Postictal psychosis: presymptomatic risk factors and the need for further investigation of genetics and pharmacotherapy

Background  

Postictal psychosis (PIP), an episode of psychosis occurring after a cluster of seizures, is common and may be associated with profound morbidity, including chronic psychosis. Symptoms are often pleomorphic, involving a range of psychotic symptoms, including hallucinations and disorders of thought. PIP is treatable and may be averted if presymptomatic risk factors are considered in susceptible patients and treatment is initiated.     

氟桂嗪治疗Tourette综合征的临床疗效观察

目的探讨氟桂嗪是否有治疗Tourette综合征(多发性抽动秽语综合征)的作用.方法163例患儿分为3组:泰必利组和泰必利加氟桂嗪治疗组以及单用氟桂嗪治疗组,用药和观察时间至少为6个月,用"不自主运动量表(AIMS)"评分法来评定治疗后抽动症状的改善程度.结果66例泰必利组中有24例抽动症状消失,痊愈率为36.36%;66例泰必利加氟桂嗪治疗组中有36例抽动症状消失,痊愈率为54.55%,两者痊愈率相比差异有显著性意义(P＜0.05).31例单用氟桂嗪治疗组中"轻度"患儿的痊愈率达100%,即病情偏轻者效果较佳,病情严重者效果较差.结论氟桂嗪对Tourette综合征确有治疗作用,其药理机制可能与氟桂嗪的抗多巴胺能活性作用有关.     

The genetics of tourette syndrome

Twin and family studies demonstrate that Tourette syndrome (TS) is a genetic disorder. Early segregation analyses of family data were consistent with the hypothesis of autosomal dominant transmission; however, more recent studies suggest that the mode of inheritance is more complex. Current findings suggest that there are genes of major effect with other genes acting as modifiers. Several genome scans have been completed and several regions of interest have been identified that may harbor susceptibility genes for TS. Work is currently underway to replicate and extend these initial results.     

Neurobiology of non-REM sleep in depression: further evidence for hypofrontality and thalamic dysregulation

OBJECTIVE: Sleep disturbances characterize depression and may reflect the abnormal persistence of brain activity from wakefulness into non-REM sleep. The goal of this study was to investigate the functional neuroanatomical correlates of non-REM sleep relative to presleep wakefulness in depressed patients and healthy subjects. METHOD: Twelve medication-free depressed patients and 13 healthy subjects underwent polysomnography and [(18)F]fluorodeoxyglucose positron emission tomography scans during presleep wakefulness and non-REM sleep. Statistical parametric mapping contrasts were performed to detect differences in relative regional cerebral glucose metabolism between presleep wakefulness and non-REM sleep in each group as well as interactions across states and between groups. RESULTS: Relative to healthy subjects, depressed patients showed less of a decrease in relative regional cerebral glucose metabolism from presleep wakefulness to non-REM sleep in the left and right laterodorsal frontal gyri, right medial prefrontal cortex, right superior and middle temporal gyri, insula, right posterior cingulate cortex, lingual gyrus, striate cortex, cerebellar vermis, and left thalamus. CONCLUSIONS: Patterns of relative regional cerebral glucose metabolism changes from presleep wakefulness to non-REM sleep differ in healthy subjects and depressed patients. Specifically, the transition from wakefulness to non-REM sleep was characterized by the relative persistence of elevated metabolic activity in frontoparietal regions and thalamus in depressed patients compared with healthy subjects. These findings suggest that abnormal thalamocortical network function may underlie sleep anomalies and complaints of nonrestorative sleep in depressed patients.     

Effects of propranolol in tourette syndrome

Alternate medications for the treatment of Tourette syndrome are required because haloperidol in some patients either may be ineffective or may cause disturbing side effects. Propranolol, a beta-adrenergic blocking agent, has been reported as effective, in uncontrolled trials, in ameliorating symptoms of tic disorder, tardive dyskinesia, and drug-induced extrapyramidal syndrome. Propranolol, in doses up to 120 mg per day, was administered to five patients with Tourette syndrome in a placebo-controlled study and was found ineffective in ameliorating symptoms of Tourette syndrome. Results underscored the importance of placebo-controlled investigation when evaluating the effects of drugs in Tourette syndrome.     

抽动秽语综合征共患病的横断面调查研究

目的 调查了解抽动秽语综合征（TS）共患病情况。方法 连续入组2013 年6 月— 2015 年 5 月所有在北京安定医院就诊的TS 患儿,并对TS 患儿及家长进行横断面调查研究,由临床医师对患儿 进行一般人口学信息、TS 患病情况及治疗情况等信息进行调查,并进行简明儿童少年神经精神访谈问 卷（MINI-Kid）评估。结果 414 例患儿纳入分析,通过MINI-Kid 再诊断后,共患病率为91.30%（378/414）, 而临床诊断共患病仅为10.39%（43/414）;MINI-Kid 再诊断后,发现TS 共患注意缺陷多动障碍患儿最多, 共308例,占总人数的74.40%（308/414）;其次分别是重性抑郁障碍、恶劣心境、（轻）躁狂以及强迫症等;临 床中TS共患病治疗率低,治疗率最高为共患强迫症状,仅为22.20%（16/72）,其次分别是共患注意缺陷多 动障碍10.06%（31/308）,共患（轻）躁狂发作9.76%（8/82）,共患重性抑郁发作3.98%（8/201）。结论 TS 共患 病情况临床识别率低,治疗率也低。     

Immunopathogenic mechanisms in tourette syndrome: A critical review

Tourette syndrome (TS) has a multifactorial etiology, in which genetic, environmental, immunological and hormonal factors interact to establish vulnerability. This review: (i) summarizes research exploring the exposure of TS patients to immune‐activating environmental factors, and (ii) focuses on recent findings supporting a role of the innate and adaptive immune systems in the pathogenesis of TS and related disorders. A higher exposure prior to disease onset to group A β‐haemolytic streptococcal (GABHS) infections in children with tics and obsessive‐compulsive (OC) symptoms has been documented, although their influence upon the course of disease remains uncertain. Increased activation of immune responses in TS is suggested by changes in gene expression profiles of peripheral immune cells, relative frequency of lymphocyte subpopulations, and synthesis of immune effector molecules. Increased activity of cell‐mediated mechanisms is suggested by the increased expression of genes controlling natural killer and cytotoxic T cells, increased plasma levels of some pro‐inflammatory cytokines which correlate with disease severity, and increased synthesis of antineuronal antibodies. Important methodological differences might account for some inconsistency among results of studies addressing autoantibodies in TS. Finally, a general predisposition to autoimmune responses in TS patients is indicated by the reduced frequency of regulatory T cells, which induce tolerance towards self‐antigens. Although the pathogenic role of immune activation in TS has not been definitively proven, a pathophysiological model is proposed to explain the possible effect of immunity upon dopamine transmission regulation and the generation of tics. © 2009 Movement Disorder Society     

The need for intervention: a further delineation.

Clinical material from experimental tape sessions is used further to delineate a crucial variable in psychotherapy research: the degree to which the therapist meets the patient's need for intervention. Several discrete needs for intervention are identified, how they were (later) met by a therapist is observed, and the outcome of each is assessed.     

难治性抽动秽语综合症的立体定向手术治疗

目的 探讨脑内多靶点毁损术对伴有严重行为障碍的难治性抽动秽语综合症的疗效.方法 对11例严重行为障碍的抽动-秽语综合症患者行CT和微电极导向下单侧或者双侧苍白球腹后部毁损术 双侧内囊前肢、隔区毁损术.在手术前、术后2周、6个月、1年分别应用耶鲁综合抽动严重程度量表(Yale global tic severity scale, YGTSS)进行评分,同时观察术后患者行为障碍的改善情况.结果 术后4例患者抽动症状消失,7例患者的抽动症状明显改善;手术后YGTSS评分较术前明显减少,减分率达77%左右;手术对患者的行为障碍改善明显,术后一年仅3例患者遗留轻度行为障碍.结论 单侧或者双侧苍白球腹后部毁损术 双侧内囊前肢、隔区毁损术对伴有严重行为障碍的难治性抽动秽语综合症患者具有良好的治疗效果.     

Epidemiology of restless legs syndrome: the current status

Epidemiological studies in restless legs syndrome (RLS) have often been limited by misdiagnosis and by the fact that affected individuals, even when their symptoms are severe, might not seek medical care. Some of these limitations have been overcome in the last years as population studies based on face to face interviews have been carried out with new standardized diagnostic criteria. According to these studies, and in contrast to earlier views, RLS has been shown to be a common disorder with prevalences ranging between 2.5 and 10% of the population. Although few studies performed outside Europe/North America have shown a low prevalence, a number of methodological issues have been raised that might question these results. Furthermore, once established, RLS usually follows a chronic course, and preliminary evidence shows that it might worsen over time in some patients. Endstage renal disease, increasing age, female gender, pregnancy, frequent blood donations, iron deficiency and neuropathy are considered to be risk factors for this disorder. The association to RLS is less definitely established for other conditions, such as PD or diabetes. In summary, epidemiological evidence suggests that RLS is a common neurological disorder-with high impact on many aspects of the life of those affected.     

Randomized trial of behavior therapy for adults with tourette syndrome

CONTEXT Tics in Tourette syndrome begin in childhood, peak in early adolescence, and often decrease by early adulthood. However, some adult patients continue to have impairing tics. Medications for tics are often effective but can cause adverse effects. Behavior therapy may offer an alternative but has not been examined in a large-scale controlled trial in adults. OBJECTIVE To test the efficacy of a comprehensive behavioral intervention for tics in adults with Tourette syndrome of at least moderate severity. DESIGN A randomized controlled trial with posttreatment evaluations at 3 and 6 months for positive responders. SETTING Three outpatient research clinics. PATIENTS Patients (N?=?122; 78 males; age range, 16-69 years) with Tourette syndrome or chronic tic disorder were recruited between December 27, 2005, and May 21, 2009. INTERVENTIONS Patients received 8 sessions of comprehensive behavioral intervention for tics or 8 sessions of supportive treatment for 10 weeks. Patients with a positive response were given 3 monthly booster sessions. MAIN OUTCOME MEASURES Total tic score on the Yale Global Tic Severity Scale and the Clinical Global Impression-Improvement scale rated by a clinician masked to treatment assignment. RESULTS Behavior therapy was associated with a significantly greater mean (SD) decrease on the Yale Global Tic Severity Scale (24.0?[6.47] to 17.8?[7.32]) from baseline to end point compared with the control treatment (21.8?[6.59] to 19.3?[7.40]) (P?<?.001; effect size?=?0.57). Twenty-four of 63 patients (38.1%) were rated as much improved or very much improved on the Clinical Global Impression-Improvement scale compared with 4 of 63 (6.4%) in the control group (P?<?.001). Attrition was 13.9%, with no difference across groups. Patients receiving behavior therapy who were available for assessment at 6 months after treatment showed continued benefit. CONCLUSION Comprehensive behavior therapy is a safe and effective intervention for adults with Tourette syndrome. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00231985.     

Neurobiology and neurochemistry of Rett syndrome

The current status of neurobiological and neurochemical research on Rett syndrome is reviewed, and correlations are developed with previously described neurophysiological, neuroimaging, neuropathological, and immunohistochemical changes. We review the abnormalities reported in the biogenic amine neurotransmitters/receptor systems, and of β-phenylethylamine, an endogenous amine synthesized by the decarboxylation of phenylalanine in dopaminergic neurons of the nigrostriatal system. We also discuss the roles of other neurotransmitters, including β-endorphin and substance P, and neurotrophic factors, including nerve growth factors. Recently, DNA mutations in the methyl–CpG binding protein 2, mapped to Xq28, have been identified in some patients with Rett syndrome. The multiple abnormalities in the various neurotransmitters/receptor systems explain the pervasive effects of Rett syndrome.