The role of EEG and neuroimaging in the diagnosis of non-convulsive status epilepticus in Subacute Encephalopathy with Seizures in Alcoholics (SESA syndrome): a case report and overview of the literature.

Epileptic seizures are frequently associated with liver dysfunction and alcoholism. Subacute encephalopathy with seizures in chronic alcoholics (SESA) is an underrecognized condition with peculiar clinical, EEG and neuroradiological features.We report the case of a 58-year-old man with previous alcohol use disorder (AUD) and acute-on chronic liver failure on alcohol-related cirrhosis, referred for urgent Orthotopic Liver Transplantation evaluation. The patient presented with delirium, aphasia and progressive deterioration of consciousness leading to intensive care unit admission. EEG showed slow activity with superimposed lateralized periodic discharges (LPDs) over the left temporo-occipital regions and ictal discharges with focal motor phenomena, consistent with focal status epilepticus. Antiseizure treatment with lacosamide and levetiracetam was administered with progressive improvement of consciousness.Brain MRI disclosed T2/FLAIR areas of hyperintensity in the left pulvinar and T2/FLAIR hyperintensity with corresponding DWI hyperintensity in the left hippocampal cortex, suggestive of post/peri-ictal excitotoxic changes with anatomical correspondence to focal LPDs distribution. SWI demonstrated decreased prominence of cortical veins in the left temporo-occipital region consistent with increased venous blood oxygenation in compensatory hyperperfusion.In conclusion, SESA should be suspected in the differential diagnosis of patients with AUD presenting with focal neurological deficits, seizures and focal EEG abnormalities. In this context, EEG and brain MRI represent useful tools with both diagnostic and prognostic value.

Why we prefer levetiracetam over phenytoin for treatment of status epilepticus.

Over last fifty years, intravenous (iv) phenytoin (PHT) loading dose has been the treatment of choice for patients with benzodiazepine-resistant convulsive status epilepticus and several guidelines recommended this treatment regimen with simultaneous iv diazepam. Clinical studies have never shown a better efficacy of PHT over other antiepileptic drugs. In addition, iv PHT loading dose is a complex and time-consuming procedure which may expose patients to several risks, such as local cutaneous reactions (purple glove syndrome), severe hypotension and cardiac arrhythmias up to ventricular fibrillation and death, and increased risk of severe allergic reactions. A further disadvantage of PHT is that it is a strong enzymatic inducer and it may make ineffective several drugs that need to be used simultaneously with antiepileptic treatment. In patients with a benzodiazepine-resistant status epilepticus, we suggest iv administration of levetiracetam as soon as possible. If levetiracetam would be ineffective, a further antiepileptic drug among those currently available for iv use (valproate, lacosamide, or phenytoin) can be added before starting third line treatment.

Cerebral hemiatrophy and hemiparesis following hemiclonic status epilepticus in Dravet syndrome.

Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome. This sequence of events has been previously published in patients with Dravet syndrome and does not contradict the recent classification by the International League Against Epilepsy (ILAE). The ILAE guidance identifies "Focal neurological findings" as alert criteria and "MRI showing a causal focal lesion" as exclusionary criteria for making an initial diagnosis of Dravet syndrome at presentation. Our three patients would correspond to a severe phenotype, similar to the well-known presentation of generalized atrophy following prolonged status epilepticus. Common genetic findings in cases of diffuse and unilateral brain involvement may help explain these clinical presentations. Further genotype-phenotype studies may provide additional insights into this electroclinical behavior.

Responsive neurostimulation as a treatment for super-refractory focal status epilepticus: a systematic review and case series.

OBJECTIVE: Super-refractory status epilepticus (SRSE) has high rates of morbidity and mortality. Few published studies have investigated neurostimulation treatment options in the setting of SRSE. This systematic literature review and series of 10 cases investigated the safety and efficacy of implanting and activating the responsive neurostimulation (RNS) system acutely during SRSE and discusses the rationale for lead placement and selection of stimulation parameters. METHODS: Through a literature search (of databases and American Epilepsy Society abstracts that were last searched on March 1, 2023) and direct contact with the manufacturer of the RNS system, 10 total cases were identified that utilized RNS acutely during SE (9 SRSE cases and 1 case of refractory SE [RSE]). Nine centers obtained IRB approval for retrospective chart review and completed data collection forms. A tenth case had published data from a case report that were referenced in this study. Data from the collection forms and the published case report were compiled in Excel. RESULTS: All 10 cases presented with focal SE: 9 with SRSE and 1 with RSE. Etiology varied from known lesion (focal cortical dysplasia in 7 cases and recurrent meningioma in 1) to unknown (2 cases, with 1 presenting with new-onset refractory focal SE [NORSE]). Seven of 10 cases exited SRSE after RNS placement and activation, with a time frame ranging from 1 to 27 days. Two patients died of complications due to ongoing SRSE. Another patient's SE never resolved but was subclinical. One of 10 cases had a device-related significant adverse event (trace hemorrhage), which did not require intervention. There was 1 reported recurrence of SE after discharge among the cases in which SRSE resolved up to the defined endpoint. CONCLUSIONS: This case series offers preliminary evidence that RNS is a safe and potentially effective treatment option for SRSE in patients with 1-2 well-defined seizure-onset zone(s) who meet the eligibility criteria for RNS. The unique features of RNS offer multiple benefits in the SRSE setting, including real-time electrocorticography to supplement scalp EEG for monitoring SRSE progress and response to treatment, as well as numerous stimulation options. Further research is indicated to investigate the optimal stimulation settings in this unique clinical scenario.

Focal status epilepticus may trigger relapse of primary angiitis of the CNS.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS. Ictal-induced blood-brain barrier dysfunction allows the entry of water and inflammatory molecules that, in the context of CNS inflammatory diseases, may trigger a self-reinforcing process. Caution should be observed when tapering antiepileptic drugs in patients with such conditions.

Two cases of focal status epilepticus in pregnancy.

The management of women with epilepsy (WWE) presents many challenges for physicians. The primary goal during pregnancy is to achieve the best possible control of seizures with the least adverse effects associated with exposure to antiseizure medications (ASMs). Even though the guidelines for managing pregnant WWE are expanding, no definitive guidelines exist for the treatment of status epilepticus (SE). Additionally, much of our data comes from the effect of generalized tonic clonic seizures on the fetus. There is very little data on the effect of focal seizures and even less on focal SE. Here we present two cases of pregnant WWE who presented in focal SE, who underwent simultaneous video-EEG monitoring and fetal heart tracing (FHT). During each focal seizure the FHT demonstrated a normal baseline heart rate with moderate variability. In the second case due to continuous seizures more aggressive treatment had to be started. This led to maternal relative autonomic instability as well as absent variability on FHT. These findings raise many questions on the management of focal SE during pregnancy including if the effect of treatment is worse than the seizures, and how do we balance our goals for both mother and fetus?

Sudden Onset Fluent Aphasia: Stroke or Seizure?

Conventional understanding of acute onset language deficits indicates that fluent aphasias are due to perisylvian lesions in the dominant hemisphere, most often in the setting of acute stroke. Case studies and retrospective analyses, however, suggest the need to keep ictal phenomena as an alternative diagnostic possibility. The following case illustrates an epileptic mechanism of sudden onset fluent aphasia mimicking an acute stroke presentation. We utilize the case to illustrate means by which to differentiate stroke versus ictal etiology by way of electroencephalography/response to antiseizure drugs as well as perfusion/diffusion imaging. We review the literature case reports to demonstrate that isolated fluent aphasia typically localizes to left-hemispheric, temporal foci. Finally, we provide a brief synthesis of potential neurologic mechanisms by which left temporal lesions may cause fluent aphasia.

Lateralized periodic discharges in insular status epilepticus: A case report of a periodic EEG pattern associated with ictal manifestation.

OBJECTIVE: Insular lobe seizures generally represent a misconceived ictal phenomenon characterized by specific neurological signs. Aphasia can be a rare presenting sign associated with insular lobe epilepsy which could be easily mistaken for a manifestation of other acute brain diseases. METHOD: We describe an insular status epilepticus (SE) characterized by sudden onset of language disturbance associated with hypersalivation and paraesthesia. A concomitant EEG recording showed the presence of Lateralized Periodic Discharges plus superimposed fast activity (LPDs + F). After an adequate acute endovenous anti-seizure treatment, a normalization of the EEG abnormalities with a complete resolution of all the neurological symptoms was achieved. DISCUSSION: Language disturbances can be usually found in various pathological acute pictures involving the dominant frontal and temporal lobes. The presence of certain EEG pattern, could rise the suspect of aphasia as a critical manifestation. LPDs pattern is usually correlated with structural lesions. The association between LPDs and seizure is controversial but it seems to be more consistent when they are associated with "Plus modifiers" and with an high periodic frequency. CONCLUSION: Our case underlines the importance of considering focal SE in the differential diagnosis of patients presenting aphasia, even in the absence of previous history of epilepsy. We describe how LPDs can be associated with SE in a patient affected by a brain tumour, supporting the idea that some characteristic periodic patterns could be associated with seizure occurrence.

Focal Status Epilepticus-Related Unilateral Brain Edema: Magnetic Resonance Imaging Study of Children in Southwest China.

BACKGROUND: Unilateral brain edema is a rare peri-ictal imaging abnormality related to focal status epilepticus. We present the largest series of these patients, describe their clinical features and magnetic resonance imaging (MRI) findings, and analyze the possible underlying pathophysiology. METHODS: We reviewed the medical records in Southwest China's largest tertiary's children's medical center from 2011 to 2017. Patients with focal status epilepticus were included if acute-phase cerebral MRI showed unilateral edematous swelling of the epileptic hemisphere. RESULTS: Eleven children were included. The age at which the patients presented with status epilepticus ranged from seven months to 10.8 years. All patients showed prolonged clonic seizures with marked unilateral predominance followed by hemiplegia of the ipsilateral limbs. The seizure duration ranged from one to 72 hours. All patients showed hyperintensities on T2-weighted images and diffusion-weighted images involving the whole pathologic hemisphere. Three patients showed involvement of the contralateral cerebellar hemisphere and one showed hippocampal herniation. Magnetic resonance angiography of the brain was performed in seven patients, among which three showed dilation of the affected hemispheric arteries. Three patients underwent follow-up MRI, and all the examinations revealed ipsilateral cerebral hemisphere atrophy. CONCLUSIONS: Focal status epilepticus may cause unilateral brain edema, and cytotoxic edema probably plays an important role in the pathophysiology of brain injury.

Clinicopathologic Findings of CARS2 Mutation.

OBJECTIVES: We describe a 13-year-old girl with a past medical history of epilepsy, intellectual impairment, dysphagia with gastric tube dependence, and autism spectrum disorder who presented with focal status epilepticus. METHODS: Video-electroencephalography revealed left occipital pseudoperiodic epileptiform discharges and frequent seizures originating from the left hemisphere. The seizure was refractory to antiepileptic medications and pharmacologic coma. Subsequently, left occipital lobectomy was done. Extensive evaluation including whole exome sequencing, histopathologic examination of brain and muscle samples, mitochondrial DNA content analysis of tissue sample was completed to detect the etiology. RESULTS: Skeletal muscle mitochondrial DNA content (qPCR) analysis showed approximately 37% of the mean value of age and tissue matched control group consistent with a mitochondrial depletion syndrome. Microscopic examination of the brain showed cortical abnormalities that largely consisted of infarct-like pathology in a laminar manner, abnormalities of neuronal distribution, and white matter changes. Compound heterozygous mutations of the CARS2 gene were identified by whole exome sequencing; V52G variant [p.Val52Gly (GTG>GGG):c.155 T>G in exon 1] was inherited from the mother and T188M variant[p.Thr188Met (ACG>ATG): c.563 C>T in exon 5] was inherited from the father. CONCLUSION: This is the first detailed clinicopathologic description of the Alpers-Huttenlocher syndrome phenotype from CARS mutations.

Epilepsia partialis continua: aetiology, semiology and prognosis in a Spanish adult cohort.

To describe the semiological features in patients suffering with Epilepsia Partialis Continua (EPC), also referred as Kozhevnikov syndrome and their relationship with aetiology, duration, and prognosis, as well as recurrence during follow-up. We analysed consecutive EPC patients diagnosed and followed in our centre over a seven-and-a half year period. We collected demographic and clinical data, along with neuroimaging and EEG recordings. All patients were followed for more than six months. Patients were categorised with single body area or multiple body area involvement according to the body parts affected. Recurrence was defined as a second EPC episode after one week. We collected data from 27 adult patients; 70.4% were men, the mean age was 65.2 years old (range: 17-89 years), and 40.7% had previous epilepsy. EPC causes were structural in 85.1% (stroke being the most frequent; 44.4%), metabolic in 11.1%, and of unknown origin in 7.4%. A cortical lesion on neuroimaging was shown in 70.4%. Involvement of multiple body areas was reported in 55.6% of patients. The optimal cut-off period to predict death was nine days (with a sensitivity of 62.5% and specificity of 75%; p=0.039), and this group of patients exhibited more multiple body area involvement (88.9% vs 38.9%; p=0.04). During follow-up, patients with cortical lesions had more EPC relapses (p=0.037). The most frequent aetiology of EPC in our patients was stroke. Multiple body area involvement and duration were associated with mortality. Patients with cortical lesions had more EPC relapses during follow-up.

Fatal outcome in hemiconvulsion-hemiplegia syndrome.

Hemiplegia-hemiconvulsion-epilepsy syndrome is characterized by prolonged unilateral clonic seizures in a child followed by the development of hemiplegia. Focal status epilepticus results in unilateral cerebral edema of the epileptic hemisphere in the acute phase followed by cerebral hemiatrophy. Literature in the last 5 years does not describe malignant cerebral edema or resultant death. We report a case of a 3-year-old girl with hemiplegia-hemiconvulsion-epilepsy syndrome who died due to malignant cerebral edema and temporal lobe herniation. The first indication of worsening of clinical status after being seizure free was voltage suppression on continuous electroencephalography (EEG). We describe neuroimaging, EEG findings, and neuropathologic findings at autopsy and review pertinent literature. We also evaluate the evolving role of continuous EEG monitoring in the pediatric intensive care unit.

Febrile infection-related epilepsy syndrome: a study of 12 patients.

PURPOSE: To analyze the electroclinical features, neuroimaging findings, treatment, and outcome of 12 patients with febrile infection-related epilepsy syndrome (FIRES). METHODS: This is a retrospective study of 12 children with FIRES with a mean time of follow-up of 6.5 years carried out at the Garrahan Hospital of Buenos Aires between 1997 and 2012. RESULTS: Eight males and four females had focal status epilepticus preceded by febrile infection with a mean age at presentation of 8.5 years. In the acute period, the treatment included antiepileptic drugs (AEDs) in all cases, immunotherapy in 10 cases, and burst-suppression coma in eight. The ketogenic diet was tried in two, plasmapheresis in one, and rituximab in one. Two patients treated with IVIG and one patient given steroids had a good response, but in this phase only three patients had a prolonged good response to IVIG and a ketogenic diet. No patients died in this period. In the chronic epilepsy phase, all children had seizures arising from neocortical regions. All patients had refractory epilepsy, and most mental retardation, and behavioral disturbances. All received different AEDs and in this phase a third patient was put on a ketogenic diet. One patient was operated without good results. Only two cases had a good outcome after 2 and 10 years of follow-up. CONCLUSION: FIRES is a well-defined severe epileptic syndrome, probably in the group of epileptic encephalopathies, characterized by focal or multifocal seizures arising from the neocortical regions with an unknown etiology. Immunoglobulin and the ketogenic diet may be considered a potentially efficacious treatment.

Encephalopathy with hemi-status epilepticus during sleep or hemi-continuous spikes and waves during slow sleep syndrome: a study of 21 patients.

PURPOSE: To retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 21 patients with encephalopathy with hemi-status epilepticus during sleep (ESES) or hemi-continuous spikes and waves during slow sleep (CSWSS) syndrome. METHODS: Charts of 21 patients with hemi-ESES/CSWSS syndrome followed between 1997 and 2012 were analyzed. Inclusion criteria were: (1) Focal seizures or apparently generalized seizures and focal EEG epileptiform discharges; (2) Further occurrence of atypical absences, and myoclonic, atonic, and/or generalized seizures; (3) Cognitive impairment and/or behavioral disturbances; (4) Hemi-continuous spike-and-wave discharges during slow sleep in more than 85% of non-REM sleep at onset and throughout the ESES/CSWSS period. RESULTS: Mean follow-up from onset of hemi-ESES/CSWSS was 8 years (range, 2-15 years). Idiopathic cases were not identified. Unilateral polymicrogyria was found in 11, shunted hydrocephalus in four, a porencephalic cyst associated with polymicrogyria in three, and a thalamic lesion in three children. All started with focal seizures with or without secondary generalization. During the hemi-ESES/CSWSS period, all children developed new types of seizure, such as negative and positive myoclonus, absences, motor deterioration, cognitive impairment, and behavioral disturbances. All AED responders returned to baseline cognitive development. Seven patients were refractory to AEDs. CONCLUSION: Our study suggests that the hemi-ESES/CSWSS syndrome has electroclinical features compatible with an epileptic encephalopathy. The most commonly used treatments were clobazam, ethosuximide, and sulthiame, alone or in combination. In refractory cases, high-dose corticosteroids were administered. Although the number of patients in this study is too low to draw definite conclusions, we consider that in children with hemi-ESES/CSWSS secondary to a unilateral lesion, surgery should be considered.