Aphasic status epilepticus with periodic lateralized epileptiform discharges in a bilingual patient as a presenting sign of "AIDS-toxoplasmosis complex"

We describe an HIV-infected, bilingual patient presenting with Wernicke's aphasia due to partial status epilepticus with periodic lateralized epileptiform discharges, as the first sign of AIDS-toxoplasmosis complex. The localization of the native and secondary language centers in the brain and the possible role of recurrent seizures in the fluctuating course of Wernicke's aphasia in this patient are discussed. The clinical course of this patient supports the belief that a second language area for a second language learned in the later stages of life is located in an area different from that for the native language but still in close proximity to it.     

Non-convulsive status epilepticus presenting with Wernicke's aphasia

Ictal aphasia in adults is a rare phenomenon. Most reported cases manifest with non-fluent (Broca) aphasia. Ictal fluent (Wernicke) aphasia is less common. We report a 47-year-old, right-handed woman that presented with recurrent episodes of non-convulsive seizures in the form of Wernicke's aphasia for 2 weeks. An MRI of the brain showed an old cerebral infarction in the left parieto-occipital area. Scalp EEG revealed continuous periodic sharp waves at the left temporal regions with diffusion to the whole left hemisphere and at occasions to the right. This is followed by variable periods of post ictal slowing. Recurrence of the described ictal pattern was noted. Management of status epilepticus was started in the form of intravenous diazepam and a loading dose of phenytoin and phenobarbitone. After treatment, she improved clinically and the EEG improved with disappearance of the left temporal ictal rhythm and normalization of the EEG background. Thus, establishing the diagnosis of non-convulsive partial status epilepticus manifesting as ictal aphasia.     

Frontal lobe nonconvulsive status epilepticus: a case of epileptic stuttering, aphemia, and aphasia--not a sign of psychogenic nonepileptic seizures

Stuttering is a repetitive, iterative disfluency of speech, and is usually seen as a developmental problem in childhood. Acquired causes in adults include strokes and medications. When stuttering occurs with seizure-like events, it is usually attributed to psychogenic nonepileptic seizures. We describe an elderly man who experienced personality change and bouts of stuttering, followed by anarthria with preserved writing and then aphasia affecting written and uttered language, and ending with confusion. EEG recordings showed nonconvulsive status epilepticus (NCSE) with focality in the left frontal region followed by bifrontal NCSE. This case enlarges our understanding of the behavioral correlates of focal frontal seizures to include simple partial seizures with speech and then language output disturbances (aphemia, then aphasia), progressing to complex partial phenomenology in the setting of frontal NCSE.     

"De novo" non-convulsive status epilepticus in adults and in the elderly

There is a growing number of publications in the recent literature reporting the incidence of non-convulsive status epilepticus in the elderly, including both absence epilepsy and partial epileptic seizures. Absence status epilepticus creates a diagnostic problem because of its clinical features: confusion ranging from slight disorientation to stupor. Duration of such states may vary from one hour to a few weeks, with fluctuations and epileptic features in EEG recording (a typical pattern of spikes-slow waves, 3 Hz frequency, symmetrical and synchronical) that disappear after an intravenous injection of benzodiazepines. Absence status epilepticus can be evoked by toxic, metabolic or pharmacological factors as well as by convulsive epileptic seizures. Besides absence epileptic states of middle-cerebral origin there is a rising concern about absence status resulting from simple or complex partial seizures. Generalized non-convulsive status epilepticus following either simple partial or simple complex seizures is characterized by the presence of various focal signs associated with confusion, stupor or coma. The latter may be masking the clinical picture of an underlying cerebral pathology (e.g. brain tumor, hemorrhage, etc.), and epileptic changes can be seen in EEG recording only. Absence status epilepticus can occur in various forms of brain pathology, including stroke, brain tumors, traumatic lesions and other conditions, as well as in systemic diseases affecting the central nervous system function. Therefore, the authors emphasize the importance of electroencephalography in severely ill and unconscious patients, as well as the role of proper anti-epileptic treatment, as this may improve the outcome.     

A case of non-convulsive status epilepticus worsened Wernicke's aphasia reversely]

A 62-year-old right-handed woman had presented progressive speech impediment over 4 months. She was alert without any convulsions or involuntary movements. Neurological examination showed Wernicke's aphasia, constructional apraxia. Her magnetic resonance imaging (MRI) showed an old cerebral infarction in the left parieto-occipital area, in addition to ischemic changes in the bilateral deep white matter. Electroencephalography (EEG) revealed periodic lateralized epileptiform discharges (PLEDs) predominant in the posterior left hemisphere. The PLEDs as well as the cortical symptoms improved after an administration of anti-convulsive agents, thus establishing the diagnosis of non-convulsive status epilepticus (NSE). It should be emphasized that NSE manifesting as Wernicke's aphasia should be distinguished from dementia syndrome because it is a treatable disorder.     

Periodic episodes of aphasia as an unusual manifestation of partial status epilepticus.

Recurrent episodes of aphasia due to partial status epilepticus is an uncommon clinical entity. We report here a 78-year-old-woman with episodic aphasia which occurred periodically. During the ictal period, she was conscious, but had difficulty in speech and could not comprehend verbal commands. The ictal EEG showed continuous spike and sharp waves over the left frontotemporal area. After the administration of antiepileptic drugs, her language activity returned to near the baseline level and the epileptic discharges were significantly reduced. Nonconvulsive partial status epilepticus should be considered in the differential diagnosis of recurrent aphasia, even if the symptoms occur periodically.     

Seizures and epilepsy among children with language regression and autistic spectrum disorders

Clinical and subclinical seizures occur frequently among children with autistic spectrum disorders. Electrographic status epilepticus in sleep, or continuous spike-wave in slow-wave sleep, is a typical feature of acquired epileptic aphasia and Landau-Kleffner syndrome. Seizures and epilepsy are more common among children with autistic spectrum disorder who experience language regression, especially those who experience language regression after the age of 2 years. Although the seizures associated with Landau-Kleffner syndrome and with acquired epileptic aphasia can be easily treated, improvement in language function often does not follow successful treatment of seizures. There are no published randomized clinical trials of treatments for Landau-Kleffner syndrome or for autistic language regression. Broad-spectrum antiepileptic drugs not associated with cognitive slowing are probably the treatment of choice for epilepsy among children with autistic spectrum disorder. Large multisite trials are needed to determine treatment efficacy among children with Landau-Kleffner syndrome and acquired epileptic aphasia and to establish whether there is a cause-effect relationship between electrographic status epilepticus in sleep or continuous spike-wave in slow-wave sleep and autistic language regression.     

Prolonged ictal aphasia: A diagnosis to consider

Aphasia is a common symptom encountered by clinical neurologists. It is usually caused by strokes or lesions involving language regions of the brain, yet prolonged aphasia is rarely the sole manifestation of a simple partial status epilepticus. We report six patients, who suffered from prolonged ictal aphasia. All but one patient had a structural lesion in the left hemisphere, only three suffered from clinical seizures during or shortly prior to the aphasic episode. All patients had ictal patterns on the electroencephalogram (EEG), four of whom had periodic lateralized epileptiform discharges, and five showed frequent recurrent electrographic seizures during the aphasic state. The aphasia lasted several days in all patients, and it resolved after administration of antiepileptic drug treatment. In conclusion, prolonged ictal aphasia is a rare but important treatable cause of aphasia. Surface EEG recordings should be obtained in all patients with unexplained prolonged aphasia to diagnose this rare but treatable entity.     

Alzheimer's disease underlies some cases of complex partial status epilepticus.

Alzheimer's disease is a known risk factor for seizures, and age older than 60 years is a recognized risk factor for poor outcome from convulsive and nonconvulsive status epilepticus. The authors suspect that there may be a causal relationship between dementia pathology and the development and maintenance of refractory seizures. They report two selected patients with complex partial status epilepticus whose presentation and clinical course provide partial support for this hypothesis. Their methods include case reports with clinical, EEG, imaging, and pathologic correlations. The patients were 70 and 85 years of age. Both had central and peripheral brain atrophy on imaging studies (with some regions that were affected more than others), left temporal seizure foci corresponding to areas of greatest cortical atrophy, and early presentation with inhibitory epileptic symptoms (aphasia), with evolution to complex partial status epilepticus. Pathologic confirmation of Alzheimer's disease was obtained in one patient who had not been diagnosed previously. It involved maximally the cortex underlying the seizure focus. A diagnosis of probable Alzheimer's disease was established in the other patient. Alzheimer's disease may be causal in some cases of complex partial status epilepticus. Additional observations in support of this hypothesis are needed.     

Successful management of refractory neonatal seizures with midazolam

Seizures are indicative of underlying neurologic dysfunction in neonates. Repeated seizures may be deleterious to the brain even without disturbances of ventilation or perfusion. First-line antiepileptic drugs such as phenobarbital and phenytoin are not very effective in controlling seizures in neonates. Rapid control of status epilepticus with midazolam has been demonstrated in 2 previous studies with complete clinical and electrographic response in neonates who did not respond to phenobarbital and phenytoin. We report our experience with 3 neonates with status epilepticus. Seizures in all 3 neonates did not respond to phenobarbital and phenytoin but responded to midazolam infusion. Midazolam may be considered a safe and effective antiepileptic drug in refractory neonatal seizures of diverse etiologies.     

Electrical status epilepticus during slow sleep: one case with sensory aphasia

Electrical status epilepticus during slow sleep (ESES) is characterized by an EEG picture that justifies its name. It can be accompanied by epileptic seizures, speech and behavior disturbances and in rare cases by an acquired sensory aphasia. We describe the case of a six-year-old girl, whose EEG presented the typical ESES picture, and who in the span of one year developed a complete sensory aphasia, followed by motor aphasia. After 6 months of treatment with clobazam recovery of speech was nearly complete, but after 8 months clobazam lost its effectiveness and the girl presented a speech regression. Treatment with nitrazepam led to a complete recovery of speech for a second time, while at the same time ESES in the EEG again disappeared. This case, in addition to others described in the literature, suggests the possibility of a direct correlation between electrical abnormalities of the brain and cognitive and speech disturbances.     

Status epilepticus in a patient with fragile X syndrome: electro-clinical features and peri-ictal neuroimaging.

Fragile X syndrome (Fra-X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro-clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug-resistant seizures are uncommon, and the occurrence of clustering seizures or status epilepticus has only been reported anecdotally. We describe a Fra-X patient with refractory partial epilepsy related to a malformation of cortical development who experienced a partial status epilepticus that was well documented by video-EEG and MRI. We report the electro-clinical features and peri-ictal neuroimaging data.     

Aphasic status epilepticus

We studied a case of focal status epilepticus with aphasia as the sole manifestation, lasting 21 days. A 77-year-old woman developed aphasic seizures followed by aphasic status epilepticus 10 months after a left temporo-occipital hemorrhage. The diagnosis was made only after EEG, and seizures were stopped by anticonvulsant medication. Aphasic status epilepticus may be only a reflection of cerebral irritability, suggesting the possibility of a functional etiology for prolonged language disorders also in adults.     

Drug-induced seizures: a 10-year experience

Of 53 patients with drug-induced seizures seen in the last decade, 45% had single seizures, 40% had multiple convulsions, and 15% had status epilepticus. Generalized seizures with focal features were common, but simple partial (motor) seizures occurred in only two patients. Isoniazid, insulin, lidocaine, and psychotropic medications were the most common drugs that caused seizures. Forty-nine patients recovered without ill effects, but 4 patients died of cardiovascular complications. The combined cardiovascular toxicity of the convulsants, antidotes, and anticonvulsants was more important than the number or duration of seizures in determining outcome.     

A comparative study of absence status epilepticus between children and adults

Based on the clinicoelectrographic data of 28 patients (14 children and 14 adults) with absence status epilepticus thoroughly documented by CCTV/EEG, it was found that there were significant differences between the children and adults. In childhood, absence status tended to occur in those who had experienced individual short-lived atypical absence seizures and also other types of generalized seizure. In contrast, there was a general tendency for absence status in adulthood to occur in females without individual absence seizure. With respect to the clinicoelectrographical manifestations, absence status with a decreased postural tone was prone to be associated with a more profound clouding of consciousness, whereas in those with myoclonic components there was a less profound clouding. The former was found solely in children while the latter was both in children and in adults. During absence status, the focal motor features with or without secondarily generalized convulsions were observed in 8 adult patients (57%). On the other hand, no focal motor manifestations were observed during absence status in children. Absence status is composed of two modalities: either a prolongation or repetition of absence seizures. It was demonstrated that, in children, either the prolongation or repetition of individual absence seizures developed into absence status. The short-lived absence was of an atypical nature, whereas in adults, absence status consisted of the prolongation of an absence seizure which occurred in patients with no experience of individual absence seizures. With respect to the drug treatment, antiabsence drugs had some effect in children, while none of the adult patients responded either to the antiabsence drugs or other antiepileptic drugs. The long-term seizure prognosis was not necessarily poor in children but invariably poor in adults. Four adult patients showed diffuse, but unilateral frontally accentuated asymmetrical paroxysmal activity during the status. Three of them showed initial localized spike-wave discharges in the unilateral frontal region followed by a generalized spike-wave rhythm. Furthermore, all of these 4 patients with focally accentuated ictal EEGs have shown partial motor seizures intermingled with absence status.(ABSTRACT TRUNCATED AT 400 WORDS)     

Recurrent prolonged fugue states as the sole manifestation of epileptic seizures

A fugue state is defined as an altered state of consciousness with varying degrees of motor activity and amnesia for the event. It may last for hours to days and may be psychogenic or organic in nature. Epileptic fugue states can be encountered in patients with absence or complex partial nonconvulsive status epilepticus or may occur as a postictal phenomenon in patients with generalized seizures. “absence status epilepticus” (AS) is rare and seen in only 2.6% of the cases with “childhood absence epilepsy” (CAE). The diagnosis of AS can be elusive, but sudden onset and termination of the fugue state, classical electroencephalogram (EEG) features, and response to a therapeutic trial of benzodiazepines helps in confirming the diagnosis and differentiating it from nonepileptic fugue states.We report a childhood onset case, with a 10 years history of recurrent episodes of prolonged fugue state lasting for up to 24 h, as the sole manifestation of epileptic seizures. The EEG features were suggestive of an AS, but there was no history of typical absences, myoclonus, or generalized tonic clonic seizures. This unusual and rare case cannot be categorized into one of the defined epilepsy syndromes like CAE but belongs to a recently identified syndrome of idiopathic generalized epilepsy known as “Absence status epilepsy” in which AS is the sole or the predominant seizure type.Key Words: Absence status epilepticus, fugue state, idiopathic generalized epilepsy     

Early-Onset Benign Childhood Occipital Seizure Susceptibility Syndrome: A Syndrome to Recognize

Early-onset benign childhood occipital seizures (EBOS) described by Panayiotopoulos constitute the commoner after the rolandic phenotype of a childhood seizure susceptibility syndrome. EBOS are the clinical representative of occipital spikes. Their cardinal features are infrequent (often single) partial seizures manifested with deviation of the eyes and vomiting, frequently evolving to hemi- or generalized convulsions. Ictal behavioral changes, irritability, pallor, and rarely cyanosis, and eyes wide open are frequent. Retching, coughing, aphemia, oropharyngolaryngeal movements, and incontinence may occur. Consciousness is usually impaired or lost, either from the onset or the course of the fits, but in a few children, it may be preserved. Duration varies from a few minutes to hours (partial status epilepticus). Seizures are usually nocturnal, but semiology is similar in nocturnal or diurnal fits. Onset is between 1 and 12 years with a peak at 5 years. One third of children have a single seizure, the median total number of fits is two to three, and the prognosis is invariably excellent, with remission usually occurring within 1 year from onset. A few children may later develop rolandic or other benign partial seizures. The likelihood to have seizures after age 12 years is exceptional and rarer than that of febrile convulsions. EEG shows occipital paroxysms demonstrating fixation-off sensitivity, but random occipital spikes, occipital spikes in sleep EEG alone, or normal EEG may occur. Centrotemporal and other spike foci may appear in the same or more frequently in subsequent EEGs. The EEG does not reflect clinical course and severity.     

Febrile seizures in patients with complex partial seizures

Febrile seizures occurred in 14 of 155 (9%) out-patients with complex partial seizures. Twelve patients had prolonged or recurrent febrile seizures, convulsive status epilepticus or a transient postictal neurological deficit. Febrile seizures were associated with perinatal abnormalities, an earlier onset of epilepsy and with a poor seizure control. Recurrent febrile seizures or those with complicating features are associated with an unfavourable therapeutic outcome in adult patients with complex partial seizures.     

Neurophysiological aspects of neonatal seizures

Recently, amplitude-integrated EEG (aEEG) has been increasingly used and proved useful in neonatal intensive care units (NICU) for the management of neonatal seizures. It does not replace, but is supplementary to standard EEG. This article reviews some of findings obtained with standard EEGs, and tries to interpret them with recent findings in the field of basic science. Seizures mainly occur in active-REM sleep in neonates. This is in sharp contrast to those in older children and adults, in whom epileptic seizures occur mainly in NREM sleep. This may be explained by neurotransmitter effects on sleep mechanisms of the neonatal brain that are different from those of older individuals. When all clinical seizures have no electrical correlates, they are non-epileptic, but when the correlation between clinical seizures and frequent electrical discharges are inconsistent, they should rather be considered epileptic, reflecting progression of status epilepticus causing electro-clinical dissociation. Electro-clinical dissociation is not a characteristic of neonatal seizures per se, but a feature of prolonged status epilepticus in adults as well as children. It occurs when prolonged status epilepticus itself causes a progressively severe encephalopathy, or when status occurs in the presence of a severe underlying encephalopathy. In neonates without pre-existing brain damage, frequent seizures per se may cause mild depression characterized by the loss of high voltage slow patterns, an important constituent of slow wave sleep reflecting cortico-cortical connectivity. Mild depression only in the acute stage is not associated with neurological sequelae, but previously damaged brain may be more vulnerable than normal brain.