Epilepsia Partialis Continua (EPC) is a variant of focal motor status epilepticus wherein frequent repetitive, usually rhythmic muscle jerks continue over prolonged periods of time. EPC can occur because of any space-occupying lesion, vascular aetiology, mitochondrial disease, and Rasmussen encephalitis. There is a paucity of evidence regarding the prevalence; however, regional studies have suggested a prevalence of one per million population. This case series presents three cases wherein the aetiology has been known to have a highly rare correlation with the clinical presentation of EPC. Index case one, already a known case of generalised epilepsy, presented with clinical features suggestive of acute viral hepatitis, later found to be hepatitis A positive, had EPC that lasted around four days. In index case two, the child presented with features suggesting focal status epilepticus, which also persisted despite use of multiple anti-epileptic agents, and was later found to be positive for parvovirus antibodies in serum as well as Cerebrospinal Fluid (CSF). In index case three, the child presented with focal seizures, which on imaging had structural changes and an abnormal Electroencephalography (EEG). These children were given multiple antiepileptic agents; however, the repetitive movement subsided on its own and all of them were discharged on haemodynamically stable conditions. The present case series highlights the clinical heterogeneity of the disease process, as well as the challenges and complexities involved in diagnosis and management.

Epilepsia partialis continua and Todd paralysis: Case report and literature review

Late-onset Rasmussen encephalitis: 3 illustrative cases and a review of the literature.

Rasmussen encephalitis (RE) is a progressive disease characterised by unilateral brain atrophy, drug-resistant epilepsy, epilepsia partialis continua, hemiparesis and cognitive decline. Early initiation of immunomodulatory therapy is crucial to slow disease progression. However, early formal diagnosis is challenging as it typically requires hemispheric atrophy or brain biopsy. This case reports on a preschool-aged boy with RE who began immunotherapy before demonstrating clear hemispheric atrophy. Follow-up MRI did not indicate global hemispheric atrophy; however, FreeSurfer-based volumetric analysis revealed a decreased left:right hemispheric volume ratio, suggesting early left hemispheric atrophy. Subsequently, intensive immunotherapy was administered. Over 3 years of treatment, the patient exhibited gradual hemispheric atrophy on MRI, along with mild motor and cognitive impairments. Serial FreeSurfer-based volumetric analysis may contribute to detecting subtle hemispheric volume changes in RE, facilitating prompt diagnosis and early initiation of immunotherapy-potentially limiting disease progression.

Multifocal paraneoplastic encephalitis associated with anti-GABA-B and anti-Hu antibodies manifesting with status epilepticus and epilepsia partialis continua: Expanding the clinical-radiological spectrum.

Rasmussen syndrome (RS) is a neuroimmune disease typically characterized by refractory focal epilepsy, epilepsia partialis continua, progressive focal neurologic deficits, and cognitive impairment. Seizures are often a presenting and prominent symptom, but RS may be diagnosed in the absence of seizures when a patient has 2 of the 3 Part B Bien criteria: (1) consistent histopathologic findings, (2) progression in unilateral cortical deficits, or (3) progression in focal cortical atrophy. We describe a patient who presented with hemiparesis and unihemispheric atrophy, meeting Bien criteria through progressive clinical changes and consistent histopathology on a brain biopsy. She did not have progression in focal cortical atrophy on imaging at the time of diagnosis. The patient has not developed seizures in the 4.5 years of monitoring after symptom onset. Our case illustrates that the underlying pathophysiology of RS is an immune-driven, progressive neurodegenerative disorder, which frequently-but not always-causes seizures.

Background: Epilepsia partialis continua (EPC) is a form of focal status epilepticus, often requiring multiple anti-seizure medications (ASM). There are no established guidelines for pharmacological management. Perampanel (PER), an AMPA receptor antagonist, has gained attention in treating status epilepticus based on limited case reports. This study evaluates the efficacy of PER in treating EPC. Methods: We retrospectively analyzed the treatment response and adverse effects of PER in patients with EPC at our local hospital from January 2024 to January 2025. Seven patients with EPC were included. The loading dose of PER ranged from 6 mg to 20 mg. The etiology of EPC was intracranial hemorrhage (70%) and glioblastoma (30%). A clinical response was defined as seizure freedom within 72 hours of initiating PER, with PER being the last ASM added. Results: PER resulted in seizure termination in 6 of 7 patients. Time to response ranged from 24 to 72 hours. Oral PER, up to a dose of 16 mg, was well-tolerated in conscious patients. Common side effects included sedation, delirium, agitation, and nausea. Conclusions: Our real-world data suggest that PER can be an effective and well-tolerated early adjunct therapy in EPC, particularly at higher oral loading doses in conscious patients.

Lingual seizures are rare hyperkinetic tongue movements with significant clinical implications due to their epileptogenic origin. Despite their diagnostic value, these seizures are often underrecognized, particularly when electroencephalographic (EEG) findings are inconclusive. This study aims to characterize their clinical features, EEG patterns, imaging findings and underlying causes, emphasizing the need for increased awareness and improved diagnosis. A retrospective review identified patients with isolated lingual seizures or those with additional motor involvement. Data on demographics, seizure characteristics, EEG findings, imaging results and underlying causes were collected and analyzed. Seizures were classified based on the International League Against Epilepsy (ILAE) 2017 framework to refine their clinical and diagnostic profiles. Thirteen patients were identified: 11 with focal-aware and 1 with focal-unaware seizures. Seven had epilepsia partialis continua, and five experienced frequent seizures. Seizure involvement was limited to the tongue in four cases, extended to cranial muscles in seven and affected the tongue, cranial and extremity muscles in two. Significant ictal EEG findings were noted in only three patients with extensive motor involvement. However, nine patients had acute cerebral lesions, associated with glial tumors, encephalitis, chronic gliosis or cortical hemorrhage. This study provides a detailed characterization of lingual seizures, highlighting their clinical, electrophysiological and imaging features. Given their rarity and underdiagnosis, our findings offer valuable guidance for clinicians, underscoring the importance of improved recognition and diagnostic strategies for this distinct seizure type.

Repetitive Transcranial Magnetic Stimulation for Refractory and Super-refractory Status Epilepticus: A Systematic Review.

Rasmussen’s Encephalitis is a rare neurological condition causing inflammation of the brain, which thereafter can result in seizures, neuronal death, and significant neurological deficits. Its exact cause is unknown and it typically presents in children. The best known treatment is a surgical hemispherectomy to prevent the spread of inflammation, however this is invasive and not without risk. The best therapeutic approach has been with immunomodulation using the antimetabolite agent, azathioprine. Monoclonal antibodies such as rituximab have shown promise in early clinical trials for the treatment of Rasmussen’s Encephalitis, but little has been done to compare the efficacy of rituximab versus azathioprine. A systematic review was then conducted with a study in which patients were treated with rituximab for Rasmussen’s Encephalitis (RE) compared to a study in which patients were treated with azathioprine. The presence of focal encephalitis characterized by epilepsia partialis continua (EPC) was measured in both studies following the treatments. Azathioprine and Rituximab both had statistically significant effects on decreasing the incidence of epilepsia partialis continua when compared to their respective controls. However, rituximab seemed to have a greater effect on reducing the incidence of EPC than azathioprine. Rituximab seems to have an immense potential in reducing EPC in patients with RE. For this review, Rituximab seemed to have a greater reduction in EPC than azathioprine. Further studies with greater sample sizes assessing reductions in the several other complications of RE must be conducted in order to confirm whether or not Rituximab is truly a better choice than Azathioprine.

Epilepsia Partialis Continua without Radiological Abnormalities in an HIV-infected Patient: A Case Report

Rasmussen encephalitis (RE) is a rare and progressive form of chronic encephalitis that typically affects one hemisphere of the brain and primarily occurs in pediatric individuals. The current study aims to narratively review the literature about RE, including historical information, pathophysiology, and management of this condition. RE often occurs in individuals with normal development, and it is estimated that only a few new cases are identified each year in epilepsy centers. Approximately 10% of cases also occur in adolescents and adults. The hallmark feature of RE is drug-resistant focal seizures that can manifest as epilepsia partialis continua. Also, patients with RE usually develop motor and cognitive impairment throughout the years. Neuroimaging studies show progressive damage to the affected hemisphere, while histopathological examination reveals T-cell-dominated encephalitis with activated microglial cells and reactive astrogliosis. The current therapy guidelines suggest cerebral hemispherotomy is the most recommended treatment for seizures in RE, although significant neurological dysfunction can occur. Another option is pharmacological management with antiseizure medications and immunomodulatory agents. No significant progress has been made in understanding the pathophysiology of this condition in the last decades, especially regarding genetics. Notably, RE diagnosis still depends on the criteria established by Bien et al., and the accuracy can be limited and include genetically different individuals, leading to unexpected responses to management.

Paragonimiasis is a parasitic infection that occurs after ingesting freshwater crayfish or crabs, and <i>Paragonimus westermani</i> (<i>P. westermani</i>) species are the most important in clinical practice. Cerebral paragonimiasis is one of the most important extra-pulmonary forms with severe outcomes and higher mortality compared to the pulmonary forms. Epilepsia partialis continua (EPC) is defined as a variant of focal motor status epilepticus in which frequent repetitive muscle jerks, usually arrhythmic, continue over 1 hour. Cerebral infections of <i>P. westermani</i> often cause simple epileptic seizures, but cases progressing to EPC are very rare. The authors report a case of confirming and treating a newly developed recurrent focal onset seizures in a patient with chronic cerebral paragonimiasis treated over 30 years ago.

IntroductionEpilepsia Partialis Continua (EPC) is a challenging condition in which repetitive transcranial magnetic stimulation (rTMS) can induce a neuromodulation effect of potential diagnostic and therapeutic value. MethodsA comprehensive literature search was conducted using Pubmed and Web of Science databases to identify cases of EPC who underwent rTMS, including children and adults. Additionally, we present two patients from our centre who underwent rTMS at a low frequency (0.5 Hz) with simultaneous EEG recording with the aim of assessing potential improvement in seizure frequency and severity. ResultsEight articles were selected comprising 16 patients (15 with EPC and one with continuous myoclonia). In three of these patients, no clinical or EEG changes were noted; the remaining cases showed transitory clinical improvements. We report two patients with EPC, in whom low frequency rTMS was associated with transient reduction in frequency and severity of seizures and improvements in hand function and dexterity. In one of these cases, rTMS suggested a potential target for intracranial recordings, subacute cortical stimulation and localised resection. ConclusionIn selected patients with EPC, rTMS can be used as a potential diagnostic and therapeutic tool.

BackgroundFocal epilepsy is common in children and adults with mitochondrial disease. Seizures are often refractory to pharmacological treatment and, in this patient group, frequently evolve to refractory focal status epilepticus (also known as epilepsia partialis continua). Where this occurs, the long-term prognosis is poor. Transcranial DC stimulation (tDCS) is a promising, non-invasive, adjunctive treatment alternative to common surgical procedures. Limited recruitment of study participants with this rare disease and the ethical challenges of administering a treatment to one group and not another, while maintaining strict methodological rigour can pose challenges to the design of a clinical study.MethodWe designed the first delayed start, double-blinded, sham-controlled study to evaluate the efficacy of tDCS as an adjunctive treatment for focal epilepsy. We will include participants with a genetically confirmed diagnosis of mitochondrial disease with drug-resistant focal epilepsy aged ≥ 2 years, aiming to collect 30 episodes of focal status epilepticus, each treated for a maximum period of 14 days. The early start intervention arm will receive tDCS from day 1. The delayed start intervention arm will receive sham stimulation until crossover on day 3. Our primary endpoint is a greater than 50% reduction from baseline (on day 0) in seizure frequency assessed by 3x daily reporting, accelerometery, and video monitoring. Changes in the underlying epileptogenic focus within the brain related to the tDCS intervention will be assessed by magnetic resonance imaging (MRI) and/or electroencephalography (EEG).DiscussionStudy results in favour of treatment efficacy would support development of tDCS into a mainstream treatment option for focal epileptic seizures related to mitochondrial disease.Trials registrationISRCTN: 18,241,112; registered on 16/11/2021.

Epilepsia Partialis Continua Following Alemtuzumab Treatment: First Report Following Solid Organ Transplant and the First in a Pediatric Patient

Epilepsia partialis continua (EPC) is a variant of focal motor status epilepticus that can occur as a single or repetitive episode with progressive or nonprogressive characteristics. The authors describe the feasibility of identifying focal EPC in a 33-year-old woman using video electroencephalography (VEEG), electroencephalography source localization, [18F]fluorodeoxyglucose positron emission tomography, magnetic resonance imaging, and psychiatric and neuropsychological assessments and of treating it with stereo electroencephalography-guided radiofrequency (SEEG-RF) ablation. EPC comprised recurrent myoclonus of the right thigh and iliopsoas with a progressive pain syndrome after left anterior-temporo-mesial resection. Switching between VEEG under regular and epidural block helped to define myoclonus as the presenting ictal symptom with a suspected seizure onset zone in the left parietal paramedian lobule. After the epileptic network was identified, SEEG-RF ablation abolished all seizures. No correlation was found between pain and VEEG/SEEG abnormalities. Rehabilitation began 3 days after the SEEG-RF ablation. By 1 year of follow-up, the patient had no EPC and could walk with assistance in rehabilitation; however, due to the abrupt abolishment of EPC and underlying psychological factors, the patient perceived her pain as overriding, which prevented her from walking. The application of SEEG-RF ablation is an efficient therapeutic option for focal EPC with special concerns regarding concurrent nonepileptic pain. https://thejns.org/doi/10.3171/CASE23611.

Epilepsia partialis continua: Kozhevnikov and his German contemporaries

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We aimed to provide a detailed phenotypic description of status epilepticus (SE) in a large cohort of patients with POLG disease and identify prognostic biomarkers to improve the management of this life-threatening condition. In a multinational, retrospective study with data on patients with POLG disease from seven European countries, we identified those who had SE. The age of SE onset, accompanying clinical, laboratory, imaging and genetic findings were analysed. One hundred and ninety-five patients with genetically confirmed POLG disease were recruited, of whom 67% (130/194) had epilepsy. SE was identified in 77% (97/126), with a median age of SE onset of 7 years. SE was the presenting symptom of the disease in 43% (40/93) of those with SE, while 57% (53/93) developed SE during the disease course. Convulsive SE was reported in 97% (91/94) followed by epilepsia partialis continua in 67% (56/84). Liver impairment 78% (74/95), ataxia 69% (60/87), stroke-like episodes 57% (50/88), were the major comorbidities. In the majority (66%; 57/86) with SE this became refractory or super-refractory. The presence of seizures was associated with significantly higher mortality compared to those without (P ≤ 0.001). The median time from SE debut to death was 5 months. SE is a major clinical feature of POLG disease in early and juvenile to adult-onset disease and can be the presenting feature or arise as part of a multisystem disease. It is associated with high morbidity and mortality, with the majority of patients with SE going on to develop refractory or super-refractory SE.