Complex Partial Status Epilepticus Research Articles

Nonconvulsive status epilepticus: Epilepsy Research Foundation Workshop Reports

In April 2004, a group of physicians with an interest in nonconvulsive status epilepticus representing a spectrum of opinion met in Oxford, sponsored by the Epilepsy Research Foundation (a charitable organization), to discuss and debate the definition, diagnosis and treatment of nonconvulsive status epilepticus. We felt that such a meeting would be useful, as nonconvulsive status epilepticus is a subject that provokes strong reactions, perhaps largely due to the relative lack of evidence and the surfeit of opinion. The meeting was arranged such that there were formal talks followed by a discussion led by one of the attendees. We present here the extended abstracts of the main talks with the points raised by the discussants. Despite disagreements on certain issues there was much in the way of consensus. First, it was agreed that nonconvulsive status epilepticus is a term that covers a range of disparate conditions with varying prognoses and treatments. The agreed definition was thus suitably vague, «Nonconvulsive status epilepticus is a term used to denote a range of conditions in which electrographic seizure activity is prolonged and results in nonconvulsive clinical symptoms». Secondly, it was agreed that even within a specific condition ( e.g. complex partial status epilepticus), the prognosis and treatment depends upon the context in which the condition occurs ( e.g. in the critically ill, in coma, in the «walking wounded» and in people with prior epilepsy). Perhaps, most importantly it was agreed that we lacked good clinical data, and the challenge was to design good studies for a condition that is underrecognised and often difficult to diagnose.

A case of systemic lupus erythematosus with complex partial status epilepticus

We describe a woman with systemic lupus erythematosus who had complex partial status epilepticus as the sole epileptic manifestation. The patient presented with a confusional state, initially considered to be psychotic in nature, due to SLE. However, the EEG demonstrated the epileptic etiology of her symptoms. In patients with lupus presenting with prolonged episodes of confusion and behavioural disturbance, an EEG should be performed, whenever possible during the episode, even in the absence of a previous history of epilepsy.

Diffusion-weighted and perfusion MRI demonstrates parenchymal changes in complex partial status epilepticus

Diffusion-weighted MRI (DWI) and perfusion MRI (PI) have been mainly applied in acute stroke, but may provide information in the peri-ictal phase in epilepsy patients. Both transient reductions of brain water diffusion, namely a low apparent diffusion coefficient (ADC), and signs of hyperperfusion have been reported in experimental and human epilepsy case studies. We studied 10 patients with complex partial status epilepticus (CPSE) with serial MRI including DWI and PI. All patients showed regional hyperintensity on DWI, and a reduction of the ADC in (i) the hippocampal formation and the pulvinar region of the thalamus (six out of 10 patients), (ii) the pulvinar and cortical regions (two out of 10), (iii) the hippocampal formation only (one out of 10), and (iv) the hippocampal formation, the pulvinar and the cortex (one out of 10). In all patients a close spatial correlation of focal hyperperfusion with areas of ADC/DWI change was present. In two patients hyperperfusion was confirmed in additional SPECT (single photon emission computed tomography) studies. All patients received follow-up MRI examinations showing partial or complete resolution of diffusion and perfusion abnormalities depending on the length of the follow-up interval. The clinical course, EEG and SPECT results all indicate that MRI detected changes related to prolonged epileptic activity. Combined PI and DWI can visualize haemodynamic and tissue changes after CPSE in the hippocampus, thalamus and affected cortical regions.

0158 Multimodal MR imaging during complex-partial status epilepticus

0158 Multimodal MR imaging during complex-partial status epilepticus

P4-148 Genetic analyses of a family with frontotemporal dementia

The characteristic features of Kluver–Bucy syndrome include hypersexuality, hyperorality, placidity, hypermetamorphosis, visual agnosia, changes in dietary habits, and memory impairment. Human cases have been reported with herpes simplex encephalitis, head injury, Pick’s disease, transtentorial herniation, adrenoleukodystrophy, and Reye’s syndrome, all involving bilateral temporal lobe pathology. We present the case of a patient with no evidence of a structural lesion in the temporal lobes and behavioral changes consistent with Kluver–Bucy syndrome following complex partial status epilepticus.

The development of hippocampal sclerosis in a patient with occipital lobe epilepsy and migraine

We examined a 36-year-old right-handed woman. Her mother’s pregnancy and the patient’s birth and early development were normal. At the age of 4 years, she had simple febrile convulsions. Her mother, who had migraine, had an isolated, unprovoked epileptic seizure at the time of her menarche, while watching television. The first afebrile seizure of our patient was at the age of 12 years, also at her menarche, while watching television. This complex partial status epilepticus (CPSE) started with visual hallucinations and illusions characterized by palinopsia, polyopia, tunnel vision, and flashing lights, and lasted for more than an hour. She was treated with valproate for 6 months. Since that time she experienced monthly left-sided migraine headaches. In around 20% of migraine headaches she experienced an aura characterized by flashing lights, which were similar to those of her seizures. She remained seizure free until 1995, at the age of 28 years. Interictal EEG remained normal. She then had a second episode of occipital lobe CPSE that lasted for 1 hour and ended with a secondarily generalized tonic-clonic seizure. The first MRI …

Kinetics of Glutamate and .GAMMA.-Aminobutyric Acid in Cerebrospinal Fluid in a Canine Model of Complex Partial Status Epilepticus induced by Kainic Acid

An imbalance of excitatory and inhibitory transmitters in the brain has been suggested to cause epileptic seizures. In this study, we investigated the kinetics of glutamate (GLU) and gamma-aminobutyric acid (GABA) in cerebrospinal fluid (CSF-GLU and CSF-GABA, respectively) using a high performance liquid chromatography (HPLC) in a canine model of complex partial status epilepticus (CPSE) induced by the microinjection of kainic acid (KA) into the unilateral amygdala. During the acute phase (3, 6, 12 and 48 hr after the onset of CPSE), CSF-GLU was significantly increased, while CSF-GABA was decreased, although not significantly. In the chronic phase, both CSF-GLU and CSF-GABA were significantly lower than normal at 72 hr after the onset of CPSE, and their levels returned to normal at 2 months. Results of the present study demonstrate that CSF-GLU is gradually increased in relation with seizure severity, and suggested the possibility that CSF-GABA was consistently decreased during CPSE induced by KA in dogs.

Topiramate and status epilepticus: report of three cases

Objective. The goal of this study was to report the effectiveness of topiramate in treating status epilepticus. Methods. Three patients with status epilepticus were treated with topiramate 500 mg twice daily for 2–5 days, with the dose gradually tapered thereafter to 200 mg twice daily. The patients’ clinical status and EEG recordings were followed. Results. Patient 1 was admitted for subacute encephalopathy that was complicated by secondarily generalized status epilepticus resistant to lorazepam, fosphenytoin, and pentobarbital coma. Topiramate was added and, 2 days later, pentobarbital was tapered with no recurrence of ictal discharges as the patient improved clinically. Patient 2 had end-stage liver disease and was hospitalized for peritonitis, and his course was complicated by partial status epilepticus. Topiramate was started and, 2 days later, his mental status improved as repeat EEG showed no further ictal discharges although periodic epileptiform discharges were seen in the left centroparietal area. The patient later died from complications of variceal bleeding. Patient 3 suffered cardiopulmonary arrest, and developed postanoxic seizures, which did not respond to lorazepam, fosphenytoin, valproate, and propofol coma as continuous EEG recordings showed recurrent generalized ictal discharges. Two days after topiramate was started, propofol was tapered and discontinued and EEG showed generalized slow wave activity and no ictal discharges. The patient was discharged to another facility 12 days later. Conclusions. Topiramate was effective in treating two patients with refractory generalized status epilepticus and one with complex partial status epilepticus. It should therefore be considered as an option in these situations, especially when other medications cannot be used.

Topiramate and status epilepticus: report of three cases

Objective. The goal of this study was to report the effectiveness of topiramate in treating status epilepticus. Methods. Three patients with status epilepticus were treated with topiramate 500 mg twice daily for 2–5 days, with the dose gradually tapered thereafter to 200 mg twice daily. The patients’ clinical status and EEG recordings were followed. Results. Patient 1 was admitted for subacute encephalopathy that was complicated by secondarily generalized status epilepticus resistant to lorazepam, fosphenytoin, and pentobarbital coma. Topiramate was added and, 2 days later, pentobarbital was tapered with no recurrence of ictal discharges as the patient improved clinically. Patient 2 had end-stage liver disease and was hospitalized for peritonitis, and his course was complicated by partial status epilepticus. Topiramate was started and, 2 days later, his mental status improved as repeat EEG showed no further ictal discharges although periodic epileptiform discharges were seen in the left centroparietal area. The patient later died from complications of variceal bleeding. Patient 3 suffered cardiopulmonary arrest, and developed postanoxic seizures, which did not respond to lorazepam, fosphenytoin, valproate, and propofol coma as continuous EEG recordings showed recurrent generalized ictal discharges. Two days after topiramate was started, propofol was tapered and discontinued and EEG showed generalized slow wave activity and no ictal discharges. The patient was discharged to another facility 12 days later. Conclusions. Topiramate was effective in treating two patients with refractory generalized status epilepticus and one with complex partial status epilepticus. It should therefore be considered as an option in these situations, especially when other medications cannot be used.

MRI evidence for the involvement of basal ganglia in epileptic seizures: an hypothesis

Recent clinical and experimental studies have suggested that the basal ganglia are involved in epileptic seizures as a propagation pathway or as a remote inhibitory control circuit. The present case report may provide additional evidence from post‐ictal magnetic resonance imaging (MRI) supporting this hypothesis. A healthy 13 year‐old boy was admitted for a complex partial status epilepticus. MRI, performed one week later, revealed bilateral T2 hyperintense signals in the striata and a left temporal arachnoid cyst. Left temporal slow waves were noted on EEG recording. No obvious metabolic alterations were identified. During the next six years of follow‐up, no seizure occurred and striatal alterations progressively disappeared. The clinical characteristics of the seizures, EEG slow waves, and probably the presence of an arachnoid cyst suggest that seizures originated from the left temporal lobe. The long‐lasting MRI changes suggest that bilateral striatal alterations may have been secondary to an inflammatory process, which in turn could have disrupted a striatal inhibitory control over seizures. On the basis of these arguments, we speculate involvement of basal ganglia in epileptic seizures, as a part of a modulatory control system over seizures rather than a propagation pathway. Future reports will support or invalidate our hypothesis.

The management of refractory generalised convulsive and complex partial status epilepticus in three European countries: a survey among epileptologists and critical care neurologists

Objective: To survey the current clinical treatment of refractory status epilepticus and to identify steps in its management which may need further investigation. Methods: Epileptologists and critical care neurologists were...

Diffusion-weighted imaging in kainic acid-induced complex partial status epilepticus in dogs

Objective: To investigate diffusion-weighted imaging (DWI) in status epilepticus, a canine model of kainic acid (KA)-induced complex partial status epilepticus (CPSE) was produced. In order to validate its usefulness, MR imaging was carried out at various times following onset of CPSE followed by histopathology. Material and methods: Six normal dogs were used in this study. In each dog, a cannula was stereotactically inserted into the left amygdala. One week after surgery, all dogs were imaged at MRI. Pre-injection imaging consisted of T2 weighted (T2W) imaging, fluid attenuated inversion recovery (FLAIR), and DWI. Two weeks after surgery, five dogs received intraamygdaloid KA microinjections. One dog was used as a control. MRI was carried out at 3, 6, 12, 24 and 48 h after onset of CPSE. Animals were euthanized immediately after MRI for histopathological evaluation. The average of each apparent diffusion coefficient (ADC) in the regions of interest was calculated from each DWI. Results: At 3 and 6 h, DWI hyperintensity and low ADC were found in the injected amygdala, without any T2W and FLAIR imaging changes. At 12 and 24 h, all imaging showed hyperintensity with higher ADC in the amygdala and the hippocampus. At 48 h, all imaging techniques showed continued hyperintensity, but ADC showed a trend towards normalization. This increasing hyperintensity in DWIs were in agreement with the degree of histopathology during CPSE. Summary: This study suggests that DWI is a useful imaging method for finding the epileptic focus or for examining potential epileptic brain damage in status epilepticus.

Complex partial status epilepticus associated with anti-Hu paraneoplastic syndrome

Although seizures can be a manifestation of paraneoplastic disorders, there are few descriptions of the association between the anti-Hu paraneoplastic syndrome and epilepsia partialis continua. A new case of refractory complex partial status epilepticus in a patient with a paraneoplastic syndrome associated with a poorly differentiated mediastinal tumor that expressed Hu antigen is described clinically, pathologically, and electrographically. We discuss the presentation of focal seizures in a disease that is characterized by diffuse pathologic involvement of the brain. The progression of EEG, MRI, and clinical findings during the course of the illness is also discussed. To our knowledge, this is the first description of paraneoplastic epilepsia partialis continua associated with diffuse pathologic abnormalities.

Transient Kluver–Bucy syndrome following complex partial status epilepticus

The characteristic features of Kluver–Bucy syndrome include hypersexuality, hyperorality, placidity, hypermetamorphosis, visual agnosia, changes in dietary habits, and memory impairment. Human cases have been reported with herpes simplex encephalitis, head injury, Pick’s disease, transtentorial herniation, adrenoleukodystrophy, and Reye’s syndrome, all involving bilateral temporal lobe pathology. We present the case of a patient with no evidence of a structural lesion in the temporal lobes and behavioral changes consistent with Kluver–Bucy syndrome following complex partial status epilepticus.

Diagnosis and Treatment of Nonconvulsive Status Epilepticus in an Intensive Care Unit Setting.

Nonconvulsive status epilepticus (NCSE) in adults is a heterogeneous epileptic emergency and includes absence status (AS), complex-partial status epilepticus (CPSE), and the status epilepticus of epileptic encephalopathy (SEEE). The latter seems to be strikingly frequent among patients in intensive care units (ICU). Diagnosis of NCSE is difficult, but has to be made quickly. It relies on clinical signs and a confirmation electroencephalography (EEG). According to the different etiologies and outcomes of AS, CPSE, and SEEE, treatment has to be individually adapted, but needs to follow some basic principles--treatment should take place in the ICU and be monitored by continuous EEG. With a few exceptions, the first drug is an intravenous benzodiazepine, mainly lorazepam. Intravenous fosphenytoin or phenytoin or valproate may follow next. If some forms of NCSE are resistant to first- and second-line treatments, single or combinations of anesthetics and enteral antiepileptic drugs (AEDs) may be added. This opinion is not evidence-based, and randomized controlled prospective trials to evaluate optimal treatment of NCSE are of first priority.

Estado epiléptico no convulsivo

Non-convulsive status epilepticus (NCSE) is an epileptic condition lasting more than 30 minutes in which continuous or recurrent seizure activity on the electroencephalogram (EEG) is responsible for diverse clinical symptoms including alteration of mental state, abnormal behaviour, perception disturbances or consciousness impairment. Most authors recognize two types of NCSE depending on the ictal electroencephalographic changes: generalized (absence status epilepticus) and complex partial status epilepticus (CPSE). In addition, there is also a simple form of partial status which is likely underdiagnosed because of its frequent progression to CPSE, subtle and varied symptomatology and high proportion of normal electroencephalograms (EEG). Absence status epilepticus (ASE) may occur in different clinical situations including idiopathic generalized epilepsy (typical AS), symptomatic generalized epilepsy (atypical AS), and elderly subjects without antecedents of epilepsy on chronic treatment with psychotropic drugs (de novo AS of late onset). Furthermore, CPSE is more frequent than previously mentioned. Although initially was equated with temporal lobe status epilepticus, extratemporal cases of CPSE have been well-documented, and the electroclinical characteristics of two types (I and II) of frontal CPSE have been recently described. NCSE may occur in patients with diverse clinical diagnosis such as hypoxic-anoxic encephalopathy, cancer, drugs, autoimmune disorders, Creutzfeldt-Jakob disease, chromosomal alterations, peritoneal dialysis, infections, cerebral hamartomas or head trauma. The identification of NCSE may be particularly arduous and, therefore, a high level of suspicion is essential to obtain an early diagnosis. An urgent EEG with administration of intravenous benzodiazepines is considered as the method of choice in the diagnostic evaluation of NCSE.

Complex partial status epilepticus of extratemporal origin in a patient with systemic lupus erythematosus

The purpose of this case report is to describe the clinical, electroencephalographic and neuroimaging findings from a woman with systemic lupus erythematosus presenting with complex partial status epilepticus (CPSE) of neocortical temporo-parieto-occipital origin. The patient experienced complex visual hallucinations that initially were attributed to treatment with corticosteroids; however, an electroencephalogram (EEG) demonstrated the epileptic aetiology of her symptoms. CPSE should be considered as a possible cause of altered mental status in lupus. An urgent EEG is essential to make an accurate diagnosis.

Complex partial status epilepticus of extratemporal origin in a patient with systemic lupus erythematosus

The purpose of this case report is to describe the clinical, electroencephalographic and neuroimaging findings from a woman with systemic lupus erythematosus presenting with complex partial status epilepticus (CPSE) of neocortical temporo-parieto-occipital origin. The patient experienced complex visual hallucinations that initially were attributed to treatment with corticosteroids; however, an electroencephalogram (EEG) demonstrated the epileptic aetiology of her symptoms. CPSE should be considered as a possible cause of altered mental status in lupus. An urgent EEG is essential to make an accurate diagnosis.

Complex partial status epilepticus induced by a microinjection of kainic acid into unilateral amygdala in dogs and its brain damage

Objective: In order to investigate kainic acid (KA)-induced amygdaloid seizure and seizure-induced brain damage in dogs, and to compare these findings with that in other species, a KA-induced seizure model in dogs was produced. Material and methods: Normal beagle dogs were used. A Teflon cannula for KA injection was inserted into the left amygdala, and cortical or depth electrodes were positioned. One week after surgery, 1.5 μg of KA was microinjected into the left amygdala. EEGs and the behavior of the animals were monitored for 2 months after KA injection. In addition, neuron-specific enolase levels in the cerebrospinal fluid (CSF-NSE) were measured intermittently. At 2 months after the injection, histopathological studies were performed. Results: KA-treated dogs showed limbic seizures that started from the left amygdala within 30 min after injection. The seizures developed into complex partial status epilepticus (CPSE), and started independently from the bilateral amygdala during the CPSE. The CPSE lasted for 1–3 days, and the animals showed no spontaneous seizures during the 2-month observation period. A significant increase in CSF-NSE was observed immediately after CPSE. Histopathologically, extensive necrosis, which formed large cavity lesions, was observed around the bilateral amygdala. Summary: A microinjection of KA into unilateral amygdala in dogs induced CPSE. The seizures elicited independently from bilateral amygdala, and bilateral limbic structures suffered extensive injury. In addition, CSF-NSE was demonstrated as a useful marker of acute neuronal damage.

Drug‐induced nonconvulsive status epilepticus with low dose of tiagabine

ABSTRACT In placebo‐controlled trials, the overall incidence of nonconvulsive status epilepticus was no higher in the tiagabine‐treated group than in the placebo‐group. Case reports of nonconvulsive status epilepticus under tiagabine suggested a specific role of dose levels, since in these patients symptoms occurred mostly at 40 mg/day or higher. We report a case of complex partial status epilepticus in a patient receiving a low dose of tiagabine and review all 11 case reports of nonconvulsive status epilepticus in patients on tiagabine, with regard to daily doses. Our analysis suggests an individual risk threshold of unknown aetiology.