Partial Status Epilepticus Research Articles

Classification of Current Experimental Models of Epilepsy.

This article provides an overview of several experimental models, including in vivo, genetics, chemical, knock-in, knock-out, electrical, in vitro, and optogenetics models, that have been employed to investigate epileptogenesis. The present review introduces a novel categorization of these models, taking into account the fact that the most recent classification that gained widespread acceptance was established by Fisher in 1989. A significant number of such models have become virtually outdated. This paper specifically examines the models that have contributed to the investigation of partial seizures, generalized seizures, and status epilepticus. A description is provided of the primary features associated with the processes that produce and regulate the symptoms of various epileptogenesis models. Numerous experimental epilepsy models in animals have made substantial contributions to the investigation of particular brain regions that are capable of inducing seizures. Experimental models of epilepsy have also enabled the investigation of the therapeutic mechanisms of anti-epileptic medications. Typically, animals are selected for the development and study of experimental animal models of epilepsy based on the specific form of epilepsy being investigated. Currently, it is established that specific animal species can undergo epileptic seizures that resemble those described in humans. Nevertheless, it is crucial to acknowledge that a comprehensive assessment of all forms of human epilepsy has not been feasible. However, these experimental models, both those derived from channelopathies and others, have provided a limited comprehension of the fundamental mechanisms of this disease.

Medial Pulvinar Stimulation in Temporal Lobe Epilepsy: A Literature Review and a Hypothesis Based on Neuroanatomical Findings.

While bilateral stimulation of the anterior thalamic nuclei remains the only approved deep brain stimulation (DBS) option for focal epilepsy, two additional thalamic targets have been proposed. Earlier work indicated the potential of centromedian thalamic nucleus stimulation with recent findings highlighting the medial pulvinar nucleus. The latter has been shown to exhibit electrophysiological and imaging alterations in patients with partial status epilepticus and temporal lobe epilepsy. On this basis, recent studies have begun assessing the feasibility and efficacy of pulvinar stimulation, with encouraging results on the reduction of seizure frequency and severity. Building on existing neuroanatomical knowledge, indicating that the medial pulvinar is connected to the temporal lobe via the temporopulvinar bundle of Arnold, we hypothesize that this is one of the routes through which medial pulvinar stimulation affects temporal lobe structures. We suggest that further anatomic, imaging, and electrophysiologic studies are warranted to deepen our understanding of the subject and guide future clinical applications.

Progression or Response: New Liver Lesions in a Patient with Responding Hodgkin Lymphoma.

Significant neurotoxicity including seizures, encephalopathy and coma may complicate the use of cyclosporin A (CyA). Two patients are described, receiving CyA, who presented with abnormal behaviour, stupor, focal motor activity and were shown to be in complex partial status epilepticus (CPSE). Abnormalities of behaviour and/or stupor in patients receiving CyA may be a manifestation of CPSE. Patients receiving CyA who develop an encephalopathy should have electroencephalography performed at the time of the abnormal behaviour.

Occipital Lobe Status Epilepticus; A Rare Stroke Mimic with Novel Imaging Findings: A Case Report

Introduction This case reviews a patient who presented to the emergency department (ED) with homonymous hemianopsia, a rare manifestation of partial status epilepticus of the occipital lobe. Her initial brain computerized axial tomographic (CT) perfusion scan and magnetic resonance imaging (MRI) revealed novel findings associated with this diagnosis. Case Report A 70-year-old female presented to our ED with left visual field hemianopsia, dyskinesia, dysmetria and facial droop. Her initial diagnosis was left posterior fossa circulation cerebrovascular accident. However, her neuroimaging indicated hypervascularity of the left occipital lobe without evidence of infarct or structural lesion. A cerebral angiogram excluded arterio-venous malformation. Subsequently, an electroencephalogram showed left occipital lobe status epilepticus. Conclusion Hemianopsia is a rare presentation of partial status epilepticus mimicking stroke. Hypervascularity seen on advanced neuroimaging may have suggested this diagnosis on initial ED evaluation.

Diagnostic Procedure and Therapeutic Approaches in Nonconvulsive Status Epilepticus (NCSE)

Nonconvulsive epileptic seizures are major type of seizures of all patients with predominantly focal epilepsy. However, this problem is still insufficiently studied, the literature presents a few data on the diagnosis and treatment of nonconvulsive status epilepticus (NCSE). NCSE is a prolonged seizure, not accompanied by severe motor presentation. Such attacks occur much more often than previously thought. NCSE is divided into focal NCSE (complex partial status epilepticus), and generalized NCSE, often referred as absence status. The absence status divides into a typical absence status (presents with suppression of consciousness of various depths, myoclonic twitching of the eyelids, impoverishment of speech production and hallucinations), atypical absence status (presents with retardation of mental processes and eyelid myoclonus) and late absence status (long-term disorientation accompanied by cognitive deficiency and presents mainly in elderly patients). According to the available sources, more than 30% of patients in the intensive care units with impaired consciousness of unclear genesis are patients with NCSE. Use of antiepileptic drugs for intravenous administration opens better opportunities in the treatment of patients with status epilepticus and NCSE.

Neurocutaneous melanosis

Neurocutaneous melanosis is a rare fatal neurological ailment characterised by cutaneous nevi with leptomeningeal melanosis.Though there is no effective treatment but able to diagnose and take care of symptoms and complications will help in the palliative care of these patients and would further help in counselling the relatives as per the prognosis of this disease. 12yr old boy rt.handed presented with left partial seizures in clusters followed with left sided weakness. He had episode of right partial status epilepticus 6 months back and 3 months back. On examination he was drowsy with power 0 in left UL & LL with increased salivation, neck stiffness. Skin examination showed giant melanocytic naevi on limbs, face, torso and back. MRI Brain showed parafalcine hypointense meninges on T2, hyperintense meninges on T1 images with nodular leptomeningeal enhancement with hydrocephalus. CSF study showed 1 lymphocyte, proteins 14mg/dl, sugar 56mg/dl. CSF cytology showed abnormal cells which were stained with May Grunwald Giemsa showing prominent nucleoli. CT chest and abdomen was normal.EEG showed generalised slowing with no epileptiform activity. His seizures were controlled with LVT, LAC, OXZ, CLZ. He was also started on diamox, mannitol for hydrocephalus. He had intermittent fever which was controlled with antibiotics. He had residual left hemiplegia and had to be fed through rhyles tube. He did continue to have intermittent seizures for which antiepileptic drugs were adjusted. Our subject had all the risk factors for NCM reported as presence of a GCMN, male sex, satellite nevi or multiple CMN, and head, neck, or posterior midline location.

Dengue Encephalitis Presenting as Complex Partial Status Epilepticus

Dengue Encephalitis Presenting as Complex Partial Status Epilepticus

Rheumatoid meningitis: A rare cause of aseptic meningitis with frequently stroke-like episodes.

Rheumatoid meningitis (RM) is a rare manifestation of rheumatoid arthritis (RA) and may present with stroke-like episodes. We describe diagnostic findings and the outcome in patients with RM. We identified 6 patients with RM in different stages of RA mostly admitted with stroke-like episodes or common features of meningitis. We used MRI, CSF, and histology for in-depth characterization. We observed RM in 2 patients without history of RA, 1 patient with early seropositive RA, and 3 patients with late-stage RA. Recurrent stroke-like episodes occurred in 5 of 6 patients; headache and partial status epilepticus was in the foreground in 1 patient. Symptoms were accompanied by constitutional symptoms in all patients. MRI showed leptomeningeal or pachymeningeal fluid-attenuated inversion recovery hyperintensities with contrast enhancement. CSF mostly showed mild pleocytosis but can initially be normal. Anticitrullinated peptide antibodies (ACPA) and rheumatoid factor (RF) were positive in all patients. Histopathology revealed granulomatous inflammation in 2 patients. Response to steroids was prompt and further immunosuppressive treatment prevented recurrence. RM is a rare manifestation of RA and often presents with stroke-like episodes. It is currently not implemented in the workup of aseptic meningitis in national guidelines. Crucial clues for diagnosis included recurrent stroke-like episodes refractory to antiepileptic treatment, headache and constitutional symptoms, meningeal enhancement on MRI, CSF pleocytosis, and positive serology findings for ACPA and RF. Prognosis is favorable with early immunosuppressive treatment.

Expanded infarction after partial status epilepticus in poststroke epilepsy

Expanded infarction after partial status epilepticus in poststroke epilepsy

Status Epilepticus in Our Patients, 15-Years Follow-Up Study

Introduction: Status epilepticus (SE) is the second most frequent neurological emergency. The purpose of this study was to analyse clinical presentation, causes and outcome of SE. Aim of the Study: The aim was to establish clinical characteristics, etiology and the outcome of status epilepticus as well as sex and age distribution in patients hospitalized at the Clinic of Neurology UCC RS in a 15-year follow-up. Patients and Methods: In this prospective 15-year study, all patients with SE admitted to the University Clinical Center of Republic of Srpska, Clinic of Neurology, were treated in the period of 15 years (2003-2017). Demographic and clinical data were collected. Results: In the aforesaid period, 124 patients with SE were treated, and there were 71 man (57%) with mean age of 59 years and 54 woman (43%), with mean age of 52.5 years. Primarily generalized tonic-clonic SE was identified in 70 (56%) and 44 (35.2%) patients, retrospectively. Simple partial SE occurred in 10 (8%) patients. 62% of the patients had previously had epilepsy while 38% had not. The main underlying causes were noncompliance to treatment in the first group (n=56; 72%) and cerebrovascular disease (n=36; 75%) in the second group. Overall mortality rate was 11.2% , which correlated with acute symptomatic etiology and patients of older age (mean: 73 years). Conclusion: Epileptic patients are at greater risk to develop SE. However, in patients with no prior history of epilepsy and acute neurological problems SE may also occur. Cerebrovascular disease was the most common cause of SE in those with the initial seizure. Noncompliance to treatment was the major cause in patients with preexisting epilepsy.

B-14. Pathophysiological diagnosis of status epilepticus with complementary use of the periictal MRI (arterial spin labeling perfusion and diffusion-weighted images) and EEG

In the diagnosis of nonconvulsive status epilepticus (NCSE), capture of ongoing ictal EEG findings is the gold standard; however, this is practically difficult without continuous EEG monitoring facilities. MRI, including diffusion-weighted imaging (DWI) and perfusion MRI with arterial spin labeling (ASL), have been applied mainly in emergency situations. Recent reports have described that ictal MRI findings, including ictal hyperperfusion on ASL and cortical hyperintensity of cytotoxic edema on DWI, can be obtained from epileptically activated cortex. We demonstrate the diagnostic value of ictal MRI findings in 15 patients diagnosed as having NCSE (eight had complex partial status epilepticus (SE) and seven subtle SE) who underwent an initial MRI and subsequent routine EEG. In all 10 patients with an epileptogenic lesion, there was a tight topographical relationship between the lesion and the localization of ictal MRI findings. In the other five patients, ictal MRI findings were useful in the diagnosis of NCSE of non-lesional elderly epilepsy, or de novo NCSE of frontal origin as situation-related NCSE. The present study clearly demonstrates that the initial use of ASL and DWI has the ability to diagnose partial NCSE and also allows documentation of the pathophysiological mechanism in each patient.

Non-Cirrhotic Hepatic Encephalopathy Presenting as a Spontaneous Intrahepatic Porto-systemic Shunt

Hepatic encephalopathy (HE) is a common manifestation of cirrhosis, which is treated medically. Cirrhotic patients who require TIPS may also develop HE. Spontaneous porto-systemic shunts are a rare occurrence. In patients without liver disease they can be either congenital or spontaneous. Many go undetected if patients are asymptomatic. We present a case of a non-cirrhotic patient with a spontaneous intrahepatic porto-systemic shunt presenting with encephalopathy. A 58-year-old female presented with acute onset encephalopathy and concern for seizures. She underwent an EEG which was consistent with complex partial status epilepticus. She had an ammonia level of 56 but ranged as high as 110 mcmol/L. Liver function tests, PT/INR were within normal limits. She had no prior history of liver disease. She was not on any medications to cause hyperammonemia. She was started on AEDs and lactulose but continued to have cognitive deficits, trouble with short-term memory, concentration and balance. An LP was performed and revealed pleocytosis. Neurology felt her presentation and symptomology was consistent with autoimmune/paraneoplastic encephalitis and she was placed on IV steroids. A paraneoplastic antibody panel was unrevealing. She was referred to Hepatology for evaluation of persistent hyperammonemia. Workup revealed a large porto-hepatic shunt on CT scan. Chronic liver disease workup was unremarkable. Symptoms persisted despite medical treatment for hyperammonemia. She was referred to Interventional Radiology for embolization of her spontaneous intrahepatic porto-systemic shunt as there was suspicion that her symptoms and presentation were due to non-cirrhotic hepatic encephalopathy secondary to the intrahepatic shunt. She underwent a successful embolization. Her ammonia levels normalized. She has had an improvement in her symptoms and has been without overt encephalopathy. She also remains seizure free. Further follow up will be needed to assess for concurrent autoimmune/paraneoplastic encephalitis versus symptoms entirely due to her porto-systemic shunt. Spontaneous intrahepatic porto-systemic shunts are rare with many patients being asymptomatic. Mildly symptomatic patients are initially managed conservatively with medications and dietary modifications, however if symptoms persist or are severe enough, they may require intervention such as IR guided embolization to redistribute blood flow within the portal system.Figure: large shunt between the right portal and hepatic veins.Figure: Portal venography demonstrating large portal vein-hepatic vein shunt with poor parenchymal opacification of the liver giving preferential flow through the shunt rather than into native intrahepatic portal veins.Figure: Following embolization there was near complete occlusion of the shunt itself with preferential flow into the intrahepatic portal veins. Following shunt embolization marked improvement in the degree of hepatic parenchymal opacification was noted.

Utility of 123I-IOMAZENIL spect for diagnosis of simple partial status epilepticus due to occipital lobe epilepsy: A report of two cases

Background: 123I-iomazenil (IMZ)-SPECT displays the distribution of the benzodiazepine receptor (BZR) in the late phase and cerebral blood flow (CBF) in the early phase.

Aphasic status epilepticus as the sole symptom of epilepsy: A case report and literature review.

Aphasia is a common symptom encountered by neurologists. However, the presence of aphasia as the sole manifestation of partial status epilepticus is rare. The present study reports a case of aphasic status epilepticus in a 27-year-old right-handed female who presented after the abrupt onset of aphasia, which had persisted for 1.5 days. The patient's medical history included head trauma followed by a craniectomy and cranioplasty. Computed tomography scans revealed a lesion in the patient's left parietal lobe, and an electroencephalogram showed a spike and slow wave pattern in the left hemisphere of the brain during aphasia. The patient's condition improved after the oral administration of oxcarbazepine daily. In the present study it was observed that EEGs were a simple method to diagnose aphasic seizures and therefore EEG recordings should be performed in all cases of unexplained aphasia. In addition, the present study reviewed previously reported cases of aphasic status epilepticus.

Clinical management of elderly patients with epilepsy; the use of lacosamide in a single center setting

IntroductionLacosamide (LCM) is a third-generation antiepileptic drug (AED) for which there is limited experience in the treatment of elderly patients with epilepsy. This study was performed to evaluate the use of LCM in this particular patient group, focusing on its tolerability and effectiveness. This is a retrospective, single-center study, in patients over 60years old treated with LCM between 1/2010 and 5/2015. Altogether, 233 elderly patients receiving LCM were identified; of these, 67 fulfilled the inclusion criteria, i.e., LCM administered for at least 2weeks. ResultsLacosamide was initiated for acute seizure disorders (prolonged complex partial seizures, recurrent seizures, or status epilepticus) in 54 patients (81%) and for chronic epilepsy in 13 patients in an outpatient setting. The mean follow-up period for LCM treatment was 14months. The mean daily dose of LCM at the end of follow-up was 368mg (range: 100–600) for those 57 patients that continued treatment. Ten patients (15%) stopped LCM treatment but none because of lack of efficacy and only three patients (4%) because of side effects. The most frequent side effects were dizziness, fatigue, and tremor. ConclusionsLacosamide was well tolerated even at relatively high doses and in combination therapy.

Posterior Reversible Encephalopathy Syndrome (PRES) in Adult Multi-Visceral Transplant patients- Cambridge University Hospital experience

Introduction: Neurological dysfunction after intestinal or multivisceral transplant (MVT) can arise due to drug toxicity, delerium due to sepsis, renal failure, stroke or central nervous system (CNS) infection. Less well known is posterior reversible encephalopathy syndrome (PRES).[1] We report here cases of PRES at a UK adult intestinal transplant centre. Methods: Data is collected prospectively on a database which was interrogated retrospectively to identify cases. Following this, case notes and radiology reports were reviewed. Results: We report three cases of PRES in our MVT cohort. Two females and one male with a median age of 42 years old. All patients were diagnosed by consistent imaging findings on magnestic resonance imaging (MRI) and exclusion of other causes. The time to diagnosis of PRES post transplant was 44 days, 9 days and 7 days (delayed in the first case as the initial MRI was normal but a further scan 7 days later showed consistent features of PRES). Patients presented with headache and confusion, partial status epilepticus and headache and visual hallucinations, resectively. All patients responded to withdrawal of tacrolimus and all were successfully rechallenged with tacrolimus at a later date. Two patients required temporary anti-hypertensive treatment. Interestingly, all patients had pre-transplant encephalopathy (2 cases of hepatic encephalopathy and one case of D-lactic encephalopathy). Conclusion: PRES was first Described by Hinchey et al in 1996.[2] There are variety of presenting symptoms including headache, visual disturbace, confusion, and hemianopia. The diagnosis of this condition in the setting of MVT can be challenging, characteristic features are best seen on T2-weighted FLAIR MRI sequences. The mechanism of PRES is unknown, however, there are proposed theories including cerebral autoregulation failure and hypoperfusion, immunologic theory through T cell/Endothelial cell interaction and circulating cytotoxic hypothesis.[3] PRES is uncommon but the prognosis of this reversable neurologic condition is good if recognised in a timley manner. The diagnosis of PRES can be challenging. Our data would suggest that there should be a particularly high level of suspicion in patients who were encephalopathic pre-transplant. Complete recovery with immunosuppression switching is possible and once there has been complete neurologica recovery, tacrolimus can be successfully reintroduced.

Possible cause of encephalitis in an immunodeficient child

A healthy boy developed gastroenteritis, meningitis, generalized vesicular rash and right leg flaccid monoparesis after 2 months of his 3rd immunization. He recovered and was able to walk in due time although atrophy and absence of DTR were seen in the right leg. Because of frequent infections – otitis and severe pneumonias requiring ventilation – immune status was checked. IgA, IgM, IgG were low. Repeated IVIG were given. In July 2015 he had measles. He didn’t develop Ab against measles virus. In October 2015 he had his first afebrile secondary GTCS which in a week involved into right sided partial status epilepticus. He was afebrile during one week. LT on the 8th day showed 6 lymphocytes and low protein. Brain MRI showed multiple cortical lesions. PCR of HSV was negative. Antiviral, antomicrobal and antifungal therapy, IVIG were started. Multiple AEDs failed to stop seizures. Repeated CT showed progression of brain damage. At third week continuous EEG monitoring showed Radermecker complexes which lead to an idea of possible subacute measles encephalitis. RT-PCR of measles virus was positive in CSF. In the course of the disease we observed evolution of clinical seizure activity and EEG: he started with right sided Epilepsia partialis continua (EPC), which then switched to the left side, afterwards subcortical spasms were seen with no cortical activity on EEG. The history of measles infection in immune deficient patient together with characteristic clinical, MRI and EEG features should require CSF examination for measles virus. Unfortunately 2 months after our patient died.

Letter re: Therapeutic coma for status epilepticus: Differing practices in a prospective multicenter study.

The article by Alvarez et al.1 compared the use of therapeutic coma (TC) for status epilepticus (SE) in tertiary care centers belonging to Harvard Affiliated Hospitals (HAH) and the Centre Hospitalier Universitaire Vaudois (CHUV). TC for SE was used more in HAH hospitals as compared to CHUV, and did not affect mortality, but resulted in increased length of stay and related costs.1 I frequently advise residents to avoid a shotgun approach (hasty use of wide-ranging, nonselective, and haphazard techniques) to SE treatment. Evidence-based standardized protocols need to be followed, but treatment must be tailored to the patient. Benzodiazepines (first-line) and nonsedating antiseizure drugs (ASD) should be dosed based on weight and at the recommended rate to rapidly achieve therapeutic CNS concentrations. A second loading dose of the initial nonsedating ASD, such as phenytoin (usually half the initial loading dose), or a second ASD (i.e., levetiracetam, valproic acid, levetiracetam, lacosamide) sometimes terminates SE. The decision to use TC in patients who are in electrographic partial SE or displaying electrographic patterns thought to be in the interictal to ictal continuum, such as periodic lateralized discharges and generalized epileptiform discharges, needs to be individualized after carefully considering the risk–benefit profile.

Simple Partial Status Epilepticus One-Day Post TMS to the Affected Hemisphere in A Participant with Chronic Stroke

Simple Partial Status Epilepticus One-Day Post TMS to the Affected Hemisphere in A Participant with Chronic Stroke

The initial use of arterial spin labeling perfusion and diffusion-weighted magnetic resonance images in the diagnosis of nonconvulsive partial status epileptics

BackgroundIn the diagnosis of nonconvulsive status epilepticus (NCSE), capture of ongoing ictal electroencephalographic (EEG) findings is the gold standard; however, this is practically difficult without continuous EEG monitoring facilities. Magnetic resonance imaging (MRI), including diffusion-weighted imaging (DWI) and perfusion MRI with arterial spin labeling (ASL), have been applied mainly in emergency situations. Recent reports have described that ictal MRI findings, including ictal hyperperfusion on ASL and cortical hyperintensity of cytotoxic edema on DWI, can be obtained from epileptically activated cortex. We demonstrate the characteristics and clinical value of ictal MRI findings. MethodsFifteen patients diagnosed as having NCSE (eight had complex partial status epilepticus (SE) and seven subtle SE) who underwent an initial MRI and subsequent EEG confrmation, participated in this study. Follow-up MRI and repeated routine EEG were performed. ResultsIn 11 patients (73%), ictal MRI findings were obtained on both DWI and ASL, while in four (27%) patients, ictal hyperperfusion was found on ASL without any DWI findings being obtained. In all 10 patients with an epileptogenic lesion, there was a tight topographical relationship between the lesion and the localization of ictal MRI findings. In the other five patients, ictal MRI findings were useful to demonstrate the pathophysiological mechanism of NCSE of non-lesional elderly epilepsy, or ‘de novo’ NCSE of frontal origin as situation-related NCSE. Ictal MRI findings are generally transient; however, in three cases they still persisted, even though ictal EEG findings had completely improved. ConclusionThe present study clearly demonstrates that the initial use of ASL and DWI could help to diagnose partial NCSE and also combined use of the MRI and EEG allows documentation of the pathophysiological mechanism in each patient.