Hippocampal abnormalities associated with various congenital malformations

Objective  Our objective in this retrospective study was to assess the hippocampal abnormalities, associate them with various congenital brain malformations, and define the frequency of the association in specific anomaly subgroups. Methods  A total of 62 patients with congenital malformations of the brain who had thin-slice coronal T2-weighted, fluid attenuated inversion recovery, IR, or T1-weighted 3D gradient echo images were retrospectively evaluated for the type of congenital brain malformation and morphological or rotational hippocampal abnormalities. Medical records were reviewed for age, sex, and symptoms. Conclusion  Hippocampal abnormalities are found in 55.8% of all patients with different kinds of congenital brain malformations that are mostly associated with cortical dysplasia, lissencephaly, and total agenesis of the corpus callosum. The severity of the injury may have an effect on the extent of the involvement of the brain.     

A prospective study of postpartum psychoses in a high-risk group. 5. Relationship to psychosocial aspects of labor and delivery

Within a sample of 88 pregnant index women with a history of nonorganic psychosis, the psychosocial aspects of labor and delivery, assessed through personal observation, were studied in relation to the development of 22 postpartum psychotic episodes (PPPs) occurring during the first 6 months postpartum. As compared with index cases not developing PPPs, cases with PPP onset after 3 weeks postpartum had been significantly more anxious, had received significantly less help from the midwife, and their husbands tended to have attended the birth less often. Cases with PPP onset within 3 weeks of delivery had not been more anxious, but their husbands had been significantly more anxious and the midwives tended to have helped less. Early Onset (but not Later Onset) mothers had had slightly more frequent negative reactions towards the newborn, while the husbands in both groups tended to have been more positive towards the newborn than were other husbands.     

A prospective study of postpartum psychoses in a high-risk group. 7. Relationship to later offspring characteristics

Among the offspring of 88 pregnant index women with a history of nonorganic psychosis, offspring whose mothers developed postpartum psychotic episodes (PPPs) following birth (maximum n = 17) were compared with the remaining index offspring on emotional development during the first year of life, temperament characteristics during the first 2 years, and mental characteristics at 6 years of age. The maternal PPPs were not associated with any negative consequences for offspring development, and the offspring of PPP cases evidenced a number of more positive mental developmental characteristics than did other index offspring. Further comparison with offspring of normal-risk controls also supported the conclusion of no negative effect caused by maternal PPPs. Choice of an appropriate comparison group for PPP cases is discussed.     

Nonspecific psychological distress, psychosocial stressors and delusional psychosis. A comparison with psychiatrically symptom free surgical controls

The purpose of the study was to examine patients with delusional psychosis regarding characteristic personality traits as illustrated by nonspecific psychological distress, and psychosocial stress prior to onset of the psychosis. The design of the study was a comparison of first-admitted psychiatric patients with delusional psychosis and psychiatrically symptom free surgical controls. Patients with delusional psychosis reported significantly more psychosocial stress than controls during the 3 months prior to admission. They also reported significantly higher mean values than controls on the subscales named poor self-esteem, helplessness-hopelessness, dread, sadness, anxiety and approval of rule breaking on the schedule of nonspecific psychological distress. In discriminating patients with delusional psychosis and surgical controls, approval of rule breaking, helplessness-hopelessness, and severity of psychosocial stressors were the most informative variables. Patients with delusional psychosis will be followed up in order to analyze if nonspecific psychological distress and psychosocial stressors are of importance for the prediction of the course and the outcome.     

Single fiber EMG in a congenital myasthenic syndrome associated with facial malformations

Six patients with a newly described genetic syndrome in Iraqi and Iranian Jews of congenital myasthenia associated with facial malformations were studied with voluntary and stimulation single fiber EMG (SFEMG). Voluntary SFEMG revealed abnormal jitter in all patients in both extensor digitorum communis (EDC) and orbicularis oculi (OOC) muscles, though much smaller in the clinically unaffected EDC. SFEMG study of OOC muscle by axonal stimulation at rates from 1 to 48 Hz showed the most increased jitter at the highest stimulation frequencies in the majority of end-plates, one-third of which showed maximal jitter at intermediate rates. These results may suggest a postsynaptic abnormality as the underlying cause for the neuromuscular transmission defect, and demonstrate the usefulness of SFEMG in the diagnosis of congenital myasthenia. © 1993 John Wiley & Sons, Inc.     

A prospective study of postpartum psychoses in a high-risk group. 6. Relationship to birth complications and neonatal abnormality

Somatic complications of labor and delivery and offspring abnormality were studied in relationship to the development of 24 postpartum psychotic episodes (PPPs) occurring among index women with a history of nonorganic psychosis. The 24 PPP cases were compared with 60 index cases not developing PPPs. Nonsignificant trends were found towards increased rates of induction of labor, fetal distress, instrumental deliveries and offspring abnormality among cases with PPP onset within 3 weeks of delivery. Instrumental delivery was the only complication that tended to have occurred with increased frequency among cases with PPP onset after 3 weeks.     

A prospective study of postpartum psychoses in a high-risk group. 3. Relationship to mental health characteristics during pregnancy

Within a sample of 88 pregnant women with a history of nonorganic psychosis, mental health characteristics during pregnancy, assessed prospectively through interviews and psychiatric records, were studied in relationship to the development of 25 postpartum psychotic episodes (PPPs) occurring during the first 6 months postpartum. Cases with PPP onset within 3 weeks of delivery (mostly affective or cycloid psychoses) evidenced more frequent tension-anxiety and excitement at interviews during pregnancy than did diagnostically comparable cases not developing PPPs. Cases with PPP onset later than 3 weeks postpartum (mostly schizophrenic-like psychoses) were not more frequently disturbed than were diagnostically comparable cases not developing PPPs. An absence of both affective symptoms and common fears represented a sign of increased PPP-risk in these later onset cases. The subsample of actively disturbed cases who were in contact with a psychiatrist during pregnancy were at notably increased risk for a PPP during the total 6-month period.     

A prospective study of postpartum psychoses in a high-risk group. 4. Relationship to life situation and experience of pregnancy

Within a sample of 88 pregnant women with a history of nonorganic psychosis, the woman's life situation and experience of pregnancy, assessed through interviews during pregnancy, were studied in relationship to the development of 25 postpartum psychotic episodes (PPPs) occurring during the first 6 months postpartum. Neither Early nor Later Onset PPPs were associated with more current material situational problems, interpersonal difficulties, lack of relatives' support for the pregnancy, the woman's negative attitude toward the pregnancy or her experience of somatic health complaints. Contrary to expectation, PPP cases reported several more positive situational, attitudinal and experiential characteristics than did risk cases not developing PPPs. A number of different possible explanations for this finding are discussed.     

抗癫药物对妊娠癫癎患者子代的致畸风险

目的:旨在评估抗癫癎药物(AEDs)对妊娠癫癎患者子代出现先天畸形的风险。方法:对妊娠癫癎患者采用登记和随访研究,分析其孕期AEDs用药情况、癫癎发作、妊娠结局及子代出现畸形的风险。结果:入选105例妊娠癫癎患者。服用AEDs患者79/105例(75.2%),未服用AEDs患者26/105例(24.8%)。单药治疗60/79例(75.9%),其中1/60例(1.7%)流产;患者子代中2/60例(3.3%)先天性畸形(1例服用卡马西平,出现先天性心脏动脉导管未闭;1例服用拉莫三嗪,出现无胚心)。联合用药19/79例(24.1%),子代无先天畸形出现。未服用AEDs患者中有2/26例(7.7%)流产,其余患者子代未出现先天畸形。结论:妊娠癫癎孕妇多数于孕期仍服用AEDs,且以单药治疗居多;使用AEDs(分别为卡马西平和拉莫三嗪)患者子代出现2例先天性畸形;丙戊酸钠易致畸但仍在妊娠癫癎中经常使用,本研究中服用丙戊酸钠孕妇未出现子代先天性畸形。     

皮层脑电图监测在伴有癫痫的颞叶海绵状血管瘤手术中的作用

目的 探讨皮层脑电图（ECoG）监测在伴有癫痫的颞叶海绵状血管瘤手术中的作用。方法 2006年1月至2015年3月手术治疗伴有癫痫的颞叶海绵状血管瘤98例,术中采用ECoG监测53例,未用ECoG监测45例。术后随访1~8年,平均3.5年。结果 术后无死亡及永久性功能障碍。术后Engle Ⅰ级72例（73.5%） ,Ⅱ级18例（18.4%）,Ⅲ级5例（5.1%）,Ⅳ级3例（3.1%）。癫痫病史<1年的36例中,术后Engle Ⅰ级31例（86.1%）;>1年的62中,术后Engle Ⅰ级41例（66.1%）;两组差异显著（P<0.05）。ecog监测下手术的53例中,术后engle ⅰ级44例（83.0%）;未监测的45例中,术后engleⅰ级28例（62.2%）;两组亦差异显著（P<0.05）。>结论 伴有癫痫的颞叶海绵状血管瘤应尽早手术治疗;在ECoG监测下做病灶扩大切除或脑叶切除将获得更理想的癫痫控制率。     

Pineal germinoma associated with multiple congenital melanocytic nevi: A unique presentation

Intracranial germ cell tumors are rarely seen and typically localize in the pineal or suprasellar region. The largest category of germ cell tumors is the germinoma. There have been reported associations of malignant tumors and chromosomal abnormalities in germ cell tumors. In this study, we present a 22‐year‐old man with multiple congenital melanocytic nevi in association with pineal tumor. Congenital melanocytic lesions greater than 2 cm were counted to be 54 in number, and those smaller than 1 cm in diameter were found to be 25 in number. The pathological diagnosis of the pineal tumors was germinoma, and the lesions located in the occipital region and trunk were compound congenital nevi. To our knowledge, a relationship between multiple congenital melanocytic nevi and germ cell tumors has not been reported before. The connection between them remains to be clarified.     

Analysis of the treatment of 576 patients with congenital craniovertebral junction malformations

We aim to report our experience treating craniovertebral junction malformations (CVJM) and to investigate the management of this uncommon condition. Between 2000 and 2009, 629 patients with CVJM underwent surgery in our department. Fifty-three patients were lost to follow-up; therefore 576 patients completed follow-up, for an average period of 3 years and 2 months. All patients were diagnosed on the basis of clinical presentation and imaging features, and we found that anomalies of the occipitocervical junction manifested as four major types. Different microsurgical treatments were carried out in these patients according to disease type, and the effectiveness of individualised treatments was analysed. Categorizing patients with CVJM into these four types to simplify this somewhat unclear area could provide insight into the pathogenesis of the anomaly and a basis for rational surgical treatment.     

颅内动静脉畸形合并动脉瘤的血管内治疗

目的 总结血管内治疗颅内动静脉畸形(AVM)合并动脉瘤的经验.方法 回顾性分析25例颅内AVM合并动脉瘤的临床资料.所有病例均经全脑血管造影(DSA)确诊,按照Redekop分型进行治疗.结果 动脉瘤完全栓塞23例,部分栓塞2例.畸形血管团完全消失7例,消失90％以上9例,消失80％～90％7例,消失80％以下2例.栓塞后手术切除12例,栓塞后伽玛刀治疗6例.所有病人均未出现与血管内治疗相关的并发症.随访25例,时间0.5～3年,无再次出血及死亡.DSA复查18例,其中动脉瘤和畸形血管团均不显影14例,动脉瘤不显影但畸形血管团少许残留4例.结论血管内治疗是颅内AVM合并动脉瘤安全、有效的治疗方法,Redekop分型可对其治疗提供指导.     

Abnormal course of spinal posterior roots and tracts associated with brain malformations

Summary An unusual mode of the spinal posterior nerve root entrance and of the course of ascending tracts in the hyperplastic gray matter of the posterior horn was observed in three newborn babies. This sensory pathway malformation was combined with severe but unspecific brain anomalies. The common associated brain anomaly was agenesis of the corpus callosum.Dedicated to Professor Dr. Hans Jacob for his 80th birthday     

脑血管畸形破裂出血的影响因素分析

目的 分析脑血管畸形患者发生出血的影响因素,为防治出血的发生提供诊治依据. 方法 回顾性分析吉安市中心人民医院神经外科自2002年1月至2012年1月收治的126例脑血管畸形患者的影像学资料,根据其是否发生出血分为出血组和未出血组,结合畸形血管团大小、位置,引流静脉方向、数目,供血动脉位置,合并动脉瘤数目、与畸形血管团位置关系,有无合并瘤样变等因素进行多因素Logistic回归分析和受试者工作特征(ROC)曲线分析,并比较各影响因素的曲线下面积(AUC),评价各影响因素对脑血管畸形破裂出血的预测价值. 结果 单因素分析显示畸形血管团大小、引流静脉方向、供血动脉位置、合并动脉瘤数目、合并瘤样变、与畸形血管团位置关系在出血组和未出血组间比较差异有统计学意义(P＜0.05).多因素Logistic回归分析及ROC曲线分析显示畸形血管团(OR=0.218,P=0.012)、引流静脉方向(OR=8.023,P=0.004)、合并动脉瘤(OR=6.539,P=0.029)是脑血管畸形患者发生出血的影响因素,AUC值分别为0.674、0.897、0.729.结论畸形血管团大是患者发生出血的保护因素,深部静脉引流、合并动脉瘤是患者发生出血的危险因素;引流静脉方向对患者发生出血的预测价值最高.     

A prospective study of postpartum psychoses in a high-risk group. 1. Clinical characteristics of the current postpartum episodes

Postpartum psychotic episodes (PPPs) occurring during the first 6 months after delivery were prospectively studied in 88 pregnant index women with a history of nonorganic psychosis and 104 pregnant controls with no such history. While no control developed a PPP, PPPs were found following 28% of the index deliveries, almost all of these 25 cases being psychiatrically hospitalized. PPPs were especially frequent among cases with total illness diagnoses of Cycloid Psychosis and Affective Illness. More than half of the 25 cases had symptom onset within 3 weeks of delivery, and these early onset cases represented predominantly affective disorders, many of whom were manic in this episode. Cases with onset after 3 weeks were predominantly schizophrenic. Confusion was part of the current episode symptomatology in about one third of the cases and was well distributed across the different diagnostic groups.