儿童与成人起病的伴海马硬化的颞叶癫癎对比分析

目的对比分析儿童与成人起病组的伴海马硬化的颞叶癫癎（TLE-HS）患者临床特点的异同,以提高对该病的认识。方法：回顾性收集我院癫癎诊疗中心就诊的TLE-HS患者201例,依据患者起病年龄分成儿童起病组和成人起病组,收集病史、发作类型和频率、药物治疗、头部影像学、24 h视频脑电图（V—EEG）等临床资料,作对比分析。结果：儿童起病组较成人起病组伴热性惊厥（FS）史多、间断发作少,频繁发作多,差异均有统计学意义（P=0．013、0．013、0．013）;儿童起病组较成人起病组在发作间期EEG颞区以外癎样放电较多,差异有统计学意义（P=0．015）。两组患者在HS侧别、发作起源、发作类型、单用或联合用药、头部外伤史、脑炎史、家族史、精神发育迟滞、精神异常等方面比较差异均无明显统计学意义。结论：与成人起病组相比,儿童起病组伴FS史多,发作频率更高,这提示FS是导致癫癎患者更早起病的原因之一;儿童起病组发作间期EEG颞区以外癎样放电较多。     

Emergency EEG and factors associated with nonconvulsive status epilepticus.

The purpose of this study was to identify factors associated with nonconvulsive status epileptics (NCSE) and optimize efficient use of emergency electroencephalography (EEG) services. A retrospective review of medical records identified features associated with NCSE over a 1-year period. Seventy-eight EEGs were identified and divided into four groups. Group I, normal EEG; group II, EEG slowing; group III, epileptiform discharges; and group IV, NCSE. Three risk factors were associated with EEG-confirmed NCSE. These three risk factors are: history of epilepsy, witnessed seizure around the time of presentation, and seizure-like motor activity (SLMA). At least one of these three risk factors was identified in 35 patients, and all of the patients with NCSE were within this group. Forty-three patients had absence of these factors, and none of these patients were found to have NCSE, giving these factors a negative predictive value of 100%. Our study suggests that these three factors are highly associated with NCSE. A prospective study should be done to confirm this finding.     

Adrenal medullary hyperplasia: A clinicopathologic study of four cases

This study presents the clinicopathologic findings in four new cases of adrenal medullary hyperplasia. The patients presented with episodic hypertension frequently associated with palpitations headache, and diaphoresis. All four had elevated urinary catecholamine levels during attacks, and were thought clinically to have a pheochromocytoma. In each case laparotomy revealed a diffusely enlarged adrenal gland without a discrete tumor. Histologic examination of the adrenals demonstrated a diffuse or diffuse and nodular expansion of the medulla confirmed by morphometric study. Of the four patients, three underwent unilateral and one bilateral adrenalectomy. Two patients who underwent unilateral adrenalectomy have been free of symptoms for three years. Thus, it would appear that adrenal medullary hyperplasia may occur unilaterally or asynchronously in the two glands. The bilaterally adrenalectomized patient has had persistent attacks, suggesting that the stimulus to adrenal medullary hyperplasia may possibly affect other chromaffin tissues. On the basis of our cases and a review of the literature, we propose the following criteria for the diagnosis of adrenal medullary hyperplasia: a clinical history of episodic attacks suggesting pheochromocytoma (generally with associated increased urinary catecholamine levels), an adrenal gland showing diffuse expansion of the medulla into the alae or tail of the gland with or without nodule formation, a medulla composed of enlarged cells with or without pleomorphism, and, most important, an increased medulla/cortex ratio, together with an increased calculated medullary weight asddetermined by morphometric analysis.     

The exploding head syndrome: polysomnographic recordings and therapeutic suggestions

Attention has recently been drawn to a condition termed the exploding head syndrome, which is characterized by unpleasant, even terrifying sensations of flashing lights and/or sounds during reported sleep. Nine patients complaining of sensations of explosions in the head during sleep or drowsiness were investigated with polysomnographic recordings. None of them had any neurological disorder. Five patients reported explosions during the recording sessions. According to the recordings, the attacks always took place when the patients were awake and relaxed. In two cases abrupt electroencephalographic (EEG) and electromyographic changes indicating increasing alertness were recorded at the time of the reported attacks. In the remaining three cases no EEG changes were seen. Thus, there were no indications of an epileptic etiology to the condition. In all patients the symptoms ameliorated spontaneously with time. The severity of the symptoms was reduced by reassurance of the harmlessness of the condition. Clomipramine was prescribed to three patients who all reported immediate relief of symptoms. It is concluded that symptoms of this type are probably not true hypnagogic phenomena but may be an expression of emotional stress in the awake state.     

Clinical and quantitative EEG studies of mania.

BACKGROUND: Earlier EEG studies reported essentially normal findings during acute manic episodes but some atypical EEG characteristics and distinctions between familial and sporadic cases were described. Recently quantitative EEG (qEEG) studies differentiating mania from schizophrenia and depression have been published. METHODS: Clinical EEGs were obtained in 202 patients hospitalized for acute mania. EEGs were repeated in 75 patients rehospitalized for subsequent manic attacks. Quantitative EEGs were recorded in 37 patients who were able to cooperate after drug washout and again on completion of randomly assigned pharmacotherapy. RESULTS: Normal EEGs were obtained in most patients. Moderately abnormal EEGs in 16% were significantly associated with absent family histories of affective disorder. Left sided abnormalities were more common than right. "Small sharp spikes" and "microsleep" were encountered in 17% and 10% respectively of patients who drowsed. EEG findings during subsequent episodes did not suggest increasing CNS vulnerability. qEEGs showed significant differences between each of the therapeutic agents compared-lithium, carbamazepine, and lithium combined with carbamazepine, haloperidol or risperidone. Nonresponders at baseline had significantly more diffuse theta activity than responders. During pharmacotherapy nonresponders had higher amplitudes in the left temporoparietal areas. LIMITATION: Clinical EEG findings confirmed previous reports but did not contain original observations. Applications of qEEG were limited by requirements for patient cooperation.     

癫痫发作类型与EEG的相关性

目的：分析各种发作类型癫痫及其与脑电图（EEG）表现的关系。方法：对352例确诊癫痫患者行规范的病史询问及长程EEG监测（描记时间10～15h）,并按照1981年ILAE分类和名词委员会推荐的癫痫发作的分类方案进行发作症状的分类,按照1989年II,AE推荐的癫痫和癫痫综合征的分类作出诊断,分析其发作类型和EEG之间的相关性。结果：在352例中,EEG异常246例,异常率为69．9％,24例（6．8％）记录到临床发作。EEG异常率与发作频率相关,每月发作大于4次者较每月发作少于4次者EEG异常率显著升高（P=0．014）。部分性发作和全面性发作的患者异常率比较差异无显著意义（P=0．929）,但前者更多表现为EEG局限性异常（P=0．014）。结论：规范的长程EEG检查异常率较高,EEG异常率与发作频率相关,不同癫痫发作类型患者的EEG表现有差异。     

Moderate-to-heavy alcohol intake is associated with differences in synchronization of brain activity during rest and mental rehearsal

In alcohol-dependent individuals, synchronization of brain activity is different from that in non-alcohol-dependent individuals as reflected by EEG differences at alpha and beta frequencies (8–30 Hz). These EEG differences may not only be related to long-term alcohol intake but also to genetic factors that are associated with alcohol dependence. Thus, it is not known what the pure effect of long-term alcohol intake on synchronization of brain activity is. Therefore, we investigated whether EEG synchronization differs between light (0.5–6 drinks per week), moderate (7–20 drinks per week), and heavy (21–53 drinks per week) drinkers. All participants (49 males and 47 females) were free of a personal and family history of alcohol dependence. Eyes-closed EEG was recorded at rest and during mental rehearsal of pictures. EEG synchronization was determined by computing Synchronization Likelihood for six frequency bands (0.5–4 Hz, 4–8 Hz, 8–12 Hz, 12–20 Hz, 20–30 Hz, 30–45 Hz). Both male and female heavy drinkers displayed a loss of lateralization in alpha (8–12 Hz) and slow-beta (12–20 Hz) synchronization. In addition, moderately and heavily drinking males had lower fast-beta (20–30 Hz) synchronization than lightly drinking males. It is concluded that both male and female drinkers who drink 21 alcoholic drinks per week or more have impaired synchronization of brain activity during rest and mental rehearsal at alpha and beta frequencies as compared to individuals who drink less. As individuals with a personal or family history of alcohol dependence were excluded, the confounding effects of genetic factors related to alcohol dependence on synchronization of brain activity were minimized.     

Management of acute asthma attacks in general practice.

A prospective study was carried out in a semirural group practice between June 1988 and December 1989 to investigate acute asthmatic attacks treated with nebulized salbutamol. Questionnaires were completed by the attending doctor and by the patient (or his or her parent). Sixty nine episodes, occurring in 52 patients, were recorded during the 18 month study period. The majority of the attacks were managed exclusively in the community, with hospital admission occurring on only three occasions, one patient being admitted twice. A large proportion of the patients had a severe attack of asthma as judged by their previous history. Oral steroids were prescribed in 62.3% of attacks, oral theophyllines in 31.9% and antibiotics in 37.7%; the salbutamol nebulizer was used on more than one occasion during 41.2% of attacks. Significant morbidity was experienced by the patients during the studied attack, with 85% suffering sleep disturbance and two thirds being unable to attend work or school. Only 52.5% of patients were on prophylactic treatment and 37.5% of the patients had discontinued some aspect of their asthma therapy in the three months prior to the attack. Patients' and doctors' views about the cause of the attacks differed widely: patients most commonly cited infection (26/41) and allergy (8/41) with only two patients citing poor compliance or inadequate treatment. Although doctors also attributed the cause of many attacks to infection (33/64), they cited poor compliance or inadequate treatment in 28 of 64 responses.(ABSTRACT TRUNCATED AT 250 WORDS)     

SADE3: an effective system for automated detection of epileptiform events in long-term EEG based on context information

Interictal spike detection is a time-consuming, low-efficiency task, but is important to epilepsy diagnosis. Automated systems reported to date usually have their practical efficacy compromised by elevated rates of false-positive detections per minute, which are caused mainly by the influence of artifacts (such as noise activity and ocular movements) and by the adoption of single or simple approaches. This work describes the development of a hybrid system for automatic detection of spikes in long-term electroencephalogram (EEG), named System for Automatic Detection of Epileptiform Events in EEG (SADE(3)), which uses wavelet transform, neural networks and artificial intelligence procedures to recognize epileptic and to reject non-epileptic activity. The system's pre-processing stage filters the EEG epochs with the Coiflet wavelet function, which showed the closest correlation to epileptogenic (EPG) activity, in opposition to some other wavelet functions that did not correlate with these events. In contrast to current attempts using continuous wavelet transform, we chose to work with fast wavelet transform to reduce processing time and data volume. Detail components at appropriate decomposition levels were used to accentuate spikes, sharp waves, high-frequency noise activity and ocular artifacts. These four detailed components were used to train four specialized neural networks, designed to detect and classify the EPG and non-EPG events. An expert module analyzes the networks' outputs, together with multichannel and context information and concludes the detection. The system was evaluated with 126,000 EEG epochs, obtained from seven different patients during long-term monitoring, under diverse behavior and mental states. More than 6,721 spikes and sharp waves were previously identified by three experienced human electroencephalographers. In these tests, the SADE(3) system simultaneously achieved 70.9% sensitivity, 99.9% specificity and a rate of 0.13 false-positives per minute, indicating its usefulness and low vulnerability to artifact influence. After tests, the SADE(3) system showed itself to be able to process bipolar cortical EEG records, from long-term monitoring, up to 32 channels, without any data preparation or event positioning. At the same time, SADE(3) revealed a high capacity to reject non-epileptic paroxysms, robustness in relation to a variety of spike morphologies, flexibility in adjustment of performance rates and the capacity to actually save time during EEG reading. Furthermore, it can be adapted to other applications for pattern recognition, with simple adjustments.     

Smokers' polycythemia

Twenty-two smokers with elevated hematocrits (mean, 54 per cent) had elevated blood carboxyhemoglobin (mean, 11.6 per cent; normal, less than 1 per cent) and a "left-shifted" oxygen-hemoglobin dissociation curve (mean P50, 21.6 +/- 2.3 [+/- S.D.] torr; normal, 26.7 +/- 1.1). Red-cell volume was increased in 14 of 18, and plasma volume reduced in 14 of 18. Fatigue and headache were common, and syncopal attacks occurred in four patients. Symptoms disappeared and the elevated red-cell volume decreased markedly in all five patients who were able to reduce severely their smoking habit; low plasma volume increased in three of four patients studied. We conclude that carbon monoxide exposure from cigar and cigarette smoke is a frequent cause of an elevated red-cell volume or a reduced plasma volume (or both). Measurement of carboxyhemoglobin should be a routine part of the evaluation of all polycythemic subjects.     

Narcolepsy with Concomitant Features of Obstructive Sleep Apnea

A 17-year-old man presented with daytime sleepiness, episodic attacks of sleep and probable cataplexy. His EEG showed rapid eye movements and central sawtooth waves at sleep onset, and supported the clinical impression of narcolepsy. He improved with methylphenidate but died suddenly, and had cardiomegaly, right ventricular enlargement, and pulmonary hypertension at autopsy. These findings suggested concomitant features of sleep apnea which were not evident by history or examination.     

Frequency of fainting,vomiting and incontinence in panic disorder: A descriptive study

Little is known about how often individuals with panic disorder (PD) actually experience the physical catastrophes that they fear (e.g., fainting, vomiting, incontinence). This study investigated the frequency of such events in PD and the circumstances under which they occur. A systematic chart review of 574 consecutive patients diagnosed with PD was completed. Nineteen out of 35 patients who had previously reported a history of fainting, vomiting or incontinence (i.e., loss of bowel or bladder control) during a panic attack were interviewed by telephone. Of this sample, 11 confirmed a history of fainting, four confirmed a history of vomiting and four confirmed a history of incontinence during at least one previous panic attack. Four of these individuals reported more than one type of event and several others reported that they had only come close to experiencing these feared events. Participants also reported on the timing of these events (relative to their panic attacks), the circumstances under which the events occurred, as well as possible causes and outcomes of the events. Although rare, catastrophic events such as fainting, vomiting and incontinence appear to occur in the context of panic attacks for some individuals. The implications for cognitive behavioural treatment of PD are discussed. Copyright © 2007 John Wiley & Sons, Ltd.     

Impulsive artistic creativity as a presentation of transient cognitive alterations

A 27-year-old right-handed male mosaic artisan who had not shown any interest in drawing or artistic activity was admitted to the Department of Neurology for attacks of bizarre behavior and convulsive disorder. The patient reported feeling "waves" engulfing him during the attacks, leaving him floating helplessly. During some attacks, the patient impulsively initiated drawing activity. Interictal sleep-deprivation EEG showed a left frontotemporal focus of paroxysmal discharge. Brain tomography with SPECT showed low 99Tc-HMPAO uptake in the left frontoparietal region. Psychodiagnostic tests gave evidence of dysfunction of the left frontal region, with preference of the right hemisphere. It is suggested that the attacks of altered cognitive state were, in this case, provoked by spreading depression of the left hemisphere, while the integrative functions of the right hemisphere remained intact. Thus, the impulsive artistic creativity during the attacks may represent a "release phenomenon" of the complex visuospatial skills of the right (subdominant) hemisphere. This symptomatology of transient cognitive alterations is unique and, to the best of our knowledge, has not been previously reported.     

Nocturnal sleep panic and depression: relationship to subjective sleep in panic disorder

BackgroundPatients with panic disorder (PD) often complain of sleep disturbances. PD patients have high co-morbid depression and almost 65–70% reports a history of nocturnal panic attacks. It is possible that both nocturnal-sleep panic attacks and depression contribute to sleep disturbances in PD patients. However, the individual and interactive effects of nocturnal-sleep panic attacks and lifetime depression on subjective sleep in PD are unknown.MethodsThe National Institute of Mental Health Panic Disorder Questionnaire (NIMH-PQ) was administered to 773 individuals who met DSM-IV criteria for PD. All of these subjects completed queries related to nocturnal-sleep panic attacks, lifetime depression, difficulty sleeping, and sleep duration.ResultsWe examined difficulty in sleeping and sleep duration in four subgroups [PD without nocturnal panic attacks or lifetime depression (NP?D?), PD with nocturnal panic attacks (NP+D?), PD with lifetime depression (NP?D+), and PD with both nocturnal panic attacks and lifetime depression (NP+D+)]. Significantly greater proportions of NP+D+ subjects reported difficulty sleeping compared to other three subgroups. In addition, the NP+D+ patients reported significantly decreased subjective sleep durations compared to the other three subgroups. Using ≤ 5h as a criteria for severe sleep restriction, approximately 20% of the NP+D+ patients, compared to 9.2%, 9.6%, and 2.5% in the NP+D?, NP?D+, NP?D? subgroups, respectively, reported sleeping 5h or less. 8.2% of panic disorder patients reported excessive sleeping per sleeping period.ConclusionsA high percentage of panic disorder individuals report subjective sleep disturbances. Not surprisingly, an unusually high prevalence of patients with nocturnal panic attacks or depression have sleep problems and 92.3% of patients with both nocturnal panic attacks and depression report striking extremes in sleep duration or insomnia. Thus, nocturnal-sleep panic attacks and depression are independently as well as interactively associated with increased sleep disturbances in panic disorder. Although these findings are expected, they underscore the importance of assessing sleep functions, including over-sleeping, in panic disorder patients.     

Relation between serum eosinophil cationic protein (ECP) level and asthma attack in children]

Serum eosinophil cationic protein (sECP) levels were measured in 339 patients with childhood asthma, and the clinical courses of these patients were followed for 57 weeks. While considering the history and characteristics of each patient, we examined the correlation between asthma attack frequency and sECP, blood eosinophil count, and serum total IgE (tIgE) to determine their usefulness in predicting asthma attacks. Among patients with no other allergic diseases, sECP levels in patients who had no asthma attacks two weeks before or after the measurement were significantly lower than those of patients who had attacks during the same four-week period. Among patients who had attacks, those patients with no attack for a year after the measurement were also found to have low sECP levels. Similarly, even among patients with asthma attacks and high sECP levels, there were cases where attacks were well controlled using nebulizer treatments with DSCG or BDP. The incident rate of attacks for patients with other allergic diseases and a low sECP was low. Yet, there was no common trend in patients with high sECP levels. Moreover, this study detected a significant correlation between sECP level and blood eosinophil count as well as between sECP level and serum tIgE. The most significant correlation with asthma attack frequency was sECP level. Thus, sECP level seems to reflect the allergy activity level, especially two weeks prior to and after the measurement. For patients without other allergic diseases, asthma attack prediction during the two weeks period after the measurement of sECP also seems possible. Therefore, periodic measurement of sECP level is useful in objectively monitoring the improvement of symptoms and establishing the treatment plan, including treatment with DSCG or BDP.     

The natural history and clinical characteristics of aspirin-exacerbated respiratory disease.

BACKGROUND: Aspirin-exacerbated respiratory disease (AERD) is a clinical syndrome characterized by chronic rhinitis, nasal polyps, asthma, and precipitation of asthma and rhinitis attacks after ingestion of aspirin (ASA) and most nonsteroidal anti-inflammatory drugs (NSAIDs). Most information about the disease in the United States has come from small samples of patients. OBJECTIVE: The purpose of this study was to examine the natural history and clinical characteristics of 300 AERD patients, referred to our institution for aspirin desensitization. METHODS: All potential AERD patients were evaluated using a standard questionnaire that included information about clinical characteristics and natural progression of their disease, previous history of reactions to ASA and other NSAIDs, current use of medications, and ethnic backgrounds. All patients underwent oral ASA challenges to prove they had AERD. RESULTS: From patients' history we found that the average age at onset of AERD was 34 years, and that 57% were female. Counting ASA as an NSAID, 33% had previously reacted on two occasions to NSAIDs and 36% on more than three occasions to NSAIDs, whereas only 27% had reacted to one NSAID before they came to us for evaluation. Our patients had averaged 5.5 episodes of sinusitis per year. There were no significant differences in the clinical characteristics or use of medications between genders. Ethnicity was heterogeneous in most participants. CONCLUSIONS: AERD begins in the third decade of life and in both sexes. The disease progressed over the 13 years between historical onset and current evaluation, with more sinusitis and need for controller medications over time. There was no ethnic or familial distribution of AERD.     

发作性运动诱发性运动障碍患者的脑电图改变

目的：观察发作性运动诱发性运动障碍(PKMD)的脑电图改变。方法：详细观察PKMD41例的临床特征，全部进行了常规脑电记录，21例进行Holter脑电长时间监测，在脑电图监测下8例作运动诱发试验。结果：11例有自发痫样放电，1例美解眠诱发阳性。5例临床发作时2例有癫痫样放电，8例运动诱发试验皆阴性。用具有Na^ 通道阻断作用的多种抗痫药治疗均有效。结论：PKMD部分患者在发作期和发作间期有痫样放电，具有癫痫的某些性质，与癫痫可能有共同的生物学基础。     

Acetazolamide-responsive hereditary paroxysmal ataxia: report of a family.

Hereditary paroxysmal ataxia is a rare dominantly inherited disorder characterized by recurrent attacks of cerebellar ataxia, dysarthria, and nystagmus. Each attack lasts from several minutes to few hours or days. Usually there are no motor difficulties between attacks. We report a patient who had had recurrent ataxic episodes since early childhood. Four members of the family over two generations had similar attacks. There were no abnormalities in the laboratory studies including plasma amino acid, lactate, pyruvate, and EEG. Treatment with acetazolamide resulted in complete abolition of the attacks. Because of its dramatic response to acetazolamide, the recognition of this rare disorder is important.