Epilepsia Partialis Continua Research Articles

PP13.10 – 2890: Chronic and relapsing paediatric anti-NMDAR encephalitis: Two cases and a literature review

Background Clinical presentation of anti-NMDAR encephalitis is usually by a combination of neurologic, psychiatric and autonomic dysfunction. Prompt recognition and early immunotherapy is associated with better outcomes. The relapse rate has been reported to be 12–24%. Pruss et al. described the presence of NMDAR antibodies in the course of 30% of individuals with herpes simplex encephalitis (HSE). In a paediatric sample with HSE, 7 out of 20 children (35%) relapsed and 3 out of those 7 patients were found to be NMDAR antibodies positive. Case 1 This 12-year-old boy presented with fever and confusion. Herpes simplex DNA PCR was positive on cerebrospinal fluid (CSF). He received intravenous acyclovir with excellent response and clearance of his CSF. He represented few days later with epilepsia partialis continua requiring intensive care unit (ICU) admission. Serum anti-NMDAR antibodies were positive. He was treated with steroids, plasmapharesis, rituximab and cyclophosmide. He made a slow recovery over a few weeks; however serum anti-NMDAR antibodies continue to be positive with persistent elevation of CSF protein over the following 8 months despite regular immunotherapy. Case 2 This 15-year-old girl presented initially with seizures and agitation. She subsequently developed orofacial dyskinesia with worsening encephalopathy and seizures necessitating ICU admission. Serum anti-NMDAR antibodies were positive. She was treated with steroids, plasmapharesis and rituximab with good recovery. She represented 30 months later with psychiatric symptoms. Anti-NMDAR antibodies were positive in the CSF but negative in the serum. She responded well to immunotherapy. Discussion and conclusion: In their large cohort, Titulaer et al defined a relapse as “new onset or worsening of symptoms occurring after at least 2 months of clinical improvement”. Clinical recovery can take up to 18 months, however no literature exists regarding length of seropositivity. These two cases illustrate the variable natural history of the condition.

Intravenous levetiracetam treatment in status epilepticus: A prospective study

To assess the efficacy of intravenous (IV) levetiracetam (LEV) in the treatment of status epilepticus (SE) and treatment outcomes. This study was conducted on patients, who were classified according to the clinical characteristics of their seizures, in the emergency department, neurology, and other services of our hospital. Patients were administrated IV LEV for the treatment of their SE after failing to respond to IV diazepam. We prospectively investigated 30 patients, 16 females and 14 males whose ages ranged between 17 and 90 years (55.6 ± 19.6). Fourteen patients had convulsive SE (CSE), 11 had nonconvulsive SE (NCSE), and 5 had epilepsia partialis continua (EPC). The patients were given IV LEV with dosages ranging between 1000 and 4000 mg/day. Twenty-nine of the patients continued to receive LEV orally as maintenance treatment. The most common etiologies were cerebrovascular diseases (n = 7) and brain tumors (n = 6). SE was terminated in 23 (76.6%) patients. In the 12 months that followed SE, 9 of our patients (30%) died and 4 patients could not be contacted. Fifteen patients reported having no adverse effects, whereas three had mild adverse effects. No major adverse effects or complications causing disability were observed in twelve patients who were unconscious. Treatment with IV LEV is well-tolerated and effective both in focal and generalized SE. IV LEV has the combined advantage of efficacy, safety, and ease of use, which qualifies it to be the first choice after benzodiazepines (BZD) in the treatment of SE. This is the first prospective study of IV LEV treatment in status epilepticus and has the longest follow-up period, one year.

Neurosyphilis presenting with rapidly progressive dementia and epilepsia partialis continua

Abstract Symptomatic Neurosyphilis can have varied syndromic presentations classically categorized as meningeal, meningovascular and parenchymatous syphilis. Nowadays parenchymatous Neurosyphilis rarely presents in its classical forms, tabes dorsalis and general paresis and often presents with atypical forms. There are reports by Roberts and Emsley of failure of diagnosis of Neurosyphilis due to low index of suspicion. Here we describe a case of Neurosyphilis initially presenting with altered mental status that was treated by psychiatrists for several months till he developed epilepsia partialis continua.

Epilepsia partialis continua

Epilepsia partialis continua (EPC) is a rare syndrome of continuous focal jerking of a body part, usually localized to a distal limb, occurring over hours, days or even years. Etiology is multiple and diverse as well as the outcome is also variable. Here we report two cases of EPC admitted almost in a same period in our hospital in Khulna with different etiology and outcome. The first one, an adult diabetic patient, developed status epilepticus followed by right sided epilepsia partialis continua over a period of three days after a viral illness. He responded well with treatment. The second case, a three year old girl, progressively developed epilepsia partialis continua over a period of six months with no antecedent event. Her treatment response is not satisfactory. DOI: http://dx.doi.org/10.3329/bmjk.v47i1-2.22559 Bang Med J (Khulna) 2014; 47 : 25-28

Epilepsia Partialis Continua as Presenting Manifestation of AIDS

Seizures, most commonly generalized tonic-clonic, are common in known human immune deficiency virus (HIV) sero-positive patients, and they usually have a focal lesion on brain imaging. However, it is very unusual to see a patient with no premorbid illness presenting with epilepsia partialis continua (EPC) and then being detected HIV seropositive with an Acquired Immune Deficiency Syndrome (AIDS)-defining illness. We report the case of a teenaged boy with no past significant history or known high-risk behavior who presented with recurrent focal seizures of 5 days' duration, EPC, and encephalopathy. His electroencephalogram showed periodic lateralized epileptiform discharges (PLEDS), and magnetic resonance imaging (MRI) of the brain showed abnormal signal changes in the right parieto-occipital cortex and thalamus, both as yet unreported in cytomegalovirus (CMV) encephalitis, which was diagnosed by the cerebrospinal fluid (CSF) analysis.

Lemierre’s Syndrome Presenting with Atypical Features

Lemierre's syndrome is characterized by anaerobic bacterial infection in the head and neck, causing thrombophlebitis of the jugular vein. This disease is usually associated with a history of pharyngitis. The most common pathogens are Fusobacterium species, particularly Fusobacterium necrophorum. Lemierre's syndrome is seen most commonly in teenagers and young adults. We present a case report of a 67-year-old man with an atypical clinical manifestation of an uncommon pathogen in Lemierre's syndrome with epilepsia partialis continua. J Korean Neurol Assoc 33(1):21-25, 2015

Is It a Pathogenic ATP7A Variation and Is It Menkes Disease?

This paper is a comment on “Menkes Disease Presenting with Epilepsia Partialis Continua.” While updating the LOVD (Leiden Open Variation Database) ATP7A mutation database (https://grenada.lumc.nl/LOVD2/MD/home.php) I came across a case described by Rizk and colleagues and published in your journal [1]. Rizk and colleagues report a 17-month-old girl who was referred to their hospital with continuous right hand movements which developed suddenly at age of 14 months, regression of milestones, and repetitive episodes of hypothermia. She had hypotonia and constipation. Brain magnetic resonance imaging showed among others cortical and cerebral atrophy. EEG was abnormal and she was diagnosed with epilepsia partialis continua. Magnetic resonance angiography showed relatively tortuous but patent intracranial vessels, with appearance of “hair pin” sign. She had normal hair also with light microscopy. Blood copper and ceruloplasmin levels were normal. The patient was born full term and had normal birth measures. Genetic testing revealed presence of a heterozygous variation, c.1138G>A [p.(Val380Met)], and the authors concluded the diagnosis of Menkes disease (MD) in this patient. Even though the clinical features of this female patient overlap with some of the features observed in Menkes disease patients, she lacks some of the major pathogenic criteria (such as reduced serum copper and ceruloplasmin levels, abnormal hair, hypopigmentation, and cutis laxa) (review in [2]) questioning the clinical diagnosis of Menkes disease, even though female patients may be less severely affected. Furthermore, the ATP7A variation identified is not convincing enough to establish a genetic diagnosis. Firstly, this variation is described at least once in a healthy individual (rs149523862, dbSNP database of short genetic variations, http://www.ncbi.nlm.nih.gov/SNP/), which questions its pathogenicity especially when taking into consideration that MD is a Mendelian disorder. This variation is a missense substitution in exon 4 of ATP7A proximal to but not within the 4th MBD (metal binding domain) of the ATP7A protein. Until now 70 different missense mutations in ATP7A have been described in 94 unrelated patients and none of these mutations were within the first 7 exons of ATP7A encoding the six MBDs [3]. It is generally believed that variations within these regions are more acceptable and do not necessarily lead to MD [3]. Furthermore, two commonly used and publicly available pathogenicity prediction methods, PhD-SNP (http://snps.biofold.org/phd-snp/phd-snp.html) and nsSNP analyser (http://snpanalyzer.uthsc.edu/), categorize this variation as neutral. Secondly, MD is an X-linked recessive disorder and manifesting heterozygotes are rare [4]. It is therefore extremely important to investigate the inheritance of this mutation and to perform X-inactivation studies before evaluating the clinical effect of this variation. In conclusion, the clinical diagnosis of this patient and the pathogenicity of the identified ATP7A variation should be reevaluated.

P463: Dysfunction guided neuromodulation with rTMS in severe childhood epilepsy

Background Over recent years, neuromodulation has been reported to be of value for the treatment of different conditions where the pathophysiologic background is believed to be a central nervous dysfunction. One such entity is epilepsy where positive effects have been reported in adults. In children, the experience is extremely scarce. Given that the technique is non-invasive and that there is no reason to believe that it could be harmful, it seems reasonable to try it at least in severe cases that do not respond to conventional therapy. Methods Daily one hour sessions of low frequent repetitive TMS (rTMS, 0.5 Hz) were used to treat two children with severe epilepsy. A two year, eight months old boy with Alper’s disease developed epilepsia partialis continua with rhythmic jerking of the left upper extremity, mainly the hand. Pharmacological therapy was unsuccessful and scalp EEG could not reveal the seizure focus. The hand area of the primary motor cortex was localised using navigated TMS based on the patients structural MR. This position was used for rTMS treatment. A six year, three months old girl developed a CSWS syndrome due to a cortical malformation. After tapering of corticosteroids her seizure situation worsened. Scalp EEG revealed a number of electrical seizure foci. While waiting for a planned neurosurgical treatment, rTMS was used directed by the EEG localisations. Results The treatment sessions were mainly uneventful, in the majority the children slept during the stimulations. (The boy often needed a light procedural sedation though.) During about two weeks of treatment, the number and severity of seizures reported by parents and personnel were reduced for both patients. Conclusions rTMS for the treatment of epilepsy can be performed successfully even in small children. The procedure requires a lot of resources. If tried in less severe cases than the two presented, it may be even more efficient.

Mitochondrial disorders and epilepsy

IntroductionMitochondrial respiratory chain defects (RCD) often exhibit multiorgan involvement, affecting mainly tissues with high-energy requirements such as the brain. Epilepsy is frequent during the evolution of mitochondrial disorders (30%) with different presentation in childhood and adulthood in term of type of epilepsy, of efficacy of treatment and also in term of prognosis. State of artMitochondrial disorders can begin at any age but the diseases with early onset during childhood have generally severe or fatal outcome in few years. Four age-related epileptic phenotypes could be identified in infancy: infantile spasms, refractory or recurrent status epilepticus, epilepsia partialis continua and myoclonic epilepsy. Except for infantile spasms, epilepsy is difficult to control in most cases (95%). In pediatric patients, mitochondrial epilepsy is more frequent due to mutations in nDNA-located than mtDNA-located genes and vice versa in adults. Ketogenic diet could be an interesting alternative treatment in case of recurrent status epilepticus or pharmacoresistant epilepsy. ConclusionEpileptic seizures increase the energy requirements of the metabolically already compromised neurons establishing a vicious cycle resulting in worsening energy failure and neuronal death.

An overview of neurological and neuromuscular signs in mitochondrial diseases

Mitochondrial disorders have a broad clinical spectrum and are genetically heterogeneous, involving two genomes. These disorders may be develop at any age, with isolated or multiple system involvement, and any pattern of inheritance. Neurological involvement is the most frequent, and concerns muscular, peripheral and central nervous system. Among these diverse signs, some are suggestive of mitochondrial disease, such as progressive external ophthalmoplegia, exercise intolerance, psychomotor regression, stroke-like episodes, refractory epilepsy and Epilepsia Partialis Continua. Others are less specific and mitochondrial hypothesis may be evocated because of either association of different neuromuscular signs or a multisystemic involvement. This review describes the wealth of this neurological and neuromuscular symptomatology through different syndromes reported in the literature, according to preponderant signs and to modes of inheritance, as key elements to guide genetics testing.

Long‐term outcome after limited cortical resections in two cases of adult‐onset Rasmussen encephalitis

Rasmussen encephalitis (RE) is a progressive inflammatory disorder characterized by brain hemiatrophy, unilateral focal deficits, and drug-refractory focal epilepsy. Epilepsia partialis continua (EPC) is a hallmark of the disease. Several immunomodulatory treatments may slow but not halt the disease progression. The treatment of choice still relies on surgical hemispheric disconnection, which is burdened by heavy neurologic morbidity. More limited cortical resections, although more tolerable, are usually considered to be, at best, only transiently effective in RE. Hemispheric disconnections may be not feasible when neurologic functions are preserved and the dominant hemisphere is affected. Adult patients with a milder RE course that preserves neurologic function for a long period are particularly at risk of developing severe deficits after surgery. In this study we present the histories of two patients with adult-onset RE who have undergone selective cortical resections to control EPC, avoiding, at the same time, the severe postsurgical deficits that may be induced by hemispheric disconnective surgery. The good result obtained on EPC has been stable over a prolonged period; however, this result was not paralleled by the stop of neurologic progression in one of the two cases. A PowerPoint slide summarizing this article is available for download in the Supporting Information section http://dx.doi.org/10.1111/epi.12596/supinfo.

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.

Encephalitis with refractory seizures, status epilepticus, and antibodies to the GABAA receptor: a case series, characterisation of the antigen, and analysis of the effects of antibodies

Increasing evidence suggests that seizures and status epilepticus can be immune-mediated. We aimed to describe the clinical features of a new epileptic disorder, and to establish the target antigen and the effects of patients' antibodies on neuronal cultures. In this observational study, we selected serum and CSF samples for antigen characterisation from 140 patients with encephalitis, seizures or status epilepticus, and antibodies to unknown neuropil antigens. The samples were obtained from worldwide referrals of patients with disorders suspected to be autoimmune between April 28, 2006, and April 25, 2013. We used samples from 75 healthy individuals and 416 patients with a range of neurological diseases as controls. We assessed the samples using immunoprecipitation, mass spectrometry, cell-based assay, and analysis of antibody effects in cultured rat hippocampal neurons with confocal microscopy. Neuronal cell-membrane immunoprecipitation with serum of two index patients revealed GABAA receptor sequences. Cell-based assay with HEK293 expressing α1/β3 subunits of the GABAA receptor showed high titre serum antibodies (>1:160) and CSF antibodies in six patients. All six patients (age 3-63 years, median 22 years; five male patients) developed refractory status epilepticus or epilepsia partialis continua along with extensive cortical-subcortical MRI abnormalities; four patients needed pharmacologically induced coma. 12 of 416 control patients with other diseases, but none of the healthy controls, had low-titre GABAA receptor antibodies detectable in only serum samples, five of them also had GAD-65 antibodies. These 12 patients (age 2-74 years, median 26.5 years; seven male patients) developed a broader spectrum of symptoms probably indicative of coexisting autoimmune disorders: six had encephalitis with seizures (one with status epilepticus needing pharmacologically induced coma; one with epilepsia partialis continua), four had stiff-person syndrome (one with seizures and limbic involvement), and two had opsoclonus-myoclonus. Overall, 12 of 15 patients for whom treatment and outcome were assessable had full (three patients) or partial (nine patients) response to immunotherapy or symptomatic treatment, and three died. Patients' antibodies caused a selective reduction of GABAA receptor clusters at synapses, but not along dendrites, without altering NMDA receptors and gephyrin (a protein that anchors the GABAA receptor). High titres of serum and CSF GABAA receptor antibodies are associated with a severe form of encephalitis with seizures, refractory status epilepticus, or both. The antibodies cause a selective reduction of synaptic GABAA receptors. The disorder often occurs with GABAergic and other coexisting autoimmune disorders and is potentially treatable. The National Institutes of Health, the McKnight Neuroscience of Brain Disorders, the Fondo de Investigaciones Sanitarias, Fundació la Marató de TV3, the Netherlands Organisation for Scientific Research (Veni-incentive), the Dutch Epilepsy Foundation.

Childhood-onset nonprogressive chronic encephalitis

PurposeThe purpose of this study was to describe a series of patients with pathologically proven chronic encephalitis who had a nonprogressive course during a long follow-up, suggestive of a “benign” variant of Rasmussen's encephalitis (RE). MethodsFour patients who were referred to our Comprehensive Epilepsy Program at London Health Science Centre in London, Ontario, were diagnosed with chronic encephalitis on a pathological basis after epilepsy surgery to treat their partial-onset seizures. ResultsNone of our four cases followed the typical course of RE despite their childhood-onset seizures between ages 2 and 12years. One was preceded by a mild head trauma and fever at onset. None had epilepsia partialis continua (EPC). Their long-term follow-up revealed a nonprogressive form of the syndrome with respect to the neurological examination, EEG, MRI, and neuropsychological findings. ConclusionThese cases extend the spectrum of childhood-onset intractable epilepsy with chronic encephalitis to include nonprogressive variants of RE. The absence of EPC may be a prognostic indicator of a nonprogressive course.

Epilepsia Partialis Continua and Generalized Nonconvulsive Status Epilepticus during the Course of Argininemia: A Report on Two Cases

Argininemia is a rare inherited disorder of the urea cycle because of a deficiency of the enzyme arginase I causing an increase of arginine and guanidino compounds in the blood, urine, and cerebrospinal fluid. The clinical picture is characterized by a mild cognitive dysfunction, progressive asymmetrical paraparesis, and seizures. Here, we describe two cases of argininemia where either epilepsia partialis continua (EPC) or nonconvulsive status epilepticus (NCSE) were the presenting manifestations of epilepsy. This is the first report of EPC in an urea cycle disorder. In both the cases, status epilepticus resolved with anticonvulsive drugs. EPC was successfully treated with levetiracetam, and NCSE with valproic acid. No side effects were observed. Because hyperammonemia and NCSE may have the same features of stupor, a neurophysiological approach might prove useful in differentiating these two conditions. Overall, our results strongly indicate that a correct NCSE diagnosis is mandatory to prevent further deterioration in these patients.

Vagus nerve stimulation for drug-resistant Epilepsia Partialis Continua: Report of four cases

Vagus nerve stimulation (VNS) represents an adjunctive surgical option for adult and pediatric patients with drug-resistant epilepsy, who are not eligible for surgical resection or disconnection. However, little is known on its efficacy in the treatment of Epilepsia Partialis Continua (EPC), a rare but serious form of motor status epilepticus associated either with progressive or with non-evolving neurological diseases. To evaluate the effect of VNS in a series of four children affected by medically unresponsive EPC secondary to chronic inflammatory encephalopathy (two cases), Rasmussen encephalitis (one case) and poliodystrophy (one case). After VNS implantation, the stimulation amplitude was progressively increased and, after a mean interval of 47 days, a partial reduction of EPC and associated focal seizures was observed in all patients. After a mean follow-up of three years, one child stopped EPC, two presented short and rare episodes and in one patient 2-3 residual seizures per day was reported. In all cases, reduction of epileptic activity was associated with mild improvement of motor and cognitive abilities. No serious side effects were reported. VNS may be considered as an option for EPC when medical treatment fails and other more invasive neurosurgical options are not feasible.

Isolated Aphasic Status Epilepticus as Initial Presentation of Nonketotic Hyperglycemia

Diagnosis of aphasic status epilepticus is sometimes not easy because of its rarity and electroclinical dissociation. Although most cases are associated with organic brain lesions, nonketotic hyperglycemia (NKH)-related aphasic status epilepticus is rare, especially if it is isolated (without other clinical seizure activity). On the other hand, unlike other metabolic disorders, or hypoglycemia-related generalized seizures, focal motor seizure and epilepsia partialis continua can occur in 25% of NKH, with seizures being the initial manifestation in up to 50% of patients. However, the presentation of epileptic aphasia is rare in NKH patients. We report a rare case of NKH presenting initially as persistent and isolated aphasic status epilepticus. Brain magnetic resonance imaging did not reveal any focal lesion, but ictal electroencephalography (EEG) disclosed left frontotemporal continuous theta to delta waves, intermingled with epileptiform discharges. Correcting the hyperglycemia failed to improve the language disorder, and the seizure was controlled only by the addition of carbamazepine. Patients with NKH may initially present with isolated aphasic status epilepticus. Unlike stroke-related aphasia, accurate diagnosis is difficult if based solely on neurologic examination and brain neuroimaging. Use of EEG and blood sugar determination should be helpful in this special condition.

Gene therapy in status epilepticus

Gene therapy in human disease has expanded rapidly in recent years with the development of safer and more effective viral vectors, and presents a novel approach to the treatment of epilepsy. Studies in animals models have demonstrated that overexpression of inhibitory peptides can modify seizure threshold, prevent the development of epilepsy, and modify established epilepsy. More recently there has been a flurry of studies using optogenetics in which light-activated channels expressed in neurons can transiently change neuronal excitability on exposure to light, thereby enabling the development of closed loop systems to detect and stop seizure activity. The treatment of status epilepticus presents its own challenges. Because of both the delay in gene expression following transfection and also the necessity of using focal transfection, there are a limited number of situations in which gene therapy can be used in status epilepticus. One such condition is epilepsia partialis continua (EPC). We have used gene therapy in a model of EPC and have shown that we can "cure" the condition. Recent evidence suggesting that gene therapy targeting subcortical regions can modify generalized or more diffuse epilepsies, indicates that the range of situations in status epilepticus in which gene therapy could be used will expand.

Presentation, diagnosis and treatment of bilateral Rasmussen's encephalitis in a 12‐year‐old female

To describe the clinical course and pathological diagnosis of a 12-year-old female who presented with an acute syndrome of right hemispheric epilepsy and cortical dysfunction and brain MRI demonstrating atrophy of the left cerebral and right cerebellar hemispheres. The patient presented with occasional partial seizures consisting of a left calf sensation followed by left leg clonic jerking. Initial brain MRI showed left cerebral and right cerebellar atrophy with T2 hyperintensity in the left parietal region. After six months, the seizure frequency increased and semiology evolved to include frequent clonic movements of the left side of the face, arm and leg and epilepsia partialis continua (EPC) of the left arm and leg. There was progressive weakness of the left leg and, to a lesser extent, her left arm. MRI at this time demonstrated an additional T2 hyperintensity in the right frontal lobe. An extensive evaluation for paraneoplastic, mitochondrial, and genetic epilepsy syndromes was unrevealing. On biopsy evaluation, chronic T-cell mediated encephalitis was demonstrated within bilateral frontal lobes. Treatment with immunomodulatory therapy resulted in some improvement in her seizure frequency and motor function. Rasmussen's encephalitis can be a challenging diagnosis. The patient's clinical history, including EPC, with bilateral frontal lobe biopsies confirming a T-cell mediated encephalitis supports a diagnosis of bilateral Rasmussen encephalitis. This case highlights the diagnostic challenges and treatment dilemmas that arise in an adolescent presenting with bilateral inflammatory lesions of Rasmussen's encephalitis. [Published with video sequences].

Treatment and course of different subtypes of status epilepticus

Since randomized controlled trials are difficult to perform for ethical reasons in a potentially deadly condition like status epilepticus (SE), a retrospective database analysis may be welcome to broaden the evidence for the treatment of SE. In this retrospective study we evaluated every SE treatment at the neurological department of the University of Rostock from January 2000 to December 2009 in order to determine the efficacy of different antiepileptic drugs (AEDs) in terminating different kinds of SE. We analyzed the frequency of refractory courses in different types of SE, at which time which AED was administered and at which time which AED was effective to terminate the different epileptic conditions. A second aim of this study was to evaluate the course and the outcome of different kinds of SE. Statistical comparisons were performed with the χ(2)-test. 167 episodes of SE in 118 patients could be evaluated. The efficacy rates of AEDs differed significantly, mainly due to the superior efficacy of clonazepam (CZP). CZP seemed to be more effective than DZP, LEV, MDM and VPA in terminating generalized convulsive status epilepticus (GCSE), whereas there was no significant difference in the efficacy for terminating nonconvulsive status epilepticus (NCSE) and epilepsia partialis continua (EPC) between the used AEDs. Anaesthesia and CZP both terminated GCSE more effectively than NCSE and EPC. Concerning the course of the different kinds of SE the following results were obtained: 13 patients died during hospital treatment. Treatment in NCSE and EPC started significantly later than in GCSE. There was no significant difference in mortality between the types of SE. However the frequency of refractory courses differed between the types of SE. At the time of SE termination without the administration of anaesthesia a combination therapy using 2 or more AEDs was established in most episodes.