Epilepsia Partialis Continua as a Sequelae of Measles Infection in Children With Hematolymphoid Malignancies.

PURPOSE: To share our clinical experience with the diagnosis and management of children with hematolymphoid malignancies presenting with epilepsia partialis continua (EPC) as a sequelae of measles infection. MATERIALS AND METHODS: In December 2022, a series of children in our hemato-oncology unit presented with focal status epilepticus with no conclusive evidence pointing toward any underlying etiology. One such child had a typical measles rash a few weeks before the onset of this focal status epilepticus. After a series of cases with a similar presentation, a clinical pattern suspicious for measles became evident. cerebrospinal fluid polymerase chain reaction was positive for measles virus with measles immunoglobin M detected in the serum. This led to the diagnosis of measles inclusion-body encephalitis in a series of children who presented with EPC over a period of 3 months. EPC is a rare manifestation of measles that is seen only in immunocompromised patients. RESULTS: Among the 18 children reported in this series, only 10 had a history of rashes. The rash was mostly transient and elicited only on retrospective history taking. Five of the 18 children who did not lose consciousness during the prolonged seizure episode survived the disease but had residual neurologic sequelae. Among the 18 children, two were unimmunized and immunization status could not be confirmed in three other children. CONCLUSION: This case series highlights the threats posed by measles infection in children with cancer who are immunosuppressed because of the underlying disease and ongoing chemotherapy. Loss of herd immunity because of declining measles immunization rates secondary to vaccine hesitancy and COVID-19 lockdown pose a greater risk of measles infection and its complications for patients with deficient immune systems.

Response of focal refractory status epilepticus to lacosamide in an infant.

Status epilepticus (SE) is a life-threatening medical emergency which is frequently encountered in the critical care setting and can be refractory to treatment. Refractory status epilepticus (RSE) is defined as SE that has failed to respond to adequately used first-line and second-line antiepileptic medications. Super refractory status epilepticus is defined as SE that persists for 24 hours or more after the use of an anaesthetic agent or recurs after its withdrawal.If SE persists beyond a period of 7 days it is referred to as prolonged, refractory status epilepticus (PRSE). There are limited data guiding treatment of RSE in the paediatric population.Lacosamide (LCM) is licensed as an adjunctive treatment for partial-onset seizures. Evidence for the efficacy of LCM in paediatric SE is scarce. This case report may suggest a synergistic effect of LCM on slow-activation sodium channels in conjunction with medications such as phenytoin that causes fast inactivation of sodium channels. The dual fast and slow inactivation of sodium channels may enhance the effectiveness in treatment of RSE. This is the first case report of PRSE in an infant, successfully treated with LCM. A brief review of literature is also a part of this report.

Abdominal epilepsia partialis continua in a patient with astrocytoma treated with Lacosamide - value of repetitive EEG recordings.

OBJECTIVE: Isolated abdominal epilepsia partialis continua (EPC) without the involvement of other body parts is rarely seen. Abdominal EPC usually occurs either as a part of hemibody EPC or as an evolution of refractory EPC after initial treatment. As the isolated abdominal EPC was rarely reported up to date, the data regarding its pathophysiology and management are limited. Herein, we aimed to describe the clinical, neuroimaging, and electroencephalographic findings of a patient with abdominal EPC. PATIENTS AND METHODS: A 48-year-old woman with a history of surgical resection for right posterior frontal astrocytoma was admitted with left abdominal EPC. Magnetic resonance imaging of the brain showed a residual mass lesion and encephalomalacia in the right frontoparietal region. RESULTS: Although the initial electroencephalography (EEG) was normal, independent spikes were detected in the right frontal and parietal derivations in the second EEG. Although her EPC was refractory to levetiracetam, lamotrigine, phenytoin, and gabapentin, oral lacosamide treatment ceased the seizures. CONCLUSIONS: The history of this patient emphasizes the necessity of repetitive recordings in case of a normal initial EEG. The independent spikes in her frontal and parietal regions suggested the presence of a large epileptogenic zone generating independent epileptiform activities in the pre-central motor cortex and the post-central sensory cortex as the pathophysiologic phenomena in persistent abdominal EPC. To the best of our knowledge, this is the first report presenting a patient experiencing an abdominal EPC due to a cerebral mass resolved with lacosamide suggesting this drug is a promising treatment option in resistant EPC.

Successful treatment of epilepsia partialis continua with perampanel: two pediatric cases.

Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus, associated with multiple etiologies. Etiology-specific treatments, such as hemispherotomy for Rasmussen encephalitis, lesionectomy for focal cortical dysplasia, and metabolic correction for non-ketotic hyperglycemia, have proven to be efficacious in treating EPC, but, in general, EPC is difficult to treat and often drug-resistant, and there is little evidence to guide therapy. We report the successful treatment of EPC with perampanel in two pediatric patients. The first patient was a 12-year-old boy with neuronal ceroid lipofuscinosis (NCL) who started to have EPC around the age of 10 years, characterized by left hemifacial myoclonic twitches and hemi-body jerks that were almost continuous throughout the day and disappeared during sleep. He had failed several antiepileptic drugs (AEDs). The EPC stopped within three days of initiating perampanel. The second patient was a six-year-old boy with POLG-related mitochondrial disease who presented to the emergency room with continuous jerky movements of the right arm and face after a trivial head injury. After failing several AEDs, including a midazolam drip, the EPC was controlled with perampanel. Both patients showed dramatic improvement and continue to show sustained efficacy after around five months of follow-up. Based on our observations, perampanel, which has a unique mechanism of action, appears to be a promising therapeutic option for treating EPC. [Published with video sequence].

Epilepsia Partialis Continua as an Early Sign of Anti-Myelin Oligodendrocyte Glycoprotein Antibody-positive Encephalitis.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been associated with steroid-responsive cortical encephalitis and comorbid generalized epilepsy. A 44-year-old woman developed repeated epilepsia partialis continua (EPC) without generalized seizures and was anti-MOG antibody-positive. Radiological abnormalities were detected in the bilateral medial frontoparietal cortices, but there were no cerebrospinal fluid abnormalities. She achieved remission with anti-epileptic drugs alone. However, encephalitis recurred four months later when pleocytosis appeared, and steroid therapy was effective. Altogether, EPC without typical cerebrospinal fluid features can be an early sign of anti-MOG antibody-positive encephalitis. Thus, patients with EPC of unknown etiology need to be screened for anti-MOG antibodies.

Epilepsia partialis continua complicated by disseminated tuberculosis and hemophagocytic lymphohistiocytosis: a case report.

BACKGROUND: We describe a patient copresenting with epilepsia partialis continua, tuberculosis, and hemophagocytic lymphohistiocytosis. To our knowledge, this is the first documented case of this triad. CASE PRESENTATION: A 54-year-old black South African woman presented to a hospital in Scotland with an acute history of right-sided facial twitching, breathlessness, and several months of episodic night sweats. Clinical examination revealed pyrexia and continuous, stereotyped, right-sided facial contractions. These worsened with speech and continued through sleep. A clinical diagnosis of epilepsia partialis continua was made, and we provide a video of her seizures. Computed tomographic imaging of the chest and serous fluid analyses were consistent with a diagnosis of disseminated Mycobacterium tuberculosis. An additional diagnosis of hemophagocytic lymphohistiocytosis was made following the identification of pancytopenia and hyperferritinemia in peripheral blood, with hemophagocytosis evident in bone marrow investigation. We provide images of her hematopathology. The patient was extremely unwell and was hospitalized for 6 months, including two admissions to the intensive care unit for ventilatory support. She was treated successfully with high doses of antiepileptic drugs (benzodiazepines, levetiracetam, and phenytoin) and 12 months of oral antituberculosis therapy, and she underwent chemotherapy with 8 weeks of etoposide and dexamethasone for hemophagocytic lymphohistiocytosis, followed by 12 months of cyclosporine and prednisolone. CONCLUSIONS: This combination of pathologies is unusual, and this case report helps educate clinicians on how such a patient may present and be managed. A lack of evidence surrounding the coexpression of this triad may represent absolute rarity, underdiagnosis, or incomplete case ascertainment due to early death caused by untreated tuberculosis or hemophagocytic lymphohistiocytosis. Further research is needed.

Abdominal epilepsia partialis continua in neurocysticercosis.

Epilepsia partialis continua (EPC) of abdominal muscles is a rare entity with variable clinical localization and aetiology. A 25-year-old man presented with sudden onset of intermittent focal myoclonic movements involving the abdominal muscles on the right side exclusively, lasting from 20 minutes to an hour. Brain MRI revealed a ring-enhancing lesion, suggestive of cysticercal granuloma over the left precentral gyrus. The patient fulfilled the revised diagnostic criteria for definitive diagnosis of neurocysticercosis. EEG did not show focal abnormalities during the events. Episodes of EPC were controlled with difficulty using 600 mg oxcarbazepine, 200 mg lacosamide, and 2,000 mg levetiracetam. The patient received antiparasitic therapy with albendazole (15 mg/kg for two weeks) and oral dexamethasone (0.1 mg/kg) for two weeks which was then tapered. The involvement of the primary motor cortex during ictal propagation may account for this curious phenomenon. This is the first report of abdominal EPC in a patient with inflammatory granuloma as a result of neurocysticercosis.

Dexamethasone as Abortive Treatment for Refractory Seizures or Status Epilepticus in the Inpatient Setting.

Refractory seizures or status epilepticus (RS/SE) continues to be a challenge in the inpatient setting. Failure to abort a seizure with antiepileptic drugs (AEDs) may lead to intubation and treatment with general anesthesia exposing patients to complications, extending hospitalization, and increasing the cost of care. Studies have shown a key role of inflammatory mediators in seizure generation and termination. We describe 4 patients with RS/SE that was aborted when dexamethasone was added to conventional AEDs: a 61-year-old female with temporal lobe epilepsy who presented with delirium, nonconvulsive status epilepticus, and oculomyoclonic status; a 56-year-old female with history of traumatic left frontal lobe hemorrhage who developed right face and hand epilepsia partialis continua followed by refractory focal clonic seizures; a 51-year-old male with history of traumatic intracranial hemorrhage who exhibited left-sided epilepsia partialis continua; and a 75-year-old female with history of breast cancer who manifested nonconvulsive status epilepticus and refractory focal clonic seizures. All patients continued experiencing RS/SE despite first- and second-line therapy, and one patient continued to experience RS/SE despite third-line therapy. Failure to abort RS/SE with conventional therapy motivated us to administer intravenous dexamethasone. A 10-mg load was given (except in one patient) followed by 4.0- 5.2 mg q6h. All clinical and electrographic seizures stopped 3-4 days after starting dexamethasone. When dexamethasone was discontinued 1-3 days after seizures stopped, all patients remained seizure-free on 2-3 AEDs. The cessation of RS/SE when dexamethasone was added to conventional antiseizure therapy suggests that inflammatory processes are involved in the pathogenesis of RS/SE.

Epilepsia partialis continua following a Western variant tick-borne encephalitis.

Epilepsia partialis continua (EPC) is a rare entity, first described in 1894 by Kozevnikov, as a variant of simple focal motor status epilepticus. EPC is most frequently characterized by motor symptoms, but as recently described, non-motor manifestations may occur, such as somatosensory symptoms or aura continua. EPC in adults has been attributed to various etiologies: infectious, vascular, neoplastic, and metabolic. According to the recent definition, we reported a case of EPC with behavioral symptoms, following a tick-borne encephalitis (TBE) contracted in an endemic area (North Eastern Italy). Patient's symptom was a poorly localized "whole body sensation", which is reported as a condition occurring only in frontal lobe epilepsy. Patient's EEG showed a left frontal predominance of epileptiform discharges. Literature highlighted the importance of the Far-eastern TBE variant as a cause of EPC, since no Western variant TBE cases are reported. In contrast to what was claimed so far, our case demonstrates that not only the Far-eastern TBE variant, but also Western variant TBE is a cause of EPC. Prognosis of EPC depends largely on the underlying etiology, and it is frequently drug-resistant. Our patient was treated with intravenous levetiracetam, with a subsequent clinical recovery and a disappearance of epileptiform discharges. The rapid clinic and electroencephalographic response to levetiracetam confirm that it can be a promising therapeutic option for treatment of EPC.

Neuropsychological assessment as an objective tool to monitor treatment response in anti-N-methyl-D-aspartate receptor encephalitis.

We report a 1-year follow-up of a young woman with anti-N-methyl-D-aspartate receptor encephalitis. Management of autoimmune encephalitis remains challenging as objective and clinically relevant biomarkers are sought, which allow for the monitoring of treatment response. While further investigation is required, we believe that this case highlights the importance of following a comprehensive neuropsychological profile as a clinically relevant biomarker to guide therapeutic decision-making. By relying on the neuropsychological assessment of the patient, treatment with more toxic medications was avoided and her antiepileptic drug regimen was simplified.

Long-term follow-up for patients with nonprogressive epilepsia partialis continua in a single center in China.

Epilepsia partialis continua (EPC) is a rare variant of epilepsy. Cases from China are rare. We present a case series of seven patients to analyze its clinical features, imagining findings, etiology, drug use, and long-term outcome in a single epilepsy center. We made assessments of drug effects twice (Stage I - when they left our hospital; Stage II in March 2017 - by telephone interviews to rate their long-term outcome). The mean duration of the second follow-up was 4.8years. Of the seven patients, four patients characterized motor and sensory EPC and three motor EPC. Local distributions of EPC were: the left face (2 patients), right face (1 patient), left leg (3 patients), right leg and arm (1 patient). CT/MR was abnormal in four, normal in two, and not available in one patient. EEG abnormalities commonly consisted of spike-waves, sharp-waves (or) slow wave activity, and periodic lateralized epileptiform discharges. They were all nonprogressive EPC (encephalitis: 2; tumor: 2; head trauma: 1; and not found in 2 cases). In our observations, topiramate might be effective in patients with facial muscles continuous jerking, while carbamazepine in cases of limbs continuous myotonia. Our cases had favorable long-term outcome. Thus, our cases' etiology differentiated from other regions. Some drugs used by referring to EPC distributions might help to control EPC and their outcome were usually favorable.

Electro-clinical-etiological associations of epilepsia partialis continua in 57 Chinese children.

OBJECTIVE: Epilepsia partialis continua (EPC) was one type of focal status epilepticus. The aim of this study was to analyze the clinical and electroencephalography (EEG) characteristics, and outcome of 57 child-onset patients with EPC according to different etiologies, and further explore the electro-clinical-etiological associations. METHODS: We retrospectively reviewed 57 children diagnosed with EPC in our department over last ten years. Etiology, clinical and EEG data, and outcome were categorized and analyzed. RESULTS: For the 57 child-onset patients, EPC was caused by different etiologies, including immune-related disease (43.9%), focal lesions (17.5%), inborn errors of metabolism (24.6%), and unknown (14.0%). EEG background abnormalities showed generalized slowing in 45 patients (78.9%) and focal slowing in two patients (3.5%). Nineteen patients (33.3%) presented clear correlation of ictal EEG/EMG and the remaining 38 patients (66.7%) showed no clear correlation of ictal EEG/EMG. Both EEG background activity and ictal EEG/EMG correspondence among different etiologies had statistical significance (P<0.05). The ictal patterns without clear EEG/EMG correspondence in immune-related disease and the ictal patterns with clear EEG/EMG correspondence in focal lesions were more prominent (P<0.05). CONCLUSION: This is the first study of child-onset EPC with a large series in a pediatric epilepsy center in China. The most common cause for EPC was immune-related disease. The EEG background activity and the EEG/EMG correspondence might be influenced by the etiologies of EPC to some degree. These findings might guide the direction of EPC diagnosis in conjunction with other examinations.

Epilepsia partialis continua triggered by traumatic hand injury: a peripheral tuning of brain excitability?

Epilepsia partialis continua is often refractory to antiepileptic medication and its causal relation to peripheral sensory stimuli has only rarely been suggested. We report a man who received surgery for temporal lobe epilepsy 10 years ago, who presented "de novo" epilepsia partialis continua following mild traumatic injury of the left hand. Continuous myoclonus of the left upper limb started the day after injury and persisted unabated for several weeks. Non-invasive evaluation was inconclusive. Acute electrocorticography during surgery under local anaesthesia revealed continuous, rhythmic spiking over the right sensorimotor cortex. Tailored excision of the posterior bank of the motor and adjacent sensory cortex immediately stopped the continuous myoclonus. Histopathology showed abnormal radial lamination and was compatible with focal cortical dysplasia type IA. Epilepsia partialis continua did not recur for seven years. Afferent stimuli from peripheral injury can disinhibit hyperexcitable sensorimotor cortex leading to epilepsia partialis continua. [Published with video sequences online].

Seizures in an immunocompromised adolescent: a case report.

INTRODUCTION: Tuberculosis is a progressive and disabling infection predominantly seen in low-income and middle-income countries. Immunocompromised patients are at a higher risk of contracting tuberculosis than the healthy population. The presentation may also be atypical, leading to delay in diagnosis. We report the first case of tuberculous cerebral vasculitis presenting with epilepsia partialis continua. CASE PRESENTATION: A 17-year-old adolescent boy of Sri Lankan Moor heritage was taking long-term immunosuppressants for nephrotic syndrome. He presented to hospital with focal fits affecting his left arm. He later developed choreiform movements of the same arm, progressing to epilepsia partialis continua and weakness. The gradually evolving focal neurological signs and underlying immunosuppression raised the possibility of localized cerebral infection or inflammation. Analysis of his cerebrospinal fluid showed lymphocytosis with normal cellular morphology. Magnetic resonance imaging was suggestive of progressive vasculitic infarctions of the cerebral cortex and basal ganglia. There was no evidence of active autoimmune or viral disease on hematological investigations, but molecular amplification detected Mycobacterium tuberculosis in his cerebrospinal fluid. Although our patient had been established on isoniazid preventive treatment for eight months before the episode, tuberculosis was nonetheless considered to be the most likely cause of the cerebral vasculitis. He was treated with a trial of anti-tuberculosis treatment, including streptomycin and adjunctive steroids, and made an uneventful recovery. CONCLUSION: Clinicians should have a high index of suspicion for tuberculosis infection in patients with compromised immunity and other risk factors. The pathophysiological mechanisms underpinning cerebral vasculitis and epilepsia partialis continua are not completely understood. The efficacy of isoniazid prophylaxis in patients with immune suppression warrants further study. We present a regimen that successfully treated tuberculous cerebral vasculitis.

Epilepsia partialis continua of the abdominal muscles due to cerebrovascular disease.

Two elderly men, with previous history of cerebrovascular disease, were admitted to the emergency department due to focal motor status epilepticus with persistent myoclonic jerks of one side of the body. In both cases, the clinical picture evolved into a unilateral and isolated arrhythmic myoclonus of the abdominal muscles with preserved consciousness. These involuntary movements resolved with antiepileptic drugs. Although cerebrovascular disease is one of the most common causes of epilepsia partialis continua, reported cases in the literature with predominant abdominal involvement have a different aetiology. The neuroimaging and electroencephalographic findings showed a wide spectrum of different localizations and aetiologies associated with this particular type of epileptic seizure. Indeed, the pathophysiology of focal motor seizures involving the abdominal muscles is still a matter of discussion. In our second case, we present a patient with epilepsia partialis continua of the abdominal wall with an occipital focus, which, to the best of our knowledge, has not been previously reported. [Published with video sequences].

[Non-convulsive status epilepticus with a phenomenon of transitory amnesia].

This article describes clinical cases of 13 year old boy with two non-convulsive status epilepticus which had transient epileptic amnesia as a clinical implication. Status EEG pattern in form of diffuse epileptic activity "benign epileptiform discharge of childhood" type was registered.

Alcohol-responsive epilepsia partialis continua.

Epilepsia partialis continua is typically associated with lesions of the cerebral cortex. However, subcortical lesions can also cause this condition. We present a patient with epilepsia partialis continua who failed to respond to conventional anticonvulsant medications but experienced a dramatic transient response to alcohol and a subsequent response to primidone. This pattern of sensitivity, which is similar to that seen in essential tremor, has led to the hypothesis that the two disorders are associated with pathology within the same anatomical network. A new pathophysiological model is thus proposed for the occurrence of epilepsia partialis continua in both cortical and subcortical disease processes.

Efficacy of lacosamide on seizures and myoclonus in a patient with epilepsia partialis continua.

A 39-year-old male with epilepsia partialis continua, refractory to first- and second-line antiepileptic drugs, is described. Lacosamide produced a progressive antiepileptic effect on Jacksonian motor seizures and subsequently on positive myoclonus, which developed into negative myoclonus before complete resolution. Our report confirms the efficacy of lacosamide on focal motor refractory status epilepticus and documents a unique effect of lacosamide on seizure semiology.

[Experience with levetiracetam in the treatment of status epilepticus]. / Erfahrungen mit Levetiracetam in der Behandlung des Status epilepticus.

Non-convulsive status epilepticus and epilepsia partialis continua are common epileptic conditions for which straightforward recommendations based on controlled randomised trials for treatment of therapy refractory courses are lacking. Therefore in these conditions sometimes antiepileptic drugs that are not approved by governmental authorities for the treatment of status epilepticus (SE) are used. Here we review all case reports and case series concerning the treatment of SE with levetiracetam (LEV), that had been listed in pub-med up to December 12th 2011. Additionally we analysed abstracts and papers in peer reviewed journals, that were listed in the references of the primarily reviewed papers. Furthermore we looked for LEV treatments in papers on the use of lacosamide (LCM) in SE. LEV was given in dosages ranging from 500 mg to 9000 mg per day. Side effects were especially sedation and irritability. Estimated on the basis of the case series the overall success-rate of LEV in terminating status epilepticus may be set in a range between 53.7% and 58.1%. Therefore LEV may be a useful alternative for the treatment of SE when the approved drugs are contraindicated or when these drugs have been taken without success.