Short-term triazolam use improves nocturnal sleep of narcoleptics.

This study was undertaken to determine whether the use of triazolam by narcoleptic patients leads to improvement of nighttime sleep or excessive sleepiness. Ten narcoleptic patients, 5 males and 5 females, with complaints of sleep disturbance and aged between 18 and 60 years, were assigned to a single-blind within-subject crossover-designed study comparing placebo with 0.25 mg triazolam. All subjects completed sleep questionnaires and underwent 6 nights of polysomnographic testing. Following an adaptation night, subjects received either triazolam or placebo for 2 nights. Objective tests of sleepiness (multiple sleep latency testing/maintenance of wakefulness test) were performed. Sleep efficiency and overall sleep quality were improved on all triazolam nights. Daytime excessive sleepiness was not reduced objectively after triazolam. This study demonstrates that the short-term use of triazolam improves nocturnal sleep quality in narcoleptics. Studies of long-term administration of triazolam are required to determine if the improvement of nocturnal sleep is maintained.

Sleep-related laryngospasm.

Seven patients (mean age 46.6; range 33-58; 6M,1F) presented with sleep-related choking episodes and were found to have features in common that distinguished them from other known causes of choking episodes during sleep. The characteristic features include: an awakening from sleep with an acute choking sensation, stridor, panic, tachycardia, short duration of episode (less than 60 seconds), infrequent episodes (typically less than 1 per month), and absence of any known etiology. The disorder most commonly occurs in middle-aged males who are otherwise healthy. In one patient an episode of laryngospasm was polysomnographically documented to occur during stage 3. The clinical features and the polysomnographic findings suggest spasm of the vocal cords of unknown etiology.

Shy-Drager syndrome: a case report with polysomnography.

The authors report a case of Shy-Drager syndrome in a 53 year-old male patient. Autonomic failure was made evident by physical examination as well as laboratory tests. A sleep recording showed decreased percentage of REM sleep and apneas of the central type. The possible mechanisms for this sleep disorder are discussed.

Eating epilepsy.

Eating epilepsy is a rare type of reflex epilepsy. A 24 years-old male with eating reflex complex partial seizures was submitted to clinical, neurological, neuroradiological and EEG studies. Neurologic and CT examinations were normal. EEG recordings including video-EEG monitoring during meals disclosed focal abnormalities related to both temporal lobes prevailing at the left side and secondary bilateral synchrony mainly in more anterior regions. Ictal findings were similar to the interictal secondary bilateral synchrony except for its longer duration. PB, VPA and DPH monotherapies were ineffective. High dose CBZ monotherapy yielded good but incomplete seizure control. Since a big number of precipitants could be involved, no specific physiopathological basis could be established.

Non-epileptic myoclonus and mitochondrial encephalomyopathy.

Two brothers presented to us with a progressive myoclonic syndrome with slight cerebellar symptoms. Neurological examination disclosed moderate cerebellar signs and pale optic discs; asymmetric, asynchronous and arrhythmic myoclonus, an arrthesthesic deficit and no muscular weakness. EEG background activity was moderately slow with no irritative discharges. CT was normal in both cases. Intermittent photic stimulation increased the frequency of the myoclonic jerks, which became bilateral and synchronous, progressing to a generalized tonic-clonic seizure. EPs and MRI in one case were normal. Anticonvulsant drugs were ineffective. The diagnosis of mitochondrial encephalomyopathy was based on the finding, in muscle specimens, of thickened basement membranes with myofibrillary degeneration and increased number of mitochondria peripherally distributed and with a dense granular matrix and some vacuoles. The clinical and EEG data suggest a subcortical origin for this type of myoclonic syndrome.

Eating epilepsy

A epilepsia reflexa ao comer é uma rara de epilepsia reflexa. Um paciente de 24 anos com crises parciais complexas reflexas ao comer foi submetido a avaliaçöes clínica, neurológica neurorradiológica e eletrencefalográfica. O exame neurológico e a tomografia de crânio foram normais. Registros de EEG, incluindo menitorizaçäo vídeo-EEG durante a refeiçäo, mostraram presença de anormalidades focais relacionadas a ambos os lobos temporais, predominando à esquerda, e sincronia bilateral secundária, predominando em regiöes anteriores. Os achados ictais säo semelhantes à sincronia bilateral secundária interictal exceto por sua maior duraçäo. Monoterapias com PB, DPH e VPA näo surtiram efeito. Monoterapia com altas doses de CBZ trouxe bons resultados porém com controle incompleto das crises. Como grande número de fatores precipitantes estava potencialmente envolvido, näo foi possível determinar com precisäo a base fisiopatológica das crises reflexas neste caso

Non-epileptic myclonus and mitochondrial encephalomyopathy

Foram examinados dois irmäos portadores de uma síndrome miclónica progressiva comn discretos sintomas cerebelares. O exame neurológico mostrava sinais cerebelares moderados e papilas pálidas; mioclonais assíncronas, arrítmicas e assimétricas, um déficit artrestésico e ausência de fraqueza muscular. A atividade de base do EEG era moderadamente lenta e sem atividade irritativa. A TC era normal em ambos os casos. A estimulaçäo fótica intemitente aumentava a freqüência dos abalos mioclônicos que se tornavam bilaterais e sincronos, progredindo para uma crise tônico-clônica generalizada. Potenciais evocados e RMN em um caso foram normais . Drogas anticonvulsivantes foram ineficazes no controle das mioclonias. O diagníostico de encefalomiopatia mitocondrial foi realizado através do achado em espécimes musculares de membranas basais espessadas, com degeneraçäo miofibrilar e um número elevado de mitocondrias distribuídos perifericamente e com uma matriz densa, granular e com alguns vacúolos. Os achados clínicos e eletrográficos sugerem uma origem subcortical para esta síndrome mioclônica