颅咽管瘤术后肺动脉栓塞的研究

目的研究颅咽管瘤术后肺动脉栓塞的发生情况及可能原因。方法回顾性分析185例颅咽管瘤病人的临床资料,其中发生肺动脉栓塞5例(2.7%),经CT肺动脉造影证实4例。对5例病人的血电解质水平、去氨加压素及地塞米松的使用进行研究。结果随访5例,时间6~30个月,死亡3例,痊愈2例。3例病人血钠持续高于正常值(145~170 mmol/L)。5例病人均至少使用1个月的去氨加压素及7 d的地塞米松。结论高钠血症、长期使用去氨加压素和地塞米松可能诱发血液高凝状态而增加病人术后发生肺动脉栓塞的风险。     

Recurrent sleep attacks associated with a craniopharyngioma

Symptomatic narcolepsy, once regarded as common, is now believed to be very rare. A 32-year-old man had a history of recurrent sleep attacks. A magnetic resonance imaging scan revealed a third ventricle tumor. The tumor was totally removed, and the histology was a craniopharyngioma. The symptoms ceased after the operation. The chronological correlation and the anatomical location of the tumor suggest that the patient had a symptomatic narcolepsy caused by the tumor. This is the first report that documents the cessation of narcolepsy attacks after tumor removal.     

颅咽管瘤复发次数与术后生活质量的关系

目的探讨颅咽管瘤复发次数与术后病人生活质量的关系。方法回顾性分析39例复发颅咽管瘤病人的临床资料与随访结果。病人术后生活质量按Karnofsky生活质量评分法评估,分为:能工作或上学、生活能自理、生活不能自理、死亡。结果肿瘤全切除33例(84.6%),次全切除6例(15.4%)。围手术期死亡2例(5.1%)。术后平均随访15.5个月,随访期第1次复发者与第2次复发者生活质量无明显差异;第3次、第4次复发者术后生活质量明显恶化。结论第3次和第4次复发颅咽管瘤的治疗应以改善病人生活质量为主,不宜强求根治性切除肿瘤。     

颅咽管瘤全切除术后血钠紊乱分析及治疗

目的总结颅咽管瘤全切除术后血钠紊乱的发生规律和治疗方法。方法回顾性分析46例颅咽管瘤病人术后血钠紊乱的情况,分析血钠紊乱与病人年龄、肿瘤位置的关系。结果术后出现血钠紊乱者42例(91.3%),其中单纯低钠型9例(19.6%),单纯高钠型6例(13.0%),混合型血钠紊乱27例(58.7%)。42例术后血钠紊乱者中,术后3个月内恢复正常40例;1例持续高钠血症者在术后1.5年恢复正常;另1例持续高钠血症者术后2个月死于肺栓塞。15例儿童病人中7例在围手术期出现癫疒间发作,31例成年病人无一例出现癫疒间发作。结论颅咽管瘤全切除后血钠紊乱的发生率显著高于术前(P<0.001);术后血钠紊乱的类型与年龄、肿瘤复发、肿瘤囊实性和肿瘤位置无关(P>0.05)。纠正血钠紊乱是儿童颅咽管瘤病人在围手术期预防癫疒间发作的关键。     

Stability of estimated IQ across mood state in patients with bipolar disorder

OBJECTIVES: The goal of this study was to assess the impact of mania symptoms on the stability of the American version of the National Adult Reading Test (ANART), a measure that provides a putative estimate of premorbid intellectual functioning. METHODS: The ANART was administered to a sample of 10 inpatients with bipolar disorder who were experiencing an acute episode of mania. During a subsequent follow-up visit during which these individuals exhibited at most minimal mood symptoms, the ANART was re-administered. RESULTS: ANART estimated intelligence quotient (IQ) scores during mania and affective stability were assessed in the 10 patients using a paired sample t-test. Within-subject performance across the two mood states did not significantly differ [t(9) = -1.24, p = 0.25]. Additionally, the greatest individual difference across mood states was only 3.68 points, and the average estimated IQ difference across mood states was 1.66. CONCLUSIONS: While the results are preliminary and based on a small sample, the highly consistent scores achieved across mood states in this study suggest that the ANART is a reliable indicator of premorbid IQ.     

Oxytocin release deficit and social cognition in craniopharyngioma patients

Oxytocin is a neuropeptide known to affect social behaviour and cognition. Craniopharyngioma patients are considered to have an oxytocin‐release‐deficit caused by a rare tumour affecting the pituitary and/or the hypothalamus relevant for oxytocin production and release. To assess social behaviour and socio‐cognitive abilities in this patient group, we tested 13 patients and 23 healthy controls on self‐report questionnaires and an eye‐tracking paradigm including fast facial emotion recognition. Additionally, saliva oxytocin levels acquired before and after a physical stress induction were available from a previous study, representing the reactivity of the oxytocin system. The data revealed three major results. First, patients with an oxytocin‐release‐deficit scored higher on self‐reported autistic traits and reduced levels of hedonia for social encounters, although they showed no impairments in attributing mental states. Second, patients showed more difficulties in the fast emotion recognition task. Third, although automatic gaze behaviour during emotion recognition did not differ between groups, gaze behaviour was related to the reactivity of the oxytocin system across all participants. Taken together, these findings demonstrate the importance of investigating the reactivity of the oxytocin system and its relationship with social cognition. Our findings suggest that reduced emotional processing abilities may represent a pathological feature in a group of craniopharyngioma patients, indicating that this patient group might benefit from specific treatments within the social domain.     

囊性、囊实性颅咽管瘤的立体定向囊内放疗

目的研究CT、MRI引导立体定向囊内放射治疗囊性和囊实性颅咽管瘤的疗效。方法对100例囊性和囊实性颅咽管瘤的囊性部分行CT、MRI引导立体定向囊腔内置入Ommaya,吸除囊液、注入胶体磷酸铬,对囊实性颅咽管瘤的实质部分行伽玛刀治疗。结果全部病例经手术排出囊液后临床症状迅速改善,经囊腔内放疗后6～84个月随访90例病人,CT及MRI扫描显示42例瘤腔持续消失,临床症状消失,恢复正常工作和学习;18例肿瘤显著缩小,症状持续改善;20例肿瘤无明显改变;复发10例,其中再手术6例;死亡7例。结论CT、MRI引导立体定向放射治疗囊性颅咽管瘤安全、有效。     

Adverse behavioral response to clonazepam as a function of Verbal IQ-Performance IQ discrepancy

Eight of 38 consecutive patients who received clonazepam had significant behavioral side effects. In these 8 patients, the mean absolute IQ discordance between Verbal IQ (VIQ) and Performance IQ (PIQ) was 17.5 points. Thirty of 38 patients had no adverse behavioral side effects while receiving clonazepam. In these 30 patients, the mean absolute VIQ-PIQ discrepancy was 6.5 points. Full neuropsychological testing had been performed on all patients. The only test variable showing a statistically significant difference was the absolute discrepancy between VIQ and PIQ (P less than 0.0001).     

复发性颅咽管瘤的临床特点及显微外科手术治疗

目的 分析复发性颅咽管瘤的临床特点，探讨其治疗方法。方法 回顾性分析了6例复发性颅咽管瘤的临床表现、影像学资料、治疗方法及治疗结果。结果 复发颅咽管瘤手术困难，易损伤周围结构。病人术前情况较差，术后并发症较多且严重。复发与首次手术切除的程度明显相关。结论 颅咽管瘤应争取在第一次手术时采用合适的入路进行显微手术全切除。再次手术时，除选择合适的入路外，应特别注意重要血管、神经的显露和保护。围手术期的仔细观察和正确处理非常重要。     

颅咽管瘤全切除术及术后并发症的防治

目的 探讨颅咽管瘤全切除术的手术方法和并发症的防治措施。方法 24例鞍区颅咽管瘤，术前进行糖皮质激素的替代疗法和抗癫痫药物治疗。手术采取翼点人路，从鞍区各个手术间隙分块切除肿瘤。术后严密观测病人的意识、尿量和血清电解质，及时防治尿崩症、电解质紊乱等并发症。结果 22例达到全切，2例有少部分残余，无明显神经功能障碍。发生尿崩症16例，电解质紊乱12例，体温失衡6例。经过处理，电解质紊乱和体温失调完全纠正，13例尿崩症痊愈，3例缓解。结论 选用合适的手术入路，熟悉鞍区的各个手术间隙解剖是完成颅咽管瘤全切除术的关键。术前进行糖皮质激素替代治疗，术后严密监测尿量和血电解质，积极处理并发症，可以有效降低手术病死率。     

Neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy

We characterized the neuropsychological status of children with newly diagnosed idiopathic childhood epilepsy and measured differences in IQ between children with different types of epilepsy. The Korean Education Development Institute-Wechsler Intelligence Scale for Children (KEDI-WISC) was administered to 72 patients (35 males and 37 females), of mean age 8.7 ± 2.6 years, with newly diagnosed idiopathic childhood epilepsy. Of these patients, 22 (30.6%) had generalized epilepsy, 48 (66.7%) localization-related epilepsy, and 2 (2.8%) mixed epilepsy. Children with generalized epilepsy and benign childhood epilepsy with centro-temporal spikes (BCECTS) were of similar verbal IQ and full-scale IQ, although performance IQ was significantly lower in patients with generalized epilepsy. Among children with BCECTS, those with unilateral spikes had higher full-scale and performance IQ scores than those with bilateral spikes. Follow-up studies on large numbers of patients are needed to determine the effects of epilepsy per se, and antiepileptic drugs, on intelligence.     

The relationship between normal variation in IQ and common childhood psychopathology: A clinical study

The relationship between normal variation in IQ and common psychopathology was examined in a sample of 339 5- to 16-year-olds who were seen at a tertiary psychiatric clinic. The mean IQ was 9.6 points lower (95% CI 5.5 to 13.6 points lower) for conduct than for emotional disorders, with mixed disorders in between. For these common disorders, the mean IQ was 6.0 points lower (95% CI 1.6 to 10.3 points lower) for females than males. IQ variation in the normal range was inversely related to a dimensional measure of conduct problems — an association that was not attributable to social class or mediated by scholastic attainments. Other dimensional measures of psychopathology — covering emotional symptoms, developmental immaturity and relationship difficulties — were not significantly correlated with IQ. Limitations of the study are discussed in the paper.

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Zusammenfassung Die Beziehung zwischen der normalen Variabilität des IQ und häufiger Psychopathologie wurde an einer Stichprobe von 339 5- bis 16-Jährigen untersucht, die sich an einer spezialisierten psychiatrischen Einrichtung vorstellten. Der Durchschnitts-IQ war um 9,6 Punkte niedriger für Störungen des Sozialverhaltens als für emotionale Störungen (95%iges CI 5,5 bis 13,6 Punkten niedriger).Die gemischten Störungen lagen dazwischen. Für diese häufigen Störungen lag der Durchschnitts-IQ um 6,0 Punkte niedriger bei den Mädchen als bei den Knaben (95% iges CI bei 1,6 bis 10,3 Punkte niedriger). Die IQ-Variabilität im Normbereich war umgekehrt proportional zur dimensionalen Messung der sozialen Verhaltensauflälligkeiten -eine Assoziation, die nicht der sozialen Schichtgehörigkeit zugeschrieben werden konnte und auch nicht abhängig von schulischen Erfolgen war. Andere dimensionale Messungen der Psychopathologie, die emotionale Symptome, Reifungsverzögerungen und Beziehungsschwierigkeiten umfaßten, korrelierten nicht signifikant mit dem IQ. Die Einschränkungen der Studie werden in dem Artikel diskutiert.

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Résumé La relation entre la variation normale du QI et la psychopathologie commune a été examinée chez un échantillon de 339 jeunes âgés de 5 à 16 ans vus à une consultation psychiatrique tertiaire. Le QI moyen était de 9.6 points plus bas (95% CI 5.5 à 13.6 points plus bas) pour les troubles des conduites que pour les troubles affectifs, avec les troubles mixtes entre les deux. Pour ces troubles communs, le QI moyen était 6.0 points plus bas (95% CI 1.6 à 10.3 points plus bas) chez les filles que chez les gar¢ons. La variation du QI dans les limites de la normale était inversement proportionnelle à une mesure dimensionnelle des troubles des conduites-une association qui n'était pas attribuable à la classe sociale ou liée aux acquisitions scolaires. D'autres mesures dimensionnelles de la psychopathologie des symptômes affectifs, l'immaturité du développement et difficultés relationnelles -n'étaient pas corrélées significativement avec le QI. Les limites de l'étude sont discutées dans cet article.

     

The relationship between childhood stress and distinct stages of dynamic behavior monitoring in adults: neural and behavioral correlates

Childhood adversity is a major risk factor for emotional and cognitive disorders later in adulthood. Behavior monitoring, one of the most important components of cognitive control, plays a crucial role in flexible interaction with the environment. Here, we test a novel conceptual model discriminating between two distinct dimensions of childhood adversity (i.e. deprivation and threat) and examine their relations to dynamic stages of behavior monitoring. Sixty young healthy adults participated in this study using event-related potentials and the dynamic stages of behavior monitoring including response inhibition, error detection and post-error adjustments were investigated in a classical Go/NoGo task. Multiple regression analyses revealed that participants with higher severity of childhood adversity recruited more controlled attention, as indicated by larger (more negative) conflict detection–related NoGo-N2 amplitudes and larger (more negative) error detection–related error-related negativity amplitudes. Higher severity of childhood abuse (an indicator of threat) was related to smaller (less positive) error appraisal–related error positivity amplitudes on the neural level and subsequently lower post-error accuracy on the behavioral level. These results suggested that prefrontal-supported controlled attention is influenced by universal adversity in childhood while the error-related behavioral adjustment is mainly affected by childhood abuse, indicating the dimensions of deprivation and threat are at least partially distinct.     

颅咽管瘤的显微手术治疗及疗效评估

目的探讨显微手术治疗颅咽管瘤的手术入路及手术疗效。方法回顾性分析43例颅咽管瘤病人的临床资料,采用经鼻蝶入路7例,翼点入路32例,额底经纵裂终板入路2例,经胼胝体前部入路2例。评估病人手术前后神经功能、垂体功能、视功能评分。结果肿瘤全切除32例(74%),次全切除9例(21%),部分切除2例(5%)。术后发生电解质紊乱31例,双侧硬脑膜下积液1例,继发颅内硬脑膜外血肿1例,死亡2例。随访4~90个月,平均28个月。肿瘤复发3例,其中再次手术治疗2例,γ-刀治疗1例。手术前后神经功能、视功能变化不明显,垂体功能障碍发生率明显增加(P<0.05)。结论颅咽管瘤应根据肿瘤部位、生长方式及术者经验选择合适的手术入路,术后垂体功能易受影响。     

Long-term outcome and reconsideration of intracystic chemotherapy with bleomycin for craniopharyngioma in children

Object We first reported postoperative intratumoral chemotherapy with bleomycin for craniopharyngiomas in 1985. However, this local bleomycin chemotherapy has not yet been used very frequently. It seems to be necessary for us to report long-term outcome and reconsideration of this treatment.Methods and results Local bleomycin chemotherapy was performed on 7 children (to 1985) and 11 children (from 1988 to 2004). A total of 11 pediatric patients with recurrent cystic craniopharyngioma was treated by intracystic injection of bleomycin after 1988 and followed up from 3 to 16 years. After 1985, 1 of 7 children whose clinical outcome was fair underwent intracystic injection of bleomycin again. This patient has never had recurrent tumor until now. The children whose clinical outcomes were excellent have no recurrence during follow-up from 21 to 26 years. After 1988, the results showed that cystic recurrences of 7 children have almost disappeared and the children have achieved a good school life, and 3 children are also achieving a good life after additional stereotactic radiosurgery.Conclusion Our results indicate that local bleomycin chemotherapy is effective and that recurrence does not occur after follow-up, which ranged in duration from 3 to 16 years.     

Serial changes of prefrontal lobe growth in the patients with benign childhood epilepsy with centrotemporal spikes presenting with cognitive impairments/behavioral problems

Several studies have reported a higher incidence of learning and behavioral difficulties in association with frontal lobe dysfunctions in children with benign childhood epilepsy with centrotemporal spikes (BCECTS). We studied serial changes in frontal and prefrontal lobe volumes using three-dimensional magnetic resonance imaging in BCECTS with or without cognitive impairments and behavioral problems and evaluated correlations between prefrontal lobe growth and active seizure period. Serial changes in regional cerebral volumes were measured in two patients with cognitive impairments and behavioral problems (BCECTS(+)) and five patients without neuropsychiatric deficits (BCECTS(−)). Eleven normal subjects (4-13 years old) served as controls. Volumes of the frontal and prefrontal lobes were determined using a workstation, and the prefrontal-to-frontal lobe volume ratio was calculated. Frontal and prefrontal lobe volumes revealed growth disturbance in BCECTS(+) compared with BCECTS(−) and control subjects. In addition, prefrontal-to-frontal lobe volume ratio increased serially in BCECTS(−) similarly to controls, but was stagnant or decreased in BCECTS(+). Prefrontal growth also revealed more rapid recovery in a BCECTS(+) patient with shorter active seizure period. These findings suggest that longer active seizure period as frequent spike-waves coupled with the occurrence of frequent seizures may be associated with prefrontal lobe growth disturbance, which relates to neuropsychological problems.     

Malignant transformation of craniopharyngioma with detailed follow‐up

A 29‐year‐old male patient was admitted into hospital with the main complaint of progressive visual disturbance. Both CT SCAN and MRI demonstrated a cystic‐solid contrast‐enhancing sellar‐suprasellar mass with obvious calcification. Histopathological examination of the first resected specimen showed a typical appearance of adamantinomatous craniopharyngioma. The patient received gamma knife therapy after his first operation because of partial tumor removal. He experienced two relapses in the subsequent 2 years, for which only surgical resection was performed. The later histopathology presented malignant appearance with tumor cells moderate to severe pleomorphism, hyperchromasia, increased nuclear cytoplastic ratio, high mitotic activity (30/10 high power fields) and focal coagulative necrosis. The patient died 9 months after identification of histologic malignancy. Clinical and histopathological features, biological behavior of one case of malignant craniopharyngioma were discussed, with a brief review of the relevant literature.     

Processing speed is associated with differences in IQ and cognitive profiles between patients with schizophrenia and their healthy siblings

Background: Processing speed turns out to be the central area of research on cognition in schizophrenia. So far the relationship between this dimension and the IQ level of patients and their healthy siblings has not been investigated.

Aim: To investigate the differences in cognitive speed in patients with schizophrenia and their healthy siblings, and to determine whether cognitive speed as a covariate affects differences in IQ and cognitive profiles between groups.

Methods: Forty-seven inpatients diagnosed with schizophrenia according to DSM-IV (SCH) and their 36 healthy siblings (HSB) were tested with cognitive speed tasks according to Bartzokis et al. method and Wechsler Intelligence Scale. Additional control for the possible impact of antipsychotic drugs and selected demographic variables on the cognitive performance was taken into account.

Results: The siblings scored significantly higher in the cognitive speed task (p?F(10, 770)?=?2.798, p?=?0.002. Similarly, the Performance and Full Scale IQs were significantly different, at p?p?=?0.459.

Conclusions: Significant differences in cognitive speed between patients and their healthy siblings generate the differences in the cognitive profile assessed with Wechsler Intelligence Scale. Some problems of cognitive speed diagnosis and further research on the cognitive schizophrenia endophenotype were discussed.     

Malignant craniopharyngioma; case report and review of the literature

A case of malignant craniopharyngioma in a 46‐year‐old woman presenting clinically with visual disturbance and bifrontal headache is reported. Histopathologic examination of the suprasellar mass showed a lesion characterized by nests of epithelial cells with a basaloid appearance, round‐to‐oval nuclei, moderate pleomorphism, hyperchromasia, increased nuclear cytoplastic ratio and high mitotic activity. Immunohistochemically, the tumor cells were positive for Ki‐67 (44.3%), p53 (98%), and p63 (100%), but negative for estrogen and progesterone receptors. Clinical and pathologic features with a brief review of the relevant literature for malignant craniopharyngioma as a novel member of tumors of the suprasellar region, is discussed.