B. Lucas

Sleep and brain lesions: a critical review of the literature and additional new cases

We present a comprehensive review of sleep studies performed in patients with brain lesions complemented by 16 additional personal selected cases and by discussion of the corresponding animal data. The reader is cautioned about the risk of establishing an erroneous correlation between abnormal sleep and a given disorder due to the important inter and intra variability of sleep parameters among individuals. Salient points are stressed: the high frequency of post-stroke sleep breathing disorders is becoming increasingly recognised and may, in the near future, change the way this condition is managed. Meso-diencephalic bilateral infarcts induce a variable degree of damage to both waking and non-REM sleep networks producing and abnormal waking and sometimes a stage 1 hypersomnia reduced by modafinil or bromocriptine, which can be considered as a syndrome of cathecholaminergic deficiency. Central pontine lesions induce REM and non-REM sleep insomnia with bilateral lateral gaze paralysis. Bulbar stroke leads to frequent sleep breathing disorders. Polysomnography can help define the extent of involvement of various degenerative diseases. Fragmented sleep in Parkinsons disease may be preceded by REM sleep behavioural disorders. Multiple system atrophies are characterised by important sleep disorganization. Sleep waking disorganization and a specific ocular REM pattern are often seen in supra-nuclear ophtalmoplegia. In Alzheimer patients, sleep perturbations parallel the mental deterioration and are possibly related to cholinergic deficiency. Fronto-temporal dementia may be associated with an important decrease in REM sleep. Few narcoleptic syndromes are reported to be associated with a tumour of the third ventricle or a multiple sclerosis or to follow a brain trauma; all these cases raise the question whether this is a simple coincidence, a revelation of a latent narcolepsy or, as in non-DR16/DQ5 patients, a genuine symptomatic narcolepsy. Trypanosomiasis and the abnormal prion protein precociously after sleep patterns. Polysomnography is a precious tool for evaluating brain function provided it is realised under optimal conditions in stable patients and interpreted with caution. Several unpublished cases are presented: one case of pseudohypersomnia due to a bilateral thalamic infarct and corrected by modafinil, four probable late-onset autosomal recessive cerebellar ataxias without sleep pattern anomalies, six cases of fronto-temporal dementia with strong reduction in total sleep time and REMS percentage on the first polysomnographic night, one case of periodic hypersomnia associated with a Rathkes cleft cyst and four cases of suspected symptomatic narcolepsy with a DR16-DQ5 haplotype, three of which were post-traumatic without MRI anomalies, and one associated with multiple sclerosis exhibiting pontine hyper signals on MRI.

Outcome at adulthood of the continuous spike-waves during slow sleep and Landau-Kleffner syndromes

Summary:  Purpose: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike–waves during slow sleep (CSWS) and Landau–Kleffner syndromes, which are two rare epileptic syndromes occurring in children.

Emergent EEG in clinical practice.

OBJECTIVE Emergency situations require a rapid and precise diagnostic approach. However, the exact role and value of the electroencephalogram (EEG) in emergent conditions have yet to be clearly defined. Our objective was to determine why clinicians order an emergency EEG, to assess to what extent it helps establish a correct diagnosis and to evaluate the result it has on subsequent patient management. METHODS We studied all successive emergency EEGs ordered during a 3-month period in our institution. We analyzed the reasons why each EEG was ordered and interviewed the prescribing clinicians in order to determine the impact the result of the EEG had on the diagnosis and subsequent therapeutic management. RESULTS We prospectively studied a total of 111 consecutive recordings. The main reasons for ordering an emergent EEG were: suspected cerebral death (21%), non-convulsive status epilepticus (19.7%), subtle status epilepticus (14%) and follow-up of convulsive status epilepticus (11.2%). In 77.5% of the cases the clinicians considered that the EEG contributed to making the diagnosis and that it helped confirm a clinically-suspected diagnosis in 36% of the cases. When subtle status epilepticus (SSE) or non-convulsive status epilepticus (NCSE) was suspected, the diagnosis was confirmed in 45% and 43.3% of the cases, respectively. In 22.2% of the requests involving follow-up of convulsive status epilepticus after initial treatment, the EEG demonstrated persistent status epilepticus. It resulted in a change in patient treatment in 37.8% of all the cases. When the EEG helped establish the diagnosis, patient treatment was subsequently modified in 46.6% of the cases. CONCLUSIONS This prospective study confirms the value of an emergent EEG in certain specific clinical contexts: the management of convulsive status epilepticus following initial treatment or to rule out subtle status epilepticus. An emergent EEG can also be ordered if one suspects the existence of non-convulsive status epilepticus when a patient presents with mental confusion or altered wakefulness after first looking for the specific signs suggesting this diagnostic hypothesis. SIGNIFICANCE After 50 years of development and use in daily practice, the EEG remains a dependable, inexpensive and useful diagnostic tool in a number of clearly-defined emergency situations.

Are frequent spikep-waves during non-REM sleep in relation with an acquired neuro-psychological deficit in epileptic children ?

In a population of 11 children with frequent spike waves during non REM sleep who had no neurological symptoms between birth and their first symptom, 3 groups were compared according to their neuropsychological performances. In the first group, the children had no intellectual deficit, in the second group, they had an acquired aphasia as in the Landau-Kleffner syndrome and in the third they had severe behavioural disorder and mental deterioration. The non REM sleep paroxysmic activity density tended to be highest in the third group, variable in the second group and moderate in the first group, and their topography was always generalized in the acute phase in groups II and III but asymmetrical in group I. The EEG anomalies disappeared during adolescence but in group II and III children a moderate to severe delay in school work persisted.

Two distinct classifications of adult epilepsies: by time of seizures and by sensitivity of the interictal paroxysmal activities to sleep and waking

Two hundred and thirty-six adult epileptic outpatients were classified twice: firstly according to the time of seizures reported by the patient or his family in diurnal, nocturnal, awaking and diffuse epilepsies (Es) and secondly according to the sensitivity to sleep or waking of the interictal paroxysmal activities (PA) observed during a polysomnographic night session with a sleep PA increase, with a waking PA increase, with PA indifferent to sleep and waking or with few or no PA. The stability of the sensitivity of the PA to sleep and waking was 84%. Patients with diurnal epilepsy have more frequently myoclonic attacks and a lower seizure frequency. Patients with a sleep PA increase have less frequently generalized motor seizures, more frequently partial complex seizures, a higher seizure frequency, higher total night PA density and more frequently the appearance of new PA during sleep. Patients with a waking PA increase had more frequently massive myoclonus, rarely the appearance of new PA during sleep and a high total night PA density. There is no significant relationship between the two classifications. Neither classification succeeds in discriminating the electro-clinical type of the epilepsies.

Sleep and epilepsy.

This review considers the effect of sleep on seizures and interictal electroencephalogram (EEG) paroxysmal activities (PAs), as classified by the International League Against Epilepsy criteria. No type of seizure is, per se, specifically linked with non-rapid eye movement (NREM) or rapid eye movement (REM) sleep. However, in some syndromes, seizures are more frequent in slow wave sleep (SWS) [partial motor or generalized seizure in benign epilepsy with centro-temporal spikes (BECTS), frontal seizures in idiopathic familial or not familial frontal lobe epilepsy and generalized tonic seizure in secondary generalized epilepsy are increased by SWS]. Conversely myoclonia and grand mal seizures are associated with awakening in some forms of generalized idiopathic epilepsy. There is a mean increase in PAs during SWS in generalized and in partial epilepsies on the whole. However, precise analysis shows that in partial cryptogenic or symptomatic epilepsy and, most likely, in the majority of generalized idiopathic epileptic syndromes about 20% of patients have an increase in PA density during SWS, 20% experience an increase in waking, 50% have very few PAs and in 10% there is no significant difference between sleep and waking. BECTS, however, exhibits a definite increase in sleep PA increase and in juvenile myoclonic epilepsy an increase in PAs during the intra-night awakening is reported. There are at least three syndromes, which cause a huge increase in PAs during sleep: the Landau-Kleffner syndrome and the syndromes of continuous focal or generalized spike-waves during SWS. Their physiopathology and neuropsychological consequences are discussed. Neurophysiological animal data are also reported highlighting the relationships between slow sleep oscillations and the generation of spike waves. A biochemical review is also presented.

Sleep and the epilepsies

To review the numerous works concerning sleep and epilepsy, this review considers the effects of sleep, firstly on seizures and secondly on paroxysmal interictal EEG activity (PA), in the different types of epilepsy according to the International League against Epilepsy classification. Apart from the exceptions of the definite nocturnal preponderance of seizures in idiopathic rolandic epilepsy and of the mostly nocturnal occurrence of seizures in some types of familial or sporadic frontallobe epilepsy, assessing a seizure according to the time of day it occurs is of no diagnostic or predictive value. In generalised idiopathic epilepsy, as in partial symptomatic or cryptogenic epilepsy, only about 20% of the patients had a sleep increase in PA. This percentage is higher (75%) in idiopathic partial epilepsy. Stereoelectroencephalography demonstrates a relative stability of spiking within the fouus across the states of vigilance and an increase in transmitted discharges during stages 3 and 4. In the Landau and Kleffner syndrome, as in the syndromes of continuous spike-waves during sleep, there is a huge, unexplained increase in PA during sleep. The neuropsychological consequences of this PA have some relationship with their localisation and the patient’s age at the time of occurrence. Sleep PA has also been reported in several groups of non-epileptic subjects. As regards the effect of epilepsy on sleep, sleep may be lighter and abnormally discontinuous in the absence of seizures, particularly in temporallobe epilepsy.

A note on the occurrence of unusual electroencephalographic sleep patterns in selected normal children.

Polysomnographic recordings were performed in 50 children free from any familial or personal history of seizure or neurologic diseases to evaluate the frequency of epileptiform and unusual electroencephalographic patterns in a normal population. A 9-year-old boy exhibited focal spikes that became bilateral with a density of 24% to 32% during slow wave sleep, and another boy showed a few spikes during slow wave sleep. In seven cases, 14- and 6-Hz rhythms were recorded, mostly in rapid eye movement sleep. A right rhythmic and temporal discharge was observed in one girl. Epileptiform electroencephalographic patterns are not infrequent, and 14- and 6-Hz rhythms during rapid eye movement sleep are common in normal children. (J Child Neurol 1992;7:422-426).

Atypical language impairment in two siblings: Relationship with electrical status epilepticus during slow wave sleep

We report the case of a young girl who presented severe learning disabilities in oral and written language related to a continuous spike-waves during slow sleep (CSWS) syndrome. A sleep EEG recording obtained in her younger brother, who presented a clinical pattern suggesting developmental dysphasia, also showed a CSWS syndrome. These two clinical cases underscore the need to look for this syndrome in the siblings of an affected child when learning difficulties appear in a child who previously had normal psychomotor development.

The influence of vigilance states on paroxysmal EEG activities and clinical seizures in children

We studied the relationships between clinical variables and those related to the states of vigilance in 18 cases of benign partial epilepsy with centro-temporal spike-waves, 22 cases of definite symptomatic partial epilepsy, and 16 cases of undetermined partial epilepsy. The time of day during which the seizures appeared and the paroxysmal activity densities during non-REM and REM sleep are not distributed differently among the 3 electro-clinical types. However, the benign epilepsy with centro-temporal spikes group had more patients with sleep-sensitive paroxysmal activities. Patients who mainly had nocturnal seizures were found to have more frequent generalized seizures and a greater sleep-sensitive paroxysmal activity. Three cases demonstrated continuous spike-waves during sleep. The patients who had little or no paroxysmal activity during sleep were the youngest. This study illustrates that sleep-sensitive seizures and paroxysmal activities are not specific to benign childhood epilepsy with centro-temporal spikes, and that seizures and paroxysmal activities are two manifestations associated with epilepsy, affected in different ways by states of vigilance.