Electroclinical Correlation Examined by Simultaneous VTR-EEG Monitoring in a Patient with Complex Partial Status Epilepticus

Abstract: We discussed the electroclinical correlation of complex partial status epilepticus (CPSE) followed up by simultaneous VTR-EEG monitoring. An eight-year-old boy had repetitive seizures lasting for 30 to 90 minutes in addition to ordinary seizures with typical oral automatism of a few minutes duration. This case fulfilled the criteria for diagnosis of CPSE by Treiman el al . (1983), and was classified as the discontinuous type of CPSE described by Gastaut et al . (1975). The clinical symptoms were characterized by a repeated occurrence of psychomotor arrest and automatic phases. The EEG showed an alternate appearance of two phases. One was characterized by 7 to 8 Hz theta or slow alpha waves and the other by 4 to 6 Hz theta waves, respectively.     

Electroclinical correlation examined by simultaneous VTR-EEG monitoring in a patient with complex partial status epilepticus

We discussed the electroclinical correlation of complex partial status epilepticus (CPSE) followed by simultaneous VTR-EEG monitoring. An eight-year-old boy had repetitive seizures lasting for 30 to 90 minutes in addition to ordinary seizures with typical oral automatism of a few minutes duration. This case fulfilled the criteria for diagnosis of CPSE by Treiman et al. (1983), and was classified as the discontinuous type of CPSE described by Gastaut et al. (1975). The clinical symptoms were characterized by a repeated occurrence of psychomotor arrest and automatic phases. The EEG showed an alternate appearance of two phases. One was characterized by 7 to 8 Hz theta or slow alpha waves and the other by 4 to 6 Hz theta waves, respectively.     

Neuron-Specific Enolase, a Marker of Acute Neuronal Injury, is Increased in Complex Partial Status Epilepticus

Summary: Purpose: To determine whether complex partial status epilepticus (CPSE) causes brain injury in humans. Serum neuron-specific enolase (s-NSE) is an accepted marker of acute brain injury, and increases in s-NSE have been correlated with the duration and outcome of generalized convulsive status epilepticus. s-NSE levels in CPSE are unknown. Increase in s-NSE in CPSE would provide new information about the degree of brain injury in CPSE and would help confirm that CPSE is a medical emergency.
Methods: This was a pilot prospective study of serial levels of s-NSE and outcome in CPSE. Eight patients with confirmed CPSE and no acute neurologic deficit were identified prospectively. Results were compared with those of normal and epileptic control groups, and outcome was assessed at hospital discharge or at 7 days with the Glasgow Oucome Scale (GOS).
Results: The mean peak s-NSE was 21.81 ng/ml, which for the 8 patients with CPSE was four times higher than that of normal controls (mean s-NSE = 5.36 SD = 1.66, p = 0.0003) and epileptic controls (mean s-NSE = 4.61 SD = 1.74, p. = 0.001).
Conclusion: The increase in s-NSE provides new evidence that CPSE causes brain injury in humans.     

Status epilepticus with confusional symptomatology]

Non-convulsive confusional status epilepticus (NCSE) is classically separated into two forms on the basis of the ictal EEG, i.e., absence status (AS) and complex partial status epilepticus (CPSE). The diagnosis is difficult on the basis of clinical semiology alone, and requires emergency EEG investigation. Absence status, or 'petit mal' status, is a polymorphic condition that can complicate many epileptic syndromes, and is the most frequently encountered form of NCSE. It is characterized by confusion of varying intensity, associated in 50% of cases with bilateral periocular myoclonias. The EEG shows ictal generalized paroximal activity; normalization is obtained after benzodiazepine injection. In AS, there is a significant nosographic heterogeneity. Four groups can be distinguished: i) typical AS occurs in the context of a generalized idiopathic epilepsy; ii) atypical AS occurs in patients with symptomatic or cryptogenic generalized epilepsies; iii) 'de novo' AS (of late onset) is characterized by toxic or metabolic precipitating factors in middle-aged subjects with no previous history of epilepsy; iv) AS with focal characteristics occurs in subjects with a pre-existing or newly diagnosed partial epilepsy, mostly of extra-temporal origin. The majority of cases are in fact transitional forms between these four groups. CPSE is characterized by continuous or rapidly recurring complex partial seizures which may involve temporal and/or extratemporal regions. Cyclic disturbance of consciousness is characteristic of CPSE of temporal lobe origin, which requires vigorous treatment to prevent recurrence or cognitive sequelae. CPSE of frontal lobe origin is a diagnostic challenge: it is rare, the symptoms are unusual, and the patients should be documented extensively. A focal frontal lesion is revealed in one-third of cases.     

Nonconvulsive Status Epilepticus: High Incidence of Complex Partial Status

Nonconvulsive status epilepticus may be subdivided into generalized (absence) status and complex partial status. The latter is regarded as a rarity, whereas the former constitutes the dominant part of the hitherto reported cases. We report 10 consecutive cases of adult patients with nonconvulsive status epilepticus, all documented by ictal electroencephalographic (EEG) recordings. Five had a complex partial status; the origin of the complex partial status appeared to be frontal in four of these patients. Three had recurrent complex partial seizures with incomplete recovery between seizures, and two had more continuous symptoms. One of the latter exhibited neither motor phenomena nor automatisms. The effect of diazepam or clonazepam was immediate in all 10 cases though transient in eight. A lasting control of the status was not achieved in six patients until i.v. phenytoin was added. The difficulties in the differentiation between complex partial status and absence status despite ictal EEG recordings are discussed, illustrated by a case with seizure discharges of a focal onset which rapidly generalized. The study indicates that complex partial status may be more common and the clinical expressions of absence status more variable than hitherto recognized.     

Complex Partial Status Epilepticus Provoked by Ingestion of Alcohol: A Case Report

A 27-year-old man had a 5-year history of a peculiar state characterized by clouded consciousness provoked by ingestion of alcohol. The episode was identified electroclinically as nonconvulsive status epilepticus and was activated by ingestion of alcohol. Single-photon emission computed tomography (SPECT) examination during the nonconvulsive status epilepticus showed increased regional cerebral perfusion in the right frontal region. This finding, along with the EEG expressions, supports a cortical origin of the nonconvulsive status epilepticus and a diagnosis of complex partial status epilepticus (CPSE). To our knowledge, this is the first report of a documented case of CPSE during intoxication provoked by alcohol ingestion and also of a SPECT examination during CPSE.     

Complex partial status epilepticus of extratemporal origin: report of a case

A 28-year-old woman with no history of seizure was 7 months pregnant when she developed a prolonged complex partial status epilepticus (CPSE) organized in recurrent complex partial seizures of occipital origin, which was ascertained by the presence of elementary visual hallucinations and nystagmus heralding the attacks. EEG demonstrated recurrent seizures starting from the right occipital area. This especially refractory case of CPSE resolved after treatment with antiepileptic drugs and termination of pregnancy by cesarean section.     

Generalized Convulsive Status Epilepticus in the Adult

Summary: Status epilepticus (SE) is denned as recurrent epileptic seizures without full recovery of consciousness before the next seizure begins, or more-or-less continuous clinical and/or electrical seizure activity lasting for more than 30 min whether or not consciousness is impaired. Three presentations of SE are now recognized: recurrent generalized tonic and/or clonic seizures without full recovery of consciousness between attacks, nonconvulsive status where the patient appears to be in a prolonged "epileptic twilight state," and continuous/repetitive focal seizure activity without alteration of consciousness. Generalized convulsive status epilepticus (GCSE) encompasses a broad spectrum of clinical presentations from repeated overt generalized tonic-clonic seizures to subtle convulsive movements in a profoundly comatose patient. Thus, GCSE is a dynamic state that is characterized by paroxysmal or continuous tonic and/or clonic motor activity, which may be symmetrical or asymmetrical and overt or subtle but which is associated with a marked impairment of consciousness and with bilateral (although frequently asymmetrical) ictal discharges on the EEG. Just as there is a progression from overt to increasingly subtle clinical manifestations of GCSE, there is also a predictable sequence of progressive EEG changes during untreated GCSE. A sequence of five patterns of ictal discharges has been observed: discrete electrographic seizures, waxing and waning, continuous, continuous with flat periods, and periodic epileptiform discharges on a relatively flat background. A patient actively having seizures or comatose who exhibits any of these patterns on EEG should be considered to be in GCSE and should be treated aggressively to stop all clinical and electrical seizure activity to prevent further neurological morbidity and mortality.     

Early-Childhood Progressive Myoclonus Epilepsy Presenting as Partial Seizures in Dentatorubral-Pallidoluysian Atrophy

Summary: Purpose: We explored the characteristics of epileptic seizures of progressive myoclonus epilepsy (PME) in 2 brothers with dentatorubral-pallidoluysian atrophy (DRPLA).
Methods: We obtained the case history of the siblings and ictal and interictal EEGs. Postmortem examination or demonstration of elongated CAG repeat in the gene for DRPLA was used to confirm the diagnosis.
Results: Two Japanese siblings developed PME characterized by versive or himiclonic seizures with or without secondarily generalized tonic-clonic convulsions. The elder brother regressed mentally and exhibited increasing spasticity after age 1 year. Myoclonus and seizures developed at age 4 years. The younger brother had shown psychomotor retardation before age 4 years, when he began to deteriorate further neurologically as the elder brother had. He also developed myoclonus and seizures at that age. Seizures in both patients remained partial until their deaths at ages 19 and 15 years, respectively. Ictal EEG verified partial onset of seizure evolving to generalized tonic-clonic seizure (GTCS). Interictal EEGs showed multifocal paroxysmal discharges with little or no diffuse paroxysms. Postmortem examination or genetic study confirmed the diagnosis of DRPLA.
Conclusions: Seizures of patients with DRPLA may present as partial seizures in children with early-onset PME.     

Psychomotor Status Induced by Temporal Lobe Encephalitis

Abstract: A case report of psychomotor status was presented. A 51-year-old male caught a cold and had a high fever at the end of September, 1979. On October 7th, he had a visual hallucination followed by a generalized seizure. After hospitalization he went through several fits of automatism. On October 15th, he was seized with automatism status in which the seizures were repeated every 25 minutes and there was no recovery of consciousness between the seizures. Although an intravenous injection of diazepam interrupted this status, on October 17th he showed subclinical seizure status on the EEG during which automatism traces were rarely found. By the medication of carbamazepine and clonazepam, he improved gradually. CT-scan revealed the disappearance of the right sylvian fissure and CSF showed findings of viral encephalitis. Consequently, he was considered to have been suffering from temporal lobe encephalitis and shown an episode of psychomotor status with automatisms and automatism traces during which typical automatism status was observed as well.     

Complex partial status epilepticus in young children

Complex partial status epilepticus (CPSE) has rarely been described in children. We have recently studied four girls, aged 1 to 4 years, who presented with CPSE. Their seizures were characterized by decreased level of consciousness, lack of response to familiar persons, diminished response to pain, staring, slow visual tracking, eye deviation, picking at nearby objects, and lip smacking. Three patients developed focal clonic activity during their seizures and one progressed to a generalized motor seizure after 4 hours of CPSE. Two patients had ictal electroencephalograms demonstrating temporpoccipital poly spikes and slow waves.     

Prolonged Psychic Epileptic Seizures: A Study of the Absence Status

Long-lived epileptic seizures associated with spike-and-wave complexes are presently considered to be the absence status, i.e., the generalized nonconvulsive status. EEG radiotelemetry allowed us to record three prolonged seizures of 3 epileptic patients. Clinical manifestations included selective rather than global impairment of higher cortical functions. Clinical impairment appeared only when patients were in a state of activity and if those altered functions were used. EEG abnormalities were diffuse, but among them spike-and-wave complexes were never diffuse. It was impossible to establish close electroclinical correlation. However, the clinical and electrical evolution was roughly isomorphic, i.e., cyclic. Major clinical manifestations were associated with spikes rather than with slow waves. Lastly, patients showed common ictal psychopathological symptoms. The problem of classifying such seizures in either the generalized or partial status is discussed. The role of selective impairment of mental functions in psychopathological symptoms is also dealth with.     

No, some types of nonconvulsive status epilepticus cause little permanent neurologic sequelae (or: « The cure may be worse than the disease »)

Nonconvulsive status epilepticus (NCSE) is characterized by a cognitive or behavioral change which lasts for at least 30 minutes, with EEG evidence of seizures. Although there is little argument that generalized nonconvulsive status epilepticus (GNSE) does not cause lasting deficits, there is still debate regarding the morbidity of complex partial status epilepticus (CPSE). Because the EEG is used for diagnosis, a strong argument can be made that NCSE is significantly under-recognized and diagnosed. Furthermore, since the documented cases of permanent neurologic sequelae are few, the potential permanent morbidity from CPSE may be significantly exaggerated. The literature indicates that comatose patients have a poor prognosis largely as a result of comorbid conditions and coma, whereas lightly obtunded or slightly confused patients with NCSE have little or no sequelae. Patients with NCSE may suffer (hypotension and respiratory suppression) from iatrogenic 'aggressive' treatment with intravenous anti-epileptic drugs (IV-AEDs), and the findings in the literature indicate that subjects treated with benzodiazepines may have a worse prognosis. The clinician must balance the potential but rare neurologic morbidity associated with NCSE against the not infrequent morbidity caused by IV-AEDs. Better stratification of the level of consciousness and comorbid conditions is needed when evaluating outcomes so as to clearly distinguish among the deficits due to: comorbid conditions; the effects of treatment and the effects of status epilepticus (SE) proper.     

Frontal nonconvulsive status epilepticus associated with high-dose tiagabine therapy in a child with familial bilateral perisylvian polymicrogyria

PURPOSE: Antiepileptic drugs are known to exacerbate absence and myoclonic seizures, especially in patients with idiopathic generalized epilepsies. Exacerbation of nonconvulsive generalized seizures in patients with partial epilepsy is less common. Recently, however, a number of cases of putative generalized nonconvulsive status epilepticus (NCSE) or NCSE without further specification have been reported in patients with chronic partial epilepsy treated with the gamma-aminobutyric acid reuptake inhibitor tiagabine. Although complex partial status epilepticus during tiagabine therapy has also been reported, possible precipitation of NCSE specifically associated with frontal lobe discharges does not appear to have been recognized. In this communication, we describe the case of a boy with familial bilateral perisylvian polymicrogyria who developed frontal NCSE after being stabilized on high-dose tiagabine METHODS: A 12-year-old boy with familial bilateral perisylvian polymicrogyria, mental retardation, and refractory partial seizures was administered tiagabine in addition to sodium valproate. The tiagabine dosage was increased gradually up to 10 mg t.i.d. (1 mg/kg per day), resulting in complete seizure control. RESULTS: After 1 week on maintenance treatment, seizures were completely controlled, but the child developed hypoactivity, decreased reactivity, and affective detachment. An EEG recording revealed subcontinuous sharp-wave discharges with irregular runs of atypical spike-wave complexes over the anterior regions of both hemispheres, consistent with a diagnosis of frontal NCSE. A reduction in tiagabine dosage to 15 mg/day led to complete regression of the behavioral and affective changes and to disappearance of the subcontinuous EEG discharges. CONCLUSIONS: Although tiagabine-induced NCSE has been described previously, particularly in patients with preexisting spike-wave abnormalities, this is the first report that identifies its potential role in the precipitation of frontal NCSE.     

États de mal épileptiques non convulsifs

Non convulsive confusional status epilepticus is classically divided on the basis of the ictal EEG into absence status (AS) and complex partial status epilepticus (CPSE). The clinical presentation is often insufficient to establish diagnosis and emergency EEG is required. AS is a polymorphic condition that can complicate many epileptic syndromes. In half of cases, confusion of varying intensity is associated with bilateral periocular myoclonias. Clinical and EEG normalization is obtained after intravenous benzodiazepine injection. From a nosographic point of view, four types of AS may be recognized. Typical AS occurs as part of an idiopathic generalized epilepsy. Atypical AS occurs in patients with symptomatic or cryptogenic generalized epilepsies. “De novo” AS of late onset is characterized by toxic or metabolic precipitating factors in middle-aged subjects with no previous history of epilepsy. AS with focal characteristics occurs in subjects with a preexisting or newly developing partial epilepsy, most often of extratemporal origin. Most cases are transitional forms between these four entities. CPSE is characterized by continuous or rapidly recurring complex partial seizures which may involve temporal and/or extratemporal regions. Cyclic disturbance of consciousness is characteristic of CPSE of temporal lobe origin, which requires vigorous treatment to prevent recurrence or cognitive sequelae. CPSE of frontal lobe origin is a diagnostic challenge: it is rare, the symptoms are unusual, and extensive documentation is required. A focal, frontal lesion is discovered in one third of cases.     

Two Cases of Nonconvulsive Status Epilepticus in Association with Tiagabine Therapy

We report two patients with intractable partial seizures who developed generalized nonconvulsive status epilepticus (NCSE) after receiving tiagabine (TGB). Neither had a history of absence seizures or generalized epileptic discharges on prior EEG monitoring. Clinicians need to be aware of a possible association between TGB and NCSE.     

Electroencephalographic characteristics of Dravet syndrome

In Dravet syndrome, interictal and ictal electroencephalography (EEG) recording may remain misleading, and are not specifically altered. Moreover, there is a great polymorphism of clinical and EEG seizure types. Some can be observed in other epileptic syndromes, but others are more specific--particularly the peculiar unilateral seizures, the falsely generalized seizures, probably with a focal onset, and the unstable seizures. In some cases, the ictal manifestations are characterized by the persistent predominant recurrence of convulsive seizures, often induced by body temperature increase, eventually associated with partial complex seizures. The myoclonic events, absences with myoclonic component, obtundation status, and photosensitivity and/or pattern sensitivity are absent or appear relatively late and recur transiently for short periods. In these cases interictal EEG is characterized by the persistent paucity of paroxysmal discharges. In other cases, on a background of convulsive seizures and body temperature sensitivity, one may find a variable association of (1) myoclonic seizures of different types, (2) a strong sensitivity to light and pattern stimulations, with early onset and persistent in time. In these cases, interictal paroxysms and spontaneous and induced (intermittent photic stimulation, patterns, and eye closure) stimulation tend to appear early and to be frequent and persistent during the evolution. According to these electroclinical patterns it is possible to divide the population into two subsets, both sharing common genetic mechanisms but with a different clinical outcome.     

Distinct behavioral and EEG topographic correlates of loss of consciousness in absences

PURPOSE: To describe the behavioral and EEG topographic correlates of absences with 3-Hz generalized spike-waves and partitioned impairment of consciousness. METHODS: Two adult women had so-called "phantom" absences, characterized by brief and mild impairments of consciousness that were previously inconspicuous to both patient and physician. Neuropsychological examination was performed under video-EEG monitoring during absence status. EEG topographic mapping of spike-wave discharges was obtained in the two cases. RESULTS: Only mild attentional and executive disturbances were observed during absence status despite prolonged discharges. Spike-wave bursts were associated with selective impairment in the initiation of response and self-generated action, whereas short-term storage of external information during discharges was fully preserved. This is consistent with a predominant involvement of frontomesial cortex demonstrated by topographic mapping of spike-wave discharges in the two cases. By contrast, in two other patients with typical absences and a complete lack of retention for information given during the discharges, topographic mapping found a more lateral frontal involvement by spike-wave activity. CONCLUSIONS: Different types of absence seizures may impair distinct components of conscious behavior. A predominant involvement of frontomesial thalamocortical circuitry may underlie an "inconspicuous" disorder of consciousness as seen in phantom absences with selective loss of initiation and goal-oriented behavior, whereas involvement of more lateral frontal areas in typical absences may additionally disrupt working memory processes.     

Identification of ictal foci by EEG and MR apparent diffusion coefficient map in a case of human herpesvirus 6-associated encephalopathy

We report here a 1-year-old boy with human herpesvirus 6 (HHV 6) -associated encephalopathy. On the 3rd day of fever, he had a generalized tonic seizure followed by mild disturbance of consciousness, which recovered completely the next day. Two days later, he had skin rash of exanthema subitum, and his consciousness declined frequently. EEG demonstrated rhythmic wave bursts originating from central areas bilaterally, followed by a generalized spike-and-wave complex which was associated with disturbed consciousness. We made the diagnosis of a status of complex partial seizures. Because the focal discharge on ictal EEG, MRI was performed. In the subcortical white matter of the frontal-parietal lobes, there were high signals on diffusion-weighted MRI, the apparent diffusion coefficient of which was much lower than that of normal controls. The affected areas soon disappeared with improvement of clinical symptoms. The transient MRI findings may indicate reversible cytotoxic brain edema. He showed no neurological sequelae as yet.     

Dravet syndrome: a technologist's perspective

Dravet Syndrome (DS), also known as Severe Myoclonic Epilepsy in Infancy (SMEI) is a rare, primarily genetic disorder which develops in infancy. The characteristics of DS are frequent, prolonged, primarily generalized seizures which occur initially with fever and eventually evolve to multiple afebrile seizure types such as myoclonic, atypical absence, and complex partial seizures. Patients, who are initially developmentally normal, will experience concomitant developmental regression as the syndrome progresses. Because it is a childhood disorder, DS is not well known outside the realm of pediatrics. An astute EEG technologist should be able to recognize key factors both clinically and electrographically which point suspicion to the diagnosis of Dravet Syndrome.