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Idiopathic generalised epilepsy in adults manifested by phantom absences, generalised tonic-clonic seizures, and frequent absence status.

OBJECTIVES: To describe the clinical and EEG features of adult patients with very mild absences, late onset generalised tonic clonic seizures, and frequent absence status. METHODS: Patients were referrals to a clinic for epilepsies. They all had clinical assessment and EEG, video EEG, or both for documentation of absences. RESULTS: Of 86 adults with idiopathic generalised epilepsies and EEG/video-EEG documented absences, 13 patients showed similar clinico-EEG features with: (a) "phantom absences" consisting of mild ictal impairment of cognition associated with brief (3-4 s), generalised 3-4 Hz spike/multiple spike and slow wave discharges; (b) infrequent, mainly late onset, generalised tonic clonic seizures, and (c), absence status which occurred in six of them either in isolation or terminating with generalised tonic clonic seizures. None of the patients had myoclonic jerks or photosensitivity. Two patients were father and daughter and another patient had a family history of infrequent generalised tonic clonic seizures. CONCLUSION: It seems that this is an idiopathic generalised epilepsy syndrome in adults which has not been previously recognised.

Adolescent↗

Unilateral absence of the basal ganglia plus epilepsy without motor symptoms.

A patient with absence of the basal ganglia and refractory epilepsy without impairment of pyramidal or extrapyramidal motor function is reported. Imaging findings suggest a vascular insult as etiology. Preserved motor function could be explained by neuronal plasticity involving contralateral corticostriatal and pallidothalamic connections and points to a lesion received in early pregnancy.

Adolescent↗

Case summary: Benjamin.

Benjamin was diagnosed with Aspberger's syndrome on starting school. Prior to that, at the age of five years he presented with seizures involving deviation of the head and eyes to the right and jerking of the limbs. EEG was very abnormal with multifocal paroxysmal activity and occasional bursts of spike and slow wave activity. CT scan and MRI were normal and investigations failed to identify any underlying aetiology for the seizures. Carbamazepine was initially successful in controlling the seizures but improvement was not sustained despite maximal tolerated doses. A similar course of events was seen with sodium valproate, initially in combination with ethosuximide (for emergent tonic-clonic and absence seizures), then with vigabatrin (which unmasked myoclonic seizures), clonazepam and finally lamotrigine. The EEG remained abnormal even during periods of improved seizure control and there was no improvement in his Aspberger's syndrome. His epilepsy syndrome has not been classified. Subsequent treatment options include lamotrigine monotherapy.

Anticonvulsants↗

Reading-induced absence seizures.

Reading epilepsy usually presents with jaw myoclonus and generalized tonic-clonic seizures. We report a 12-year-old girl with absence seizures induced by reading, which were diagnosed by video EEG. An absence seizure with generalized 3-Hz spike-and-wave discharge occurred within 30 seconds of each reading session. Treatment with valproate caused complete seizure control, with therapy successfully discontinued after 2 years.

Child↗

Prognostic factors in absence seizures.

A prospective follow-up study was conducted on 48 epileptic patients (22 males and 26 females) who had participated in a collaborative study of absence seizures about 7 years earlier. Multivariate analysis showed that the significant prognostic factors for absence seizures were normal or above normal intelligence and normal electroencephalographic background activity. For any seizure type, significant prognostic factors were a negative history of generalized tonic-clonic seizures, normal or above normal intelligence, and a negative family history of seizure disorders. Nearly 90 percent of the patients with all significant prognostic factors for both absence seizures and seizures of all types ceased having seizures.

Adolescent↗

Epilepsies with astatic seizures of late onset.

In 17 patients with a long course of epilepsy astatic seizures became apparent after the age of 14 years. In the patients' childhood astatic seizures had not been observed. The patients suffered from epilepsies with absences and awakening grand mal or psychomotor fits and sleeping grand mal. The EEG revealed spike-wave variant and spike-and-wave complexes as in the Lennox-Gastaut syndrome. Age dependency of Lennox-Gastaut syndrome is discussed. The described type of epilepsy can be understood as a "Lennox-Gastaut syndrome of late onset".

Adolescent↗

Drug therapy reviews: drug therapy of status epilepticus.

Drug treatment of status epilepticus is reviewed. Tonic-clonic, focal motor, complex partial and absence status epilepticus are discussed. In managing tonic-clonic status epilepticus one should: (1) maintain vital functions at all times, (2) identify and treat precipitating factors and (3) administer an intravenous loading dose of phenytoin sodium or phenobarbital sodium. Careful use of i.v. diazepam sometimes helps to achieve these objectives. Intravenous phenytoin sodium and phenobarbital sodium provide definitive, long-term control of tonic-clonic seizures but must be administered slowly and require time to reach peak brain concentrations. Intravenous diazepam appears to enter and exit from the brain rapidly and may control seizures while therapeutic brain concentrations of long-acting drugs are being achieved. Phenytoin, phenobarbital and diazepam should not be administered intramuscularly in treating status epilepticus. Treatment of focal motor and complex partial status epilepticus is similar to that of tonic-clonic status epilepticus, but i.v. diazepam is required less frequently and loading doses of phenytoin and phenobarbital sometimes can be given more slowly. Status epilepticus of the absence type is managed with i.v. acetazolamide sodium or diazepam. Paraldehyde, muscle relaxants, general anesthesia and lidocaine may be tried when conventional therapies fail.

Anesthesia, General↗

Eye closure sensitivity and epileptic syndromes: A retrospective study of 26 adult cases.

PURPOSE: The transient, mainly generalized, together with brief changes in EEG baseline immediately after eye closure, is called 'eye closure sensitivity' (ECS) which was first reported by Robinson in 1930 and there have been limited number of studies investigating ECS and epilepsy syndromes. Therefore, we aimed to reveal the possible relationship between ECS and the epilepsy syndromes in our adult patients. PATIENTS AND METHODS: Adult patients monitored in Hacettepe University Hospitals EEG Laboratory, from January 1995 to December 2005, were screened retrospectively for the presence of ECS. During EEG recording, all patients were asked to open their eyes for at least for 10s and close their eyes at the end of this period in six different montages. Hyperventilation and photic stimulation were performed according to the standard protocol of IPS. RESULTS: Twenty-six patients have had ECS during EEG recording. Nineteen (73%) were women, and mean age of the patients was 24 years. The mean ages were 12.3+/-5.4 years for seizure onset and 18.5+/-4.9 years for initial detection of ECS. Eleven had a history of febrile seizures. There was a family history for epilepsy in five patients. Photosensitivity in their EEG was noticed in 11 patients. ECS was established in five epilepsy syndromes: eyelid myoclonia with absences (EMA); (n: 6), juvenile myoclonic epilepsy (JME); (n: 6), idiopathic generalized epilepsy (IGE with tonic clonic seizure); (n: 4), juvenile absence epilepsy (JAE); (n: 1) and idiopathic occipital lobe epilepsy (IOE) (n: 9). All patients were seizure free with or without anti-epileptic medication during follow up. CONCLUSION: ECS is more common in females. It may overlap with photosensitivity but be independent from photosensitivity. It may be seen in different epilepsy syndromes including IOE which was not reported previously.

Adolescent↗

[Variability of epileptic seizure phenomenology in infants and children].

It is well known that some patients may have two or more different types of seizures. Partial epilepsies and more than 15 different epileptic syndromes (e.g. West or Lennox-Gastaut syndrome or epilepsies with absences) can lead to the intraindividual manifestation of two or more seizure types. Among 728 children with epilepsy seen at the outpatient department 9.1% had two different types of seizures and 1.5% had more than two types of seizures. The most common association observed was between tonic-clonic seizures and absences. The most frequently observed epileptic syndromes were in decreasing order: multifocal epilepsies, epilepsies with absences, Lennox-Gastaut syndrome and West syndrome. The association of two or more different seizure types in the same patient seems to be a negative prognostic factor.

Child↗

Non-convulsive status epilepticus: causes, treatment, and outcome in 65 patients.

The incidence of non-convulsive status epilepticus (NCSE) in The Netherlands is not known. Files of admissions in the years 1980-7 were studied from 40 adult patients (older than 15 years) with complex partial status epilepticus (CPSE) and 25 with absence status epilepticus (ASE). The clinical presentation sometimes made distinction between CPSE and ASE possible. Focal clinical signs were more frequent in CPSE; a fluctuating level of consciousness was more often present in ASE. All patients, but one, with ASE and most patients with CPSE (28) were known to have had previous epilepsy. Outcome in ASE was good in all. Outcome in CPSE depended on the underlying cause and quality of treatment. In three patients inadequate treatment probably contributed to morbidity.

Adolescent↗

Electroencephalographic features of epileptic drop attacks and absence seizures: a case study.

A study of epileptic drop attacks (EDA) by simultaneous video-polygraphic recordings was carried out in one epileptic patient with myoclonic astatic seizures (Doose syndrome). EDA was shown to correspond to a burst of generalized bilaterally synchronous spike and wave complexes (GBSSW) at 3 Hz. Absence seizures were also observed with a burst of GBSSW with similar characteristics. The amplitudes of the corresponding slow wave component of GBSSW among the three intensities of atonia, i.e. complete atonia, minor atonia and no discernible atonia (control), was compared. A high amplitude was demonstrated to correspond with more pronounced atonia and a lower amplitude with reduced or absent atonia. These findings suggest that EDA corresponding to GBSSW have a neurophysiological mechanism in common with absence seizures, and that if the GBSSW is intense, it may be sufficient to cause immediate loss of global muscle tone.

Child, Preschool↗

Predictors in the treatment of difficult-to-control seizures by electrical stimulation of the centromedian thalamic nucleus.

OBJECTIVE: To evaluate the efficacy of chronic electrical stimulation of centromedian thalamic nuclei (ESCM) in the treatment of difficult-to-control seizures. METHODS: Thirteen patients underwent ESCM for periods ranging from 12 to 94 months (mean, 41.2 mo) with electrodes stereotactically placed in both centromedian nuclei and connected to internalized stimulation systems. Electrode placement was guided by ventriculography and confirmed with magnetic resonance imaging before stimulation systems were internalized. Anatomic and electrophysiological confirmation of the electrodes' position was accomplished by plotting electrode position on anatomic sections of Schaltenbrand and Bailey's atlas, and testing cortical recruiting responses and electroencephalogram desynchronization elicited by acute low- or high-frequency stimulation, respectively. RESULTS: Improvement was highly significant for generalized tonicoclonic seizures and atypical absences. Better results were obtained for Lennox-Gastaut syndrome. These results were accompanied by a significant decrease in generalized spike-wave and secondary synchronous discharges, as well as focal spikes in the frontal regions. In contrast, ESCM reduced neither complex partial seizures nor focal spikes in temporal regions. Outcomes using ESCM for generalized epilepsy were better in patients in whom anatomic and electrophysiological confirmation of electrode placement was correct than in those in whom the target was missed bilaterally (P < 0.001). The effect was sustained during the observation period and was better for longer-term than for shorter-term stimulation periods. CONCLUSION: ESCM is an efficient and safe procedure for controlling certain seizure types, if patient selection and stereotactic placement are satisfactory.

Adolescent↗

Imaging the neural substrates involved in the genesis of pentylenetetrazol-induced seizures.

PURPOSE: Functional imaging of animal models makes it possible to map the functional neuroanatomy contributing to the genesis of seizures. Pentylenetetrazol (PTZ)-induced seizure in rats, a relevant model of human absence and of generalized tonic-clonic epilepsy, was used to stimulate seizure activity within 30 s of administration while collecting continuous, high-resolution, multislice images at subsecond intervals. METHODS: Pilot studies were conducted to establish a quick and effective PTZ model for the imaging experiments. PTZ was then used to stimulate seizure activity in rats while collecting multislice functional MRI (fMRI) images from the entire forebrain at 4.7 Tesla. Ethosuximide (ESM) also was used to block seizure activity. RESULTS: Within 2-4 s of PTZ administration, a rapid increase in blood oxygen level-dependent (BOLD) signal intensity was noted in the thalamus, especially the anterior thalamic nuclei. Activity in the anterior thalamus peaked approximately 15 s before seizure onset and was more than twofold greater than that in all other thalamic areas. The retrosplenial cortex showed a twofold greater increase in activity as compared with other cortical areas, also peaking at approximately 15 s. The dentate gyrus was twice as active as other hippocampal areas but peaked just before seizure onset. Treatment with ESM blocked seizures, decreasing PTZ-induced activation in most forebrain areas. The anterior thalamus and retrosplenial cortex were essentially blocked by pretreatment with ESM. CONCLUSIONS: The anterior thalamus, retrosplenial cortex, and dentate gyrus show the greatest increases in BOLD signal activity before seizure onset. Neurons in these areas may contribute to the neural network controlling the initiation of generalized tonic-clonic seizure.

Animals↗

Fixation-off sensitivity and generalized epileptic EEG induced by eyes closed.

This report describes a female with eyelid fluttering with absence seizures, infrequent generalized tonic-clonic seizures, and mild mental retardation. Interictal and video-electroencephalography evaluations revealed normal activity while eyes were open but continuous generalized discharges with eyes closed (eyes closed induced abnormality), as well as fixation-off sensitivity. This patient is in the group of a pure and distinct clinical form of fixation-off sensitivity cryptogenic generalized epilepsy.

Child↗

Delayed absence seizure: a complication of intrathecal fluorescein injection. A case report and literature review.

Intrathecal fluorescein injection has a long history of use by surgeons to determine the exact site of a cerebrospinal fluid (CSF) leak from the skull base. This method, however accurate, is not without complications. We present a case of grand mal and absence seizure after intrathecal fluorescein injection and discuss the possible aetiological factors. We also review the articles in the diagnostic methods for the CSF rhinorrhoea and the complications of the intrathecal fluorescein injection.

Anesthesia, General↗