Startle epilepsy associated with infantile hemiplegia (SEIH): video-polygraphic features and long-term outcome.

OBJECTIVE: The aim of this study was to investigate the video-polygraphic features and the long-term outcome of epilepsy in two patients with startle epilepsy associated with infantile hemiplegia (SEIH). MATERIAL AND METHODS: Two patients (patient 1: a 14-year-old girl; patient 2: a 17 year-and-half-year-old girl), with hemiparesis and moderate mental retardation, underwent a full clinical and neurophysiological examination with video-polygraphic monitoring and recording of startle-evoked seizures. The follow-up was 9 years from epilepsy onset in patient 1, and 8 years from epilepsy onset in patient 2. RESULTS: Firstly, video-polygraphic recordings of startle-evoked seizures, triggered by unexpected auditory stimuli, showed tonic asymmetrical postures with ictal EEG characterized by an abrupt and diffuse electrodecremental pattern or a seizure discharge predominant over the vertex and anterior regions controlateral to the posturing limbs. Electromyogram recording showed a prevalent involvement of proximal muscles with a concomitant tachycardia and apnoea. In particular, in patient 1 ictal heart rate was high, with persisting tachycardia for 60-120 s after the end of seizures. Secondly, a high seizure frequency persisted throughout the course of the disease, as seizures were medically refractory to all currently available anti-epileptic drugs. CONCLUSIONS: The long-term outcome of epilepsy in SEIH, with constantly high seizure frequency, suggests an early surgical intervention, avoiding years with unsuccessful drug treatments and poor quality of life.

Double blind, controlled study of the efficacy and safety of alpidem in the treatment of anxiety in schizophrenic in-patients.

In this double-blind study, alpidem, a new imidazo-pyridine anxiolytic drug, was compared with placebo in order to test its efficacy and safety in chronic schizophrenic in-patients suffering from anxiety not directly related to the schizophrenic process. Sixty-six patients aged from 18 to 65 entered the trial. They also scored at least 18 on the Hamilton Rating Scale for Anxiety (HRSA) after a seven-day placebo run-in. Improvement in symptoms was evaluated by means of the HRSA, the Brief Psychiatric Rating Scale (BPRS), a Visual Analogue Scale (VAS), and the Clinical Global Impression score (CGI). Thirty-three patients were randomly allocated to alpidem and 33 to placebo. Alpidem was significantly more effective (P less than 0.0001) than placebo in improving HRSA scores (total score and factorial scores for somatic and psychic anxiety), BPRS, and VAS. Considering the results of CGI at 21 day, more patients were moderately to markedly improved on alpidem (30/33) than on placebo (2/33) (P less than 0.0001). The efficacy index, according to CGI, was significantly better (P less than 0.001 at least) for alpidem than for placebo. Side-effects were negligible in both groups.

Brain tumor death risk in mental patients.

Several investigators have previously reported a low cancer mortality in mental patients. They hypothesized there were possibilities of actual exclusion between cancer and psychosis. On the other hand, some Authors warned that the reported association between psychiatric illness and cancer death might vary, depending on the used statistical methods. To clarify this problem, we have examined the incidence of deaths caused by malignant neoplasm, particularly from brain tumor, in the chronic patients of Bisceglie Psychiatric Hospital, evaluating either absolute or relative mortality rate analysis. Our results seem to suggest an increased absolute mortality from malignant brain tumor in mental patients.

[Sleep in chronic chorea patients after therapy with sodium valproate]. / Il sonno di coreici cronici dopo terapia con valproato di sodio.

In previous researches spontaneous nocturnal sleep in chronic chorea showed short total sleep time, prolonged sleep latency, several awakenings, reduction of REM sleep time, decrease in slow waves sleep, strong increase in sleep spindles. Some of these alterations improved after therapy with lithium, haloperidol and lithium, pimozide. Since the concentration of GABA has been found to be reduced in patients with Huntington's chorea, we studied the effect of sodium valproate, a drug that enhances GABA inhibition in cerebral cortex, on nocturnal sleep of six patients with chronic chorea, aged 35 to 60 years (mean 47,3). Nocturnal polygraphic records (EEG, EOG, EMG of chin muscles) were carried out after two consecutive adjustative nights, both before therapy and after sixty days of treatment with sodium valproate (800-2000 mg four times a day, orally). Moreover, chorea, finger dexterity and gait were each rated once a week by three members of the research team and by one independent observer, using a five points rating scale from 0 (normal) to 4 (very severely abnormal). Before therapy the sleep parameters were in accordance with our previous results in chronic choreic patients. After two months therapy we observed a statistically significant (P less than 0.05) reduction of awakenings and of wake time. Sodium valproate produced no objective change in any of the parameters of motor function studied. If singularly examined, however, a reduction of chorea was obtained only in a patient, whose favourable response to therapy was also demonstrated by the normalization of other sleep parameters. These data stress the importance of sleep study in extrapyramidal disorders and suggest a different involvement of GABA-mediated transmission in various patients with chronic chorea.

[Sleep in a case of hemiballism]. / Evoluzione del sonno in un caso di emiballismo.

Sleep spindles abnormalities in extrapyramidal disorders have already been reported, but in the unilateral syndromes the data are unclear and inconclusive. In the attempt to clarify the problem we describe nocturnal sleep in a patient with hemiballism due to an ischemic damage of the subthalamic nucleus. The patient improved after one month haloperidol therapy and her unilateral (right) hyperkinesias disappeared. Electropolygraphic records (EEG, EOG, EMG of chin muscles, ECG) during spontaneous nocturnal sleep were obtained before therapy and after one month haloperidol treatment. The EEGs from bilateral fronto-central derivations were also recorded on magnetic tape to allow spectral analysis and automatic spindle detection by means of digital filtering. The pattern of spindle appearance rate for each stage 2 and 3 NonREM sleep in each night was investigated. We obtained: spindling index, that is the ratio between total spindle duration and sleep stage length, the mean number of spindle bursts/min, the average duration of sleep spindles, the mean amplitude, the mean time between spindles. Before therapy polygraphic records showed short total sleep time, a prolonged sleep latency, the lack of slow waves sleep and of REM sleep. After haloperidol treatment these parameters improved: total sleep time was longer, sleep latency shorter, slow waves sleep and REM sleep appeared. Before treatment spindle activity predominated in the left hemisphere - homolaterally to the subthalamic nucleus damage -, where the spindling index was higher, the mean number of spindle bursts/min larger, the mean time between spindles lower. After therapy, when hemisomatic hyperkinesias disappeared, spindle activity turned similar in the two hemispheres, since it decreased in the left hemisphere and it was unchanged in the right one.(ABSTRACT TRUNCATED AT 250 WORDS)

[Sleep and the circadian rhythm of cortisol in transsexuals]. / Sonno e ritmo circadiano del cortisolo in transessuali.

Polygraphic recordings of nocturnal sleep and hormonal behavior were studied in three male and two female transexual subjects, aged 17 to 26 years, who had required a surgical sex reassignment. The transexual state was assayed by psychological investigations according to the law. All subjects appeared healthy at physical examination and no abnormalities were revealed by basal laboratory data. Chromosomal picture was in accordance with sexual characteristics. Pituitary sella enlargements were excluded by radiographic examination. In each patient two adjustment days were followed by polygraphic recording (EEG,EOG,EMG of chin muscles) of nocturnal sleep and blood drawing for cortisol assay. Blood samples were drawn at 30 minutes intervals for 24 hours, starting from the bedding-time. Hormonal blood concentration were determined by radioimmunoassay. Cosinor method was employed in the analysis of circadian rhythm. In transexual subjects the percentage of sleep intermediate phase, or ambiguous sleep, with reference to total sleep time, was significantly higher than in matched controls.(ABSTRACT TRUNCATED AT 250 WORDS)

[Polygraphic findings in bird-like face syndrome with favorable spontaneous evolution]. / Rilievi poligrafici in "bird-like face syndrome" a evoluzione favorevole spontanea.

The syndrome characterized by acquired micrognathia, hypersomnia and periodic apneas during sleep is a rare consequence of mandibular underdevelopment. The pathogenesis is ascribed to incomplete obstruction of the upper airways associated with a hypoexcitability of the respiratory center. Tracheostomy, with the placement of a permanent tracheal cannula, has proved to be the only treatment producing clinical remission of the syndrome. Polygraphic findings were described in a case spontaneously recovered of hypersomnia and periodic apneas with acquired micrognathia due to a bilateral mastoiditis occurring in early childhood. In this patient three polygraphic recordings were carried out during diurnal and nocturnal sleep; another night sleep was recorded after a spontaneous improvement. In the early three recordings there is a prevalence of light sleep over slow-waves sleep and REM sleep is reduced in nocturnal sleep. There is a lot of periodic apneas during sleep stages. During apneas we observe an increase of heart frequency in NonREM sleep and a decrease in REM sleep. In the recording after clinical recovery we observe an improvement of sleep parameters and a disappearing of apneas in NonREM sleep. A temporary hyposensibility of respiratory centers is considered to be a possible interpretation of clinical and polygraphic improvement.