Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0238111 (Lennox-Gastaut syndrome)
 861 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The correlations between sleep and prolonged epileptic activity are discussed on the basis of the status classification of Gastaut (1983). Little information is available on the interrelation of sleep and the status of tonic-clonic seizures (grand mal status). Most important is the therapeutical management of these cases. Tonic seizures have been reported to occur in large numbers during NREM sleep in patients with Lennox-Gastaut syndrome. A status-like increase is possible. Tonic seizures occur almost exclusively during sleep. Myoclonic status epilepticus arising (a) in the course of primary generalized epilepsy and (b) in the course of encephalopathies, are usually markedly attenuated during sleep. In absence status (petit mal status) synchronized sleep generally fragments the continuous discharge which is replaced by isolated bursts of polyspikes, or polyspike and wave complexes. The absence status can recur upon awaking during the night or in the morning. The abnormal EEG activity of a petit mal status can, however, occasionally persist during the whole night. Improvement as well as activation during sleep have been observed in elementary (= simple) partial status epilepticus; improvement seems to be more frequent. Epilepsia partialis continua may persist or decrease during sleep. An increase as well as decrease of motor phenomena has been observed during the REM stages. 'Epileptic aphasia' of childhood is associated with subclinical bioelectric status epilepticus during sleep. The electrical status epilepticus must be delineated as a separate group. The term encephalopathy related to electrical status epilepticus during slow sleep (ESES) has been proposed on the basis of associated psychic syndromes. This form of status epilepticus disappears during the waking state and during REM sleep. Cases with hypsarrhythmia without clinical signs may also be classified under the group of electrical or bioelectrical status. In some cases, a continuous hypsarrhythmia is observed only during sleep. In this context, one must also mention those patients who demonstrate continuous activation of spikes, or spike and wave potentials (without clinical seizures) during eye closure.
 ...
PMID:Sleep and prolonged epileptic activity (status epilepticus). 176 86

Serial polysomnograms were performed on 11 children with primary Lennox-Gastaut syndrome (LGS), 6 control children with other seizure disorders, and 12 who were developmentally normal. Five LGS children had abnormal polysomnograms with either complete absence or marked reduction of REM sleep; the other six LGS children had only a mild reduction of REM sleep. The percentage of REM in LGS children was less than in the controls with other seizure disorders (p less than 0.05) or the normal children (p less than 0.005). The scatter of REM percentages in LGS may imply heterogeneity of the syndrome, perhaps related to the severity of brainstem dysfunction or neurochemical derangement.
 ...
PMID:Sleep patterns in the Lennox-Gastaut syndrome. 374 89

We report a case of a 43-year old woman with Lennox-Gastaut syndrome who exhibited atypical absence seizures, atonic seizures and generalized toniclonic seizures which were not controlled by antiepileptic drug (AED) treatment. Because of this, felbamate (FBM) (1800 mg per day) was progressively added to the pre-existent therapy. The patient underwent a 24-hour-video-EEG monitoring before and after 4 months of FBM therapy. Analysis of the video-EEG signal recorded during wakefulness revealed the presence of ictal activity represented by repetitive, bilateral, slow spike and wave bursts underlying atypical absence seizures; the ictal activity occurring during non-REM sleep was characterized by runs of bilateral, rapid, high-voltage spikes followed by slow spike and wave complexes corresponding to brief tonic seizures. FBM therapy induced disappearance of the EEG ictal slow, spike and wave complexes leaving rather unaffected the runs of spikes. Computerized analysis of both the EEG background activity and the sleep structure displayed a better organization of the global cerebral rhythms under FBM treatment. Our findings suggest a selective effect of FBM on the ictal atypical spike and wave pattern. The differential effect of FBM on ictal patterns may be a reflection of a different action on the excitatory and inhibitory systems.
 ...
PMID:Changes of the EEG paroxysmal pattern during felbamate therapy in Lennox-Gastaut syndrome: a case report. 977 42

The subjects were 25 children, including West syndrome, Lennox-Gastaut syndrome, childhood absence epilepsy (CAE) and localization-related epilepsies. Clinical seizures occurred only during waking state in 14 patients (including the cases of West syndrome, CAE and epilepsy with myoclonic absences (EMA)), only during sleeping state (especially during non-REM sleep in five patients, including the case of benign epilepsy with centrotemporal spike), and diffusely during waking and sleeping states in six patients (including the case of early infantile epileptic encephalopathy with suppression burst). These three types were observed in the cases of Lennox-Gastaut syndrome and localization-related epilepsies. Subclinical ictal discharges occurred during REM sleep in West syndrome, and during REM and non-REM sleep especially during non-REM sleep in CAE and EMA.
 ...
PMID:Epileptic seizures and sleep-wake rhythm. 1142 28

Non-rapid eye movement (NREM) sleep contains periods of arousal instability (cyclic alternating pattern or CAP) and periods of arousal stability (non-CAP). During CAP, arousal oscillates between higher (phase A) and lower (phase B) levels of activation. We evaluated the relationship between CAP and the occurrence of epileptic events, i.e. clinical seizures and generalized interictal discharges, during sleep in 10 patients with Lennox-Gastaut syndrome (LGS). The macro- and microstructure of sleep of 10 attended overnight polysomnograms were analyzed. Compared with 10 age- and gender-matched controls, patients with LGS had significantly less stage 2 and REM sleep and higher amounts of CAP rate (68% vs. 33%; P<0.0001). The number of generalized polyspike bursts per hour of sleep was highest in slow wave sleep (226.5+/-57.6) and lowest in REM sleep (3.9+/-1.5). The polyspike burst frequency was significantly greater (P<0.017) during CAP (213.2+/-60.1) than during non-CAP (100.3+/-40), and within CAP, generalized polyspikes occurred more often (P=0.005) during phase A (461.1+/-127.2) than during phase B (6.1+/-1.9). The total amount of generalized polyspike bursts identified in NREM sleep correlated positively both with the number of A phases containing at least one generalized polyspike (P=0.005) and with the mean number of polyspikes within each of these A phases (P<0.0001). Nocturnal clinical seizures occurred in 8 of the 10 patients and showed a similar trend. We conclude from our results that CAP modulates the occurrence of both clinical seizures and generalized epileptic discharges in LGS by means of a gate-control mechanism: an independent spike generator is inhibited in phase B and non-CAP and bursts with its intrinsic activity in phase A.
 ...
PMID:Sleep in Lennox-Gastaut syndrome: the role of the cyclic alternating pattern (CAP) in the gate control of clinical seizures and generalized polyspikes. 1207 49