Seizures in Klinefelter's syndrome: A clinical and EEG study of five patients

In this study, we report the cases of five unrelated patients with Klinefelter's syndrome and seizures or EEG epileptiform abnomalities; the karyotype was 47,XXY in four, and 47,XXY/46,XX in one. They were aged 13–25 years and followed up both clinically and by means of EEG. Two of the patients had epilepsy, one had only one isolated generalized tonic-clonic seizure, one had febrile convulsions and one presented focal epileptiform EEG abnormalities without seizures. In two of the patients, it was possible to classify the epilepsy (childhood epilepsy with occipital paroxysms and cryptogenic or symptomatic generalized epilepsy). Although the electroclinical patterns appeared to be rather heterogeneous in our patients, it is possible to infer the relative good evolution of seizures in Klinefelter's syndrome.

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Sommario In questo studio sono riportati 5 pazienti con sindrome di Klinefelter e crisi epilettiche o anomalie parossistiche all'EEG. In 4 soggetti il cariotipo era 47,XXY mentre in uno esso era 47,XXY/46,XX. L'età dei pazienti era compresa tra 13 e 25 anni; tutti sono stati seguiti nel tempo da un punto di vista clinico ed EEGrafico. Due pazienti erano affetti da epilessia, uno aveva presentato una crisi generalizzata tonico-clonica isolata, uno aveva presentato convulsioni febbrili e uno mostrava solo anomalie parossistiche focali all'EEG senza aver mai apparentemente presentato crisi. In due pazienti è stato possibile classificare l'epilessia (epilessia dell'infanzia a parossismi occipitali ed epilessia generalizzata criptogenetica o sintomatica). Sebbene il pattern elettroclinico nei nostri pazienti sembri piuttosto eterogeneo, è possibile inferire una certa benignità del quadro epilettologico nella sindrome di Klinefelter.

     

Sex differences in interhemispheric correlation and spectral power of EEG activity

EEG activity was recorded from left and right parietal cortex in adult male and female rats on a daily basis during 3 days. Absolute and relative power and interhemispheric correlation of the EEG activity were calculated and compared between males and females. Interhemispheric correlation of the theta rhythm was higher in males than in females. Interhemispheric asymmetry in absolute power was larger in males, who showed higher power in the right than in the left parietal for all bands except for delta. Beta2 relative power was significantly higher in females. These results indicate greater functional asymmetry and higher interhemispheric coupling of the parietal cortex in male than in female rats as assessed by cortical EEG activity.     

Sleep/waking effects following intrathecal administration of the 5-HT1A agonist 8-OH-DPAT alone and in combination with the putative 5-HT1A antagonist NAN-190 in rats

Sleep, waking, and EEG power spectra were investigated in rats after intrathecal (IT) administration of a 5-HT_1A agonist and a 5-HT_1A antagonist. Total slow wave sleep (TSWS) was increased and waking was decreased over the 8-h recording period after the 5-HT_1A agonist 8-hydroxy-2-(di-n-propylamino)tetralin (8-OH-DPAT) (38 nmol). Within TSWS, SWS1 was unchanged while SWS-2 tended to be increased. The 5-HT_1A antagonist 1-[2-Methoxyphenyl)-4-(4-(2-phthalimido)-butyl]piperazine hydrobomide (NAN-190) did not change and sleep/waking stages. Combined treatment with 8-OH-DPAT and NAN-190 increased variance. Following the combination, sleep and waking were not significantly different from control. SWS-2 tended to be reduced compared to the effect of 8-OH-DPAT alone. There were no systematic changes in neither waking nor TSWS fronto-frontal or fronto-parietal EEG power spectrum after any of the treatments, indicating that sleep quality was not changed. The results confirm earlier data suggesting that in the spinal cord, stimulation of 5-HT1A receptors have a dampening effect on transmission of sensory information, leading to deactivation and thereby increased sleep tendency. The reason why the 8-OH-DPAT effect was not clearly antagonized by the putative 5-HT1A antagonist NAN-190, may be due to the generally weak antagonistic and also partial agonistic effect of NAN-190 as reported in the literature.     

Electroclinical and ultrastructural changes associated with subconvulsant doses of pentylenetetrazol.

A series of rats was subjected to subconvulsant and convulsant doses of pentylenetetrazol (PTZ) and the brains were examined electronmicroscopically. After establishing baseline dose effects, rats were chronically implanted bilaterally with cortical bipolar electrodes in frontal and parietal regions. One week postoperatively, they received on either 3 or 4 successive days subconvulsant doses (10 to 20 mg/kg) of PTZ. The following electroclinical evolution was characteristic: Focal spike activity in some of the sampled regions was followed by asymptomatic low-voltage “spike-wave” bursts, which were in turn followed by higher voltage more diffuse “spike-wave” bursts associated with whisker twitching. The animals were killed on the third, fourth, or fifth day when the full clinical picture was present, but before a generalized convulsive seizure had occurred. Electron micrographs showed swollen glial processes around capillaries. No change could be detected in the endothelial basement membrane and the neurons appeared intact. We conclude that ultrastructural changes can be demonstrated prior to overt major seizures and that they are similar to some results in the literature on animals which received convulsant doses. Repeated subconvulsant doses of PTZ also led to a “kindling” effect.     

EEG-behavioral dissociation after morphine-and cyclazocine-like drugs in the dog: further evidence for two opiate receptors.

Previous studies have suggested the presence of multiple opioid receptors. In the chronic spinal dog, the μ receptor was postulated to mediate behavioral indifference, whereas the $\text{k}$ receptor was associated with sedation. The effects of morphine, a μ agonist, were compared with those of Win 35,197-2 (ethylketocyclazocine) and ketocyclazocine, $\text{k}$ agonists, in the unrestrained intact dog. Intravenous administration of morphine (0.5 1.0 and 2.0 mg/kg), Win 35,197-2 (0.05 0.1 and 0.2 mg/kg), and ketocyclazocine (1.6 mg/kg) caused similar dissociation of the EEG and behavior. The dissociation was characterized by high voltage delta EEG synchrony primarily in the parietal cortex which accompanied ataxia and catalepsy in nonsleeping postures. Morphine increased total sleep and slow wave sleep, and lowered temperature and heart and respiratory rates. The $\text{k}$ agonists did not increase total sleep but increased temperature and heart and respiratory rates. Vomiting occurred more often after morphine than after the $\text{k}$ agonists. In certain instances, the effects of morphine were completely antagonized by a low dose of naloxone (30μg/kg), whereas 33 times the dose (1 mg/kg) was required to antagonize the effects of Win 35,197-2. The disparate effects of the μ and $\text{k}$ opioid agonists on behavior, sleep and the EEG and their differences in sensitivity to naloxone antagonism extend and support the concept of multiple opiate receptors in the brain. It was concluded, however, that the sedative effects of the opioids are mediated at the μ receptor.     

Psychiatric assessment of healthy children with various EEG patterns.

A sample of 222 health children, 112 girls and 110 boys, aged 5--16 years, with different types of EEG pattern, were investigated by current child-psychiatric methods. The children were recruited from a large material of children selected in accordance with strict criteria of normality from the paediatric and neurological points of view (Petersén & Eeg-Olofsson (1970/71)). The aim of the child-psychiatric assessment was to find out whether any clinical correlation with the EEG patterns could be shown. First of all, 96 children with some form of EEG deviation were included. Besides these, 126 children with normal EEG were recruited at random from the material. In this selected material significant relations emerged between clinical variables and EEG patterns. Normal EEG showed negative correlations with child-psychiatric variables, while deviating EEG patterns were positively correlated. Many of the correlations disappeared in the oldest age group.     

Simulating the Waking and Sleeping EEG

A novel noise source whose fundamental frequencies are congruent with typical physiological events is presented. Design criteria are given for using it to simulate the waking and sleeping EEG and suggestions for using it as a general random event programmer.     

Aortic aneurysm in a four-year-old child with tuberous sclerosis

We present a case of aortic aneurysm in a four-year-old child complicated with tuberous sclerosis. We used the same general principles as for adult patients and successfully managed our patient. Our methods included the use of isoflurane plus epidural anaesthesia, dopamine to maintain blood pressure, and induced mild hypothermia to reduce brain metabolism and to prevent spinal cord damage during aortic cross-clamping. Intensive monitoring including EEG was beneficial to the anaesthetic management.     

异丙酚诱导期脑电效应

异丙酚是快速，短效催眠药，对中枢神经系统抑制较轻。ＥＥＧ监测是判断异丙酚诱导期麻醉深度敏感而直接的手段。１８例ＡＳＡⅠ－Ⅱ级患者在注入芬太尼３．６μｇ／ｋｇ１０ｍｉｎ后给予异丙酚２ｍｇ／ｋｇ或硫喷妥钠４ｍｇ／ｋｇ于２０ｓ内缓慢静注，诱导期间用ＳＡＮ－ＥＩ２１Ａ７１脑电图监测仪持续描记脑电变化。结果表明，异丙酚与硫喷妥纳均首先产生β高幅兴奋性活动，最大效应期异丙酚无δ波，维持期效应则相似。起效时间，