-Li,WU Li-Wen et al. Etiology and clinical features of epilepsia partialis continua: an analysis of six cases[J]. CJCP, 2018, 20(12): 1008-1014.
Trinka E, Cock H, Hesdorffer D, et al. A definition and classification of status epilepticus--Report of the ILAE Task Force on Classification of Status Epilepticus[J]. Epilepsia, 2015, 56(10):1515-1523.
[2]
Vein AA, van Emde Boas W. Kozhevnikov epilepsy:the disease and its eponym[J]. Epilepsia, 2011, 52(2):212-218.
Atmaca MM, Bebek N, Kocasoy-Orhan E, et al. Epilepsia partialis continua:Correlation of semiology, outcome and electrophysiologic features[J]. Clin Neurol Neurosurg, 2018, 171:143-150.
[5]
Mameniskiene R, Bast T, Bentes C, et al. Clinical course and variability of non-Rasmussen, nonstroke motor and sensory epilepsia partialis continua:a European survey and analysis of 65 cases[J]. Epilepsia, 2011, 52(6):1168-1176.
[6]
Bejr-Kasem H, Sala-Padró J, Toledo M, et al. Epilepsia partialis continua:aetiology, semiology and prognosis in a Spanish adult cohort[J]. Epileptic Disord, 2016, 18(4):391-398.
[7]
Guerrini R. Physiology of epilepsia partialis continua and subcortical mechanisms of status epilepticus[J]. Epilepsia, 2009, 50(Suppl 12):7-9.
[8]
Mameniškienė R, Wolf P. Epilepsia partialis continua:A review[J]. Seizure, 2017, 44:74-80.
[9]
Bien CG, Elger CE. Epilepsia partialis continua:semiology and differential diagnoses[J]. Epileptic Disord, 2008, 10(1):3-7.
[10]
Kravljanac R, Djuric M, Jovic N, et al. Etiology, clinical features and outcome of epilepsia partialis continua in cohort of 51 children[J]. Epilepsy Res, 2013, 104(1-2):112-117.
[11]
Li H, Xue J, Qian P, et al. Electro-clinical-etiological associations of epilepsia partialis continua in 57 Chinese children[J]. Brain Dev, 2017, 39(6):506-514.
[12]
Longaretti F, Dunkley C, Varadkar S, et al. Evolution of the EEG in children with Rasmussen's syndrome[J]. Epilepsia, 2012, 53(9):1539-1545.
[13]
Engel J Jr. Report of the ILAE classification core group[J]. Epilepsia, 2006, 47(9):1558-1568.
[14]
Widdess-Walsh P, Diehl B, Najm I. Neuroimaging of focal cortical dysplasia[J]. J Neuroimaging, 2006, 16(3):185-196.
[15]
Misawa S, Kuwabara S, Hirano S, et al. Epilepsia partialis continua as an isolated manifestation of motor cortical dysplasia[J]. J Neurol Sci, 2004, 225(1-2):157-160.
[16]
Ikeda KM, Aldosari MM, Mirsattari SM, et al. Focal cortical dysplasia type Ⅱa manifesting as epilepsia partialis continua for 50 years[J]. Can J Neurol Sci, 2018, 45(1):106-108.
[17]
Varadkar S, Bien CG, Kruse CA, et al. Rasmussen's encephalitis:clinical features, pathobiology, and treatment advances[J]. Lancet Neurol, 2014, 13(2):195-205.
[18]
Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description:promising prospects and a dilemma[J]. Epilepsy Res, 2009, 86(2-3):101-112.
