BACKGROUND

Super-refractory status epilepticus (SRSE) is a devastating neurological emergency with high morbidity and mortality, and fewer than 10% of survivors achieve full recovery (mRS 0). Epilepsia partialis continua (EPC) as a clinical manifestation of SRSE is rare in adults. Longterm seizure remission after SRSE, especially with documented serial EEG normalization, is seldom reported.

A 49-year-old woman with no prior comorbidities presented with focal motor seizures of the left upper limb that evolved into EPC and SRSE. The ictal EEG revealed right central (C4) epileptiform discharges correlating with contralateral motor activity and secondary left temporal spread. Despite treatment with multiple antiseizure medications and escalation to triple-anesthetic therapy (midazolam, propofol, thiopental), seizures persisted for six weeks. Complications included suspected propofol infusion syndrome, hypernatremia, stress-related gastrointestinal bleeding, multidrug-resistant pneumonia and bacteremia (K. pneumoniae, P. aeruginosa, MRSA, A. baumannii), thiopental-induced agranulocytosis, and paroxysmal atrial fibrillation. Seizures gradually abated, and she was successfully extubated. Serial EEGs showed progressive resolution, from hemispheric polyspike discharges to normalized alpha rhythm, paralleling recovery. Follow-up MRI revealed resolution of cortical hyperintensities with residual right hippocampal atrophy. Over two years, antiseizure medications were tapered to valproate monotherapy. She remained seizure-free with normal EEGs and full functional independence (mRS 0).

This case illustrates a rare trajectory of adult-onset EPC evolving into SRSE with complete neurologic and electrophysiologic recovery. It underscores the value of serial EEG monitoring, vigilant critical care, and long-term follow-up in achieving remission even in resource-limited settings.

BACKGROUND

Super-refractory status epilepticus (SRSE) is a devastating neurological emergency with high morbidity and mortality, and fewer than 10% of survivors achieve full recovery (mRS 0). Epilepsia partialis continua (EPC) as a clinical manifestation of SRSE is rare in adults. Longterm seizure remission after SRSE, especially with documented serial EEG normalization, is seldom reported.

A 49-year-old woman with no prior comorbidities presented with focal motor seizures of the left upper limb that evolved into EPC and SRSE. The ictal EEG revealed right central (C4) epileptiform discharges correlating with contralateral motor activity and secondary left temporal spread. Despite treatment with multiple antiseizure medications and escalation to triple-anesthetic therapy (midazolam, propofol, thiopental), seizures persisted for six weeks. Complications included suspected propofol infusion syndrome, hypernatremia, stress-related gastrointestinal bleeding, multidrug-resistant pneumonia and bacteremia (K. pneumoniae, P. aeruginosa, MRSA, A. baumannii), thiopental-induced agranulocytosis, and paroxysmal atrial fibrillation. Seizures gradually abated, and she was successfully extubated. Serial EEGs showed progressive resolution, from hemispheric polyspike discharges to normalized alpha rhythm, paralleling recovery. Follow-up MRI revealed resolution of cortical hyperintensities with residual right hippocampal atrophy. Over two years, antiseizure medications were tapered to valproate monotherapy. She remained seizure-free with normal EEGs and full functional independence (mRS 0).

This case illustrates a rare trajectory of adult-onset EPC evolving into SRSE with complete neurologic and electrophysiologic recovery. It underscores the value of serial EEG monitoring, vigilant critical care, and long-term follow-up in achieving remission even in resource-limited settings.

Abstract

Rasmussen’s syndrome is a neuroimmune disease characterized by progressive unilateral cerebral dysfunction, for which hemispherectomy remains the definitive treatment. We report a 9-year-old right handed Filipino girl with drug-resistant focal seizures, epilepsia partialis continua, and progressive right hemiparesis due to left hemispheric involvement. Epilepsy surgery was not immediately feasible because of parental concerns and logistical constraints in this resource-limited setting. Despite treatment with multiple antiseizure medications, seizures remained uncontrolled, prompting initiation of immunotherapy with intravenous immunoglobulin followed by rituximab. Rituximab was associated with marked seizure reduction and functional improvement, highlighting its potential role in the management of Rasmussen’s syndrome when surgery is not feasible.

Keywords: Rasmussen’s syndrome, epilepsia partialis continua, IVIG, rituximab

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

No abstract available.
Epilepsia Partialis Continua ; Hemolysis ; Midazolam

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

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