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.
Electroencephalography ; Epilepsia Partialis Continua ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Positron Emission Tomography Computed Tomography ; Retrospective Studies

Anti-N-methyl D-aspartate receptor (anti-NMDAR) encephalitis, recently recognized as a form of paraneoplastic encephalitis, is characterized by a prodromal phase of unspecific illness with fever that resembles a viral disease. The prodromal phase is followed by seizures, disturbed consciousness, psychiatric features, prominent abnormal movements, and autonomic imbalance. Here, we report a case of anti-NMDAR encephalitis with initial symptoms of epilepsia partialis continua in the absence of tumor. Briefly, a 3-year-old girl was admitted to the hospital due to right-sided, complex partial seizures without preceding febrile illness. The seizures evolved into epilepsia partialis continua and were accompanied by epileptiform discharges from the left frontal area. Three weeks after admission, the patient's seizures were reduced with antiepileptic drugs; however, she developed sleep disturbances, cognitive decline, noticeable oro-lingual-facial dyskinesia, and choreoathetoid movements. Anti-NMDAR encephalitis was confirmed by positive detection of NMDAR antibodies in the patient's serum and cerebrospinal fluid, and her condition slowly improved with immunoglobulin, methylprednisolone, and rituximab. At present, the patient is no longer taking multiple antiepileptic or antihypertensive drugs. Moreover, the patient showed gradual improvement of motor and cognitive function. This case serves as an example that a diagnosis of anti-NMDAR encephalitis should be considered when children with uncontrolled seizures develop dyskinesias without evidence of malignant tumor. In these cases, aggressive immunotherapies are needed to improve the outcome of anti-NMDAR encephalitis.
Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Anticonvulsants ; Antihypertensive Agents ; Cerebrospinal Fluid ; Child* ; Child, Preschool ; Cognition ; Consciousness ; D-Aspartic Acid ; Diagnosis ; Dyskinesias ; Encephalitis ; Epilepsia Partialis Continua* ; Female ; Fever ; Humans ; Immunoglobulins ; Immunotherapy ; Korea* ; Methylprednisolone ; Rituximab ; Seizures ; Virus Diseases

Lemierre's syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein. This disease is usually associated with a history of pharyngitis. The most common pathogens are Fusobacterium species, particularly Fusobacterium necrophorum. Lemierre's syndrome is seen most commonly in teenagers and young adults. We present a case report of a 67-year-old man with an atypical clinical manifestation of an uncommon pathogen in Lemierre's syndrome with epilepsia partialis continua.
Adolescent ; Aged ; Bacterial Infections ; Epilepsia Partialis Continua ; Fusobacterium ; Fusobacterium necrophorum ; Head ; Humans ; Jugular Veins ; Lemierre Syndrome* ; Neck ; Pharyngitis ; Thrombophlebitis ; Venous Thrombosis ; Young Adult

Seizure is one of the manifestations of nonketotic hyperglycemia (NKH). Partial motor seizures are observed in most cases and, occasionally, with epilepsia partialis continua. Generalized convulsive status epilepticus caused by NKH is rare. In this report, we present a case of a 68-year-old man who developed generalized convulsive status epilepticus as an initial manifestation of NKH.
Aged ; Epilepsia Partialis Continua ; Humans ; Hyperglycemia ; Seizures ; Status Epilepticus

No abstract available.
Epilepsia Partialis Continua ; Hemolysis ; Midazolam

Propofol has been used for the treatment of refractory status epilepticus, but propofol can cause propofol infusion syndrome (PRIS). PRIS is rarely developed and often fatal. The syndrome is characterized by metabolic acidosis, rhadomyolysis, and cardiac failure. Most reports were described in critically ill patients undergoing long-term propofol infusion at high doses. But many systemic confounding factors are mixed with the fatality of PRIS and there are no report on epilepsia partialis continua which has stable systemic conditions. We report PRIS in a patient with epilepsia partialis continua.
Acidosis ; Critical Illness ; Epilepsia Partialis Continua ; Heart Failure ; Humans ; Propofol ; Status Epilepticus

Rasmussen encephalitis (RE) is a rare, severe, and progressive disorder characterized by focal motor seizures, epilepsia partialis continua, hemiparesis, and intellectual decline. Although clinical features of RE are heterogenous, the progression of the disease is generally divided into three stages which are prodromal, acute, residual stages. We report a 29-year-old woman who had shown typical progression of the disease but preserved cognitive function during a longterm follow-up.
Adult ; Encephalitis ; Epilepsia Partialis Continua ; Female ; Follow-Up Studies ; Humans ; Paresis ; Seizures

OBJECTIVERasmussen syndrome (RS) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. The present study aimed to analyze the electroclinical characteristics and treatment of RS.

METHODSThe medical records of 16 children with RS were retrospectively reviewed.

RESULTSOf the 16 children, 8 were males and 8 were females. The age of onset was from 1 year and 11 months to 11 years and 6 months. The first symptom was seizure in all patients. The main seizure type was partial motor seizures. In all the patients, seizures gradually became frequent and in the form of epilepsia partialis continua (EPC). Thirteen cases developed hemiparesis. Fixed hemiparesis occurred from 2 months to 3 years after the onset of seizures. The cognitive deterioration was present in 14. The EEG background activity was abnormal in all the cases, asymmetric slow wave disturbances were bilateral but with unilateral predominance in 11, unilateral delta or theta wave in 8. The presence of interictal epileptiform discharges were found in all cases, unilateral in 11 and bilateral in 5. Seizures were recorded in all patients, no electroclinical correlation was found in 5. Serial magnetic resonance imaging (MRI) showed progressive unihemispheric or focal cortical atrophy in all cases. Six cases transiently showed focal cortical swelling or T2/FLAIR hyperintense signal on early scans. Antiepileptic drugs were not effective in any of the patients. Three of 10 patients receiving immunoglobulin, and 4 of 8 receiving corticosteroids, had some reduction of seizure frequency for a short period. Six patients accepted functional hemispherectomy, in 4 of them seizure no longer occurred and cognitive function was improved. The results of multiple subpial transection in 2 cases and focal resection in one patient were disappointing.

CONCLUSIONThe clinical features of RS were refractory partial epilepsy, progressive hemiplegia and cognitive deterioration. The EEG background was asymmetric with slow wave activity, interictal epileptiform discharges were unilateral or bilateral, no electroclinical correlation occurred. Serial MRI showed progressive unihemispheric focal cortical atrophy. Antiepileptic drugs were not effective for RS. In some patients, immunoglobulin or corticosteroids could reduce seizure frequency in the short term. Functional hemispherectomy could lead to seizure control and prevent further development of neurological impairment and cognitive deterioration.

Anticonvulsants ; therapeutic use ; Child ; Child, Preschool ; Cognition ; drug effects ; Electroencephalography ; Encephalitis ; drug therapy ; physiopathology ; prevention & control ; Epilepsia Partialis Continua ; drug therapy ; prevention & control ; Epilepsy ; etiology ; prevention & control ; Female ; Hemispherectomy ; methods ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Treatment Outcome

Atrophy ; Brain ; pathology ; physiopathology ; Child ; Diagnosis, Differential ; Electroencephalography ; Encephalitis ; complications ; diagnosis ; etiology ; physiopathology ; therapy ; Epilepsia Partialis Continua ; physiopathology ; Humans ; Magnetic Resonance Imaging ; Prognosis

We report a patient who showed vasogenic edema on MRI in association with partial status epilepticus. The patient, for a month, experienced clonic movements of the right arm and leg. As the amplitude and frequency of the clonic movements increased, resulting in epilepsia partialis continua, MRI showed characteristic vasogenic edema features;normal or decreased signal on diffusion-weighted imaging, and increased apparent diffusion coefficient value in the left parasagittal frontal region with strong leptomeningeal enhancement. This region was corresponded to the leg motor area, which was correlated with the degree of hypemetabolism of ictal FDG-PET findings. Considering limited progression of the seizure activity and focal slow waves on EEG, time-locked by clonic jerks, which might result from synaptic inhibition, this case suggests that vasogenic edema may be associated with a lower intensity of the seizure activity compared with cytotoxic edema noted in most of the reported patients.
Arm ; Diffusion ; Edema* ; Electroencephalography ; Epilepsia Partialis Continua ; Humans ; Leg ; Magnetic Resonance Imaging ; Seizures ; Status Epilepticus*