Conjugal amyotrophic lateral sclerosis: a report on two couples from southern France.

We report two conjugal cases of amyotrophic lateral sclerosis (ALS) occurring between 1977 and 1991 in southern France (Languedoc-Roussillon). Although conjugal ALS may occur by chance, the description of two cases in the same area points to a role of environmental or genetic factors in the etiology of the disease.

[Somatosensory evoked potentials in amyotrophic lateral sclerosis and primary lateral sclerosis]. / Les potentiels évoqués somesthésiques dans la sclérose latérale amyotrophique et la sclérose latérale primaire.

Somatosensory evoked potentials (SEPs) were studied in 21 cases of amyotrophic lateral sclerosis (ALS) and 7 cases of primary lateral sclerosis (PLS). Despite the lack of clinical sensory abnormalities, SEPs showed abnormalities in both diseases: lack or delay of some components. In ALS these abnormalities indicate widespread sensory disturbance. In PLS only, the Brodmann area 4 seems to be affected.

[Electromyographic, histoenzymologic and immunocytochemical aspects of affected muscles in HIV infection. Observations of five cases]. / Aspects électromyographiques, histoenzymologiques et immunocytochimiques dans les atteintes musculaires au cours de l'infection par le VIH. A propos de cinq observations.

In 5 acquired-immunodeficiency syndrome (AIDS) cases without peripheral neuropathy, we observed myogen clinical signs (diffuse amyotrophy of lower limbs or cramps) or an isolated increase in seric enzyme (LDH, CK). EMG abnormalities were observed in all cases: spontaneous activities (fibrillations and positive sharp waves) in 5 cases, myogenic signs in 2 case. Muscular biopsies were normal in 3 cases and showed myopathic changes in 1 case and polymyositis in another case. Antidystrophin and antilaminin antibodies reactions were altered in 1 case. The spontaneous activities together with these modifications could be in favour of a lesion at the membrane level.

Sleep organization and epilepsy.

Sleep is known to facilitate epileptic manifestations but can also protect the sleeper against the recurrence of seizures. This has been demonstrated in studies on sleep deprivation, and is particularly evident in alcoholic epilepsy and matutinal myoclonus epilepsy. Sleep organization in the epileptic patient is permanently altered by frequent awakenings and stage shifts. Nocturnal grand mal and repetitive partial seizures worsen the sleep disorder by reducing total sleep time and decreasing REM percentage by half. The cumulative effect of these sleep disorders may act on day-time vigilance in epileptics, and may even exert an influence on the recurrence of seizures.

[Effects of zopiclone on sleep, daytime somnolence and nocturnal and daytime performance in healthy volunteers]. / Effets de la Zopiclone sur le sommeil, la somnolence diurne et les performances nocturnes et diurnes chez le volontaire sain.

Ten healthy volunteers, aged 20 to 39, underwent 2 adaptation nights and 3 sessions of 2 consecutive experimental nights and days at 1 week intervals. In the 3 sessions, subjects received under double blind conditions either Zopiclone 3.75 mg or 7.5 mg or placebo, according to a latin-square design. On nights 1 and 2 of each session, subjects were continuously polygraphically monitored, except for a 45 min provoked wake episode 135 min after sleep onset on night 2. Sleep continuity and architecture were evaluated during night 1, degree of daytime somnolence during day 1 and residual effects during night 2 (0 h 00) and day 2 (8 h 00 and 12 h 00). Sleep continuity was not modified, except for a reduction of the number of night awakenings. NREM sleep stage 1 was reduced and stage 2 was increased (in duration but not in percentage) with Zopiclone 3.75 and 7.5 mg. NREM sleep stages 3 and 4 were increased with Zopiclone 3.75 mg only. REM sleep was reduced (in percentage only) with Zopiclone 3.75 and 7.5 mg. Daytime somnolence varied according to the time but not with the 3 different conditions. One performance test only (choice reaction time test) showed a significant impairment at 0 h 00 with Zopiclone 7.5 mg. From a subjective point of view, sleep quality was improved and night time awakening was reduced with Zopiclone 7.5 mg.

[Radicular evoked potential. Peroperative recording in lumbar disk hernias]. / Potentiel évoqué radiculaire. Enregistrement per-opératoire dans les hernies discales lombaires.

The evoked compound action potential of lumbar nerve roots after posterior tibial nerve stimulation at the ankle during operation were studied in 10 patients with lumbar disc herniation. The recording needle electrode was inserted into compressed the nerve root before and after excision of disc herniation. The evoked nerve root action potentials consisted of a positive-negative complex. Before excision, this predominant positive complex was recorded in every patient with a mean peak latency of P1 at 16.64 +/- 1.69 msec, and a mean peak to peak amplitude of P1-N1 of 1.94 +/- 0.52 microv. In three cases with migratory disc herniation, the P1-N1 complex was followed by smaller negative component. After excision of disc herniation, the amplitude of the P1-N1 complex decreased significantly with no significant latency modification. The second negative component was not recorded in the three cases with migratory disc herniation. The recording of nerve root evoked potential during operation is of interest for diagnostic purposes. In addition, it allows electrophysiological monitoring of nerve root traumatism during operation procedures.

[Recurrent hypersomnia]. / Les hypersomnies récurrentes.

Recurring hypersomnias are described according to 3 etiological groups: 1) idiopathic - the Kleine Levin syndrome and its clinical variants - 2) organic and 3) psychiatric. The typical form of the Kleine Levin syndrome is remarkable for the association of recurring episodes of sleep, overeating and temporary mental disturbances lasting from a few hours to several days. Its diagnosis is mainly based on clinical data Laboratory investigations have so far failed to document specific features. Emphasis is laid on circumstances at onset and pathological studies which could be in favour of a viral origin. Some clinical aspects and polysomnographic features are reminiscent of endogenous depression. The treatments of hypersomniac episodes based on stimulants are often disappointing. On the other hand, the prevention of the hypersomniac episodes of the Kleine Levin syndrome with lithium carbonate has been successful in several well-documented cases as well as the prevention of the hypersomniac episodes of the menstruation related hypersomnia with ovulatory inhibitors. Organic and psychiatric forms of recurring hypersomnias are not well known. Their clinical features are described and their various possible etiologies indicated.

Normal latency values of early cortical somatosensory evoked potentials in children.

Somatosensory evoked potentials (SEPs) after median and posterior tibial nerve stimulation were studied in 172 children ranging in age from 1 day to 16 years for the purpose of obtaining normal reference values, for use in analysing pathological cases. The mean onset and peak latencies of the N wave after median nerve stimulation and of the P wave after posterior tibial nerve stimulation were calculated for 12 age groups. N and P latencies decreased from birth to 3 years of age, when they reached their minimal values. The latencies then increased with age, the increase being greater for posterior tibial nerve SEPs than for median nerve SEPs. By contrast, the ascending time (the interval between onset and peak latencies) decreased progressively with age from birth to adolescence.

[Organization of sleep in recent temporal lobe epilepsy before and after treatment with carbamazepine]. / Organisation du sommeil dans l'épilepsie récente du lobe temporal avant et après traitement par carbamazépine.

Sleep in epileptic patients with complex partial seizures has been considered as being less well organized than in normal subjects. This study attempted to precise whether this disorganization could be related to the length of the disease and could be improved by carbamazepine treatment. The study was performed in 15 patients with recently diagnosed (less than 3 months) and untreated temporal lobe epilepsy. Neurologic examination and CT scan were normal. Patients did not present a generalized seizure in the preceding 48 hours and during the study. Nocturnal sleep polygraphic recordings were performed: 2 recordings before treatment and 2 others after one month of carbamazepine administration (800 mg/day). Before treatment sleep was characterized by a marked instability (increase in awakenings, shifts in sleep stages, waking after sleep onset, stage 1 duration) when compared to normal subjects. Carbamazepine treatment improved sleep stability. Our data support the hypothesis of another possible mechanism than the occurrence of seizures to explain the disorganization of sleep in temporal lobe epilepsy. On the other hand sleep instability could not be related to the length of the disease since it existed soon after its onset. Carbamazepine treatment improved sleep stability and this improvement could play a role in the therapeutic effect of the drug.

[EEG abnormalities of late-onset epilepsy]. / Anomalies EEG de l'épilepsie tardive.

Interictal EEG abnormalities were evaluated in 111 epileptic patients in which the first seizure occurred after the age of 18 years. Standard day-time EEG tracings performed prior to and after antiepileptic treatment, and all-night recordings were investigated. Before treatment, waking EEG was normal in 54% of patients. The percentage of normal sleep EEG recordings was only 28.6%. Focal EEG abnormalities were found in 39% of day-time recordings and in 48.6% of sleep recordings, while the percentage of generalized epileptic discharges observed was respectively 7 and 22.8. When considering the category of epilepsy and the etiology, it appears that the primary and secondary generalization of epileptic discharges is reduced with age.

HLA-DR2 and narcolepsy.

A positive association between HLA-DR2, DQw1, and narcolepsy was documented in 23 French caucasoid narcoleptic patients, 18 who were heterozygous for DR2 and 5 who were possibly homozygous. An autoimmune mechanism of narcolepsy is proposed with three successive stages, as well as relevant methodology for further investigation. A dominant mode of inheritance of narcolepsy, with an incomplete penetrance, is suggested although not yet evidenced.

Somatosensory evoked potentials to posterior tibial nerve stimulation in children.

The somatosensory evoked potentials (SEPs) to stimulation of the tibial nerve were studied in 88 children ranging in age from 1 day to 16 years. SEPs were not evidenced in 10 out of 44 infants less than 1 year old. In others it was a major positive wave (P) with a variable topographic distribution on the midline. The onset and peak latencies of this P were highly variable in different subjects of the same age or body-size, and in the same subject with the active electrode placed in different locations. The lowest values for latency were in subjects about 3 years old. The ascending time of P was the only parameter strictly correlated with age. The results are compared with SEPs to upper limb stimulation, which are constant and more reliable. These results indicate: that the maturation of the peripheral somatosensory pathway proceeds at a faster rate than that of the central somatosensory pathway; that the maturation of the somatosensory pathway of the upper limb precedes that of the lower limb; and that the ascending time of P is a good index of thalamo-cortical maturation. The clinical utility of these SEPs in pediatrics is discussed.

Daytime sleep characteristics and their relationships with night sleep in the narcoleptic patient.

Thirty-six narcoleptic patients with overwhelming sleep episodes, cataplexy, and sleep onset REM (SOREM) episodes were recorded for 34 continuous hours in the laboratory starting at 2200 h and ending at 0800 h a day and a half later. There were 94 SOREM and 60 sleep onset NREM (SONREM) episodes. While SONREM episodes were evenly distributed across daytime, SOREM episodes peaked between 0800 and 1000, 1200 and 1400, and 1600 and 1800 h. The ratio of SOREM to SONREM episodes was at its highest level between 1200 and 1400 h. Correlation coefficients between night 1 and night 2 for total sleep time (TST) and percentages of sleep stages were all positive and significant, whereas between daytime and each night, they were significant for percentages of stages 1, 2, and REM. Sleep-stage distribution across the last 24 h of continuous recording indicated that although TST levels were higher than that typical of normal subjects, REM sleep and slow wave sleep followed the same circadian distribution as that observed in normal subjects. The results are interpreted as evidence that the daytime sleep of narcoleptic patients is modified, similar to their night sleep, and that SOREM episodes are influenced by a time-of-day effect which culminates between 1200 and 1400 h.

Regional cerebral blood flow during interictal state: differences between temporal lobe epilepsy and primary generalized epilepsy.

Regional cerebral blood flow (rCBF) was investigated during interictal state by 133Xenon intravenous clearance technique in two groups of epileptic patients: group I (n = 28) having partial complex seizures (temporal lobe epilepsy); group II (n = 14) having generalized seizures (primary generalized epilepsy). Mean hemispheric CBF was decreased in group I and unchanged in group II compared to normal controls. Modifications in rCBF pattern were observed in group I but not in group II. A marked hypoperfusion of one temporal region was found in patients from group I showing a unilateral and fixed epileptic focus. Hypoperfusion of other cerebral areas at a distance from the epileptic focus was greater for patients with a left epileptic focus than for those with a right epileptic focus. Patients with the same severity of disease and bilateral and variable foci had no significant modification of mean hemispheric CBF and rCBF distribution. These observations indicate that interictal investigations of rCBF may be of interest for a differential classification of epilepsy.

[Maturation of cerebral somatosensory evoked potentials]. / La maturation des potentiels évoqués somesthésiques cérébraux.

Cerebral somatosensory evoked potentials (SEPs) were elicited by stimulation of the median nerve and/or posterior tibial nerve in 117 children of 1 day to 16 years old. A major negative wave (N) was consistently recorded from the parietal region of the scalp when the arm was stimulated. The peak latency, the onset latency, the rising time and the duration of H wave are closely correlated with age and body length. The latencies are shortest in the subjects of 1-3 years old. SEPs to lower extremity stimulation were inconstant in the infants before the age of one. The major positive wave (P) has a variable topographic distribution along the middle line, over the scalp. The latencies are also very variable in the different subjects of the same age as well as in the same subject with different locations of active electrode. Among the parameters studied as for N wave, only the rising time of P wave is significantly correlated with age. The latencies of P wave have the shortest value in the subjects of 1-3 years old. The comparison of SEPs to upper and to lower limb stimulations shows that there is no relationship between them in respect to their morphology and amplitude. The minimum value of the latencies of N and P waves was observed at the same age but the difference between the peak latencies of P and N waves in the same subject increases considerably after 2 years of age and reaches the adult value after 5 years of age. These resultats indicate that the maturation of the peripheral somatosensory pathways proceeds at a higher rate than that of the central somatosensory pathways, that the maturation of the somatosensory pathways of the upper limb precedes that of the lower limb, and that the rising time of N or P waves is a good index of cortical maturation. The clinical utility of these SEPs in pediatrics is discussed.

[Lumbar cord potentials evoked by stimulation of the nerves of the lower limb in man]. / Potentiels de moelle lombaire évoqués par stimulation des nerfs du membre inférieur chez l'homme.

Lumbar cord potentials evoked by electrical stimulation of the posterior tibial and sural nerves at the ankle were recorded with monopolar epidural electrodes, at T11-T12 level in 20 subjects and were compared with surface recorded potentials. Two quadriplegic patients with spinal section were included in this group. Curare was given in two cases. Xylocaine block of peripheral nerve was carried out in 4 cases. Double shock study was done in 5 cases. The lumbar cord evoked potentials show two successive components: a 'primary' negative-positive spike response with a latency of 19-35 msec, and the 'secondary' waves with latencies up to 200 msec. The 'primary' response is mainly produced by the afferent volley in the fibres of the dorsal roots and of their intramedullary prolongations. There is no evidence which suggests that it is correlated with presynaptic inhibition. The secondary components may be divided into the early and the late waves. The early waves (40-90 msec) are related to the polysynaptic activities from the afferent fibres of small diameters. The late waves are under the influence of supraspinal mechanism and may be related to long-loop reflexes. The clinical implications of these evoked potentials are discussed.

[Narcolepsy]. / La narcolepsie.

First described as a separate entity by Gelineau in 1880 and later considered as a symptom, narcolepsy has eventually been recognized as a disease on clinical and polygraphic grounds. Its prevalence stays between 0.04 and 0.06 percent. Age at onset varies from 5 to 50 with a peak in the second decade. Clinical symptoms include excessive daytime somnolence, overwhelming daytime sleep episodes, attacks of cataplexy, hypnagogic hallucinations, sleep paralysis and disturbed nocturnal sleep; sleep onset REM episodes are the main polygraphic feature. Natural history varies with the different symptoms. Excessive daytime somnolence never subsides completely. Cataplexy may disappear spontaneously. Hypnagogic hallucinations and sleep paralysis are not present in all patients and tend to be more transitory. A positive diagnosis of narcolepsy requires a minimum of one major symptom, daytime sleep episodes or cataplexy, together with documented sleep onset REM episodes. Prolonged polygraphic recordings or multiple sleep latency test are of special interest in difficult cases. Clinical variants can be grouped under three headings, incomplete, symptomatic and associated narcolepsies. The etiology of narcolepsy is not well understood. However the discovery of natural animal models of narcolepsy, mainly dogs, has prompted genetic, pharmacological and biochemical studies. The breeding of narcoleptic canine colonies has led to the evidence of a possible autosomal recessive model of inheritance in some species. Pharmacological and neurochemical analysis has shown an imbalance between monoaminergic and cholinergic mechanism. In man, extensive family studies suggest either a two-threshold multifactorial model of inheritance or a dominant mode of inheritance and immunologic studies have recently shown a strong association between HLA-DR2 and narcolepsy. Assays of CSF biogenic amines suggest a decreased bioavailability of dopamine to explain sleepiness and an imbalance between monoamines and acetylcholine to explain cataplexy. A disturbance of circadian rhythms has not been evidenced in narcoleptics. Treatment is still purely symptomatic. Amphetamines and tricyclic antidepressants have been extensively used. However they are not free of side-effects hence the need for alternative treatments.

[Diminished regional cerebral blood flow during the intercritical period in temporal lobe epilepsy]. / Débits cérébraux régionaux diminués en période intercritique dans l'épilepsie du lobe temporal.

Hemisphere and regional cerebral blood flow (CBF) were determined during interictal periods by intravenous Xenon 133 in 43 patients considered to have "temporal" epilepsy and presenting complex partial attacks with altered consciousness and lateralized EEG anomalies predominant in the temporal region. Brain scans were normal in all cases. Three subgroups were differentiated according to EEG and polygraphic examinations during sleep; temporal epilepsy with left or right EEG anomalies, with asynchronous bilateral EEG anomalies, with alternating labile unilateral EEG anomalies. Measurements of CBF were compared with those of normal subjects (n = 13) of comparable age and with those of epileptic patients with cerebral lesions on CT scan (n = 4). In epileptics with left EEG anomalies CBF was diminished by about 25 p. 100 in the left temporal region and from 15 to 22 p. 100 in other regions of the ipsi- and contralateral hemisphere. In epileptics with right EEG anomalies CBF was diminished by 20 p. 100 in the right temporal region but not on the left. CBF in the third group was comparable to that of normal subjects. In epileptics with abnormal CT scans the reduction in CBF could be correlated with EEG and CT scan findings. Studies were also conducted to determine variations in reactivity to CO2 in the areas with reduced flow, during ictal and interictal periods. Results emphasize the value of CBF measurements for investigation of epileptic foci. The importance of areas of reduced blood flow as a parameter of severity and course is discussed, as well as their pathophysiological significance.

[Brain stem auditory evoked potentials in "alcoholic epilepsy"]. / Potentiels évoqués auditifs du tronc cérébral dans "l'épilepsie éthylique".

BAEPs were studied in a group of subjects suffering from 'alcoholic epilepsy.' Results were then compared with data from normal subjects and chronic alcoholics without epilepsy. The latency for peak V and the inter-peak latencies (I-III, III-V, I-V) were significantly longer in the 'alcoholic epilepsy' group than in the control group. This increase of neural transmission time in the brain-stem auditory pathways was less important in the 'chronic alcoholics without epilepsy' group than in the alcoholic epilepsy group.