Marchiafava-Bignami disease treated by mianserin hydrochloride in short-term evaluated by neuropsychological analysis

Since 1903, Marchiafava‐Bignami disease has been recognized as a rare syndrome with focal demyelination and necrosis in the corpus callosum, which is usually found in chronic alcoholics. It extends into the neighboring white matter and occasionally as far as the subcortical regions. We report a Japanese patient with Marchiafava‐Bignami disease associated with alcohol abuse, who had traveled around Western Europe, North America and China for more than 30 years. As he suffered extreme delirium in the early stages we administered a low dose (10 mg) of mianserin hydrochloride. He was very irritable and uncooperative on admission, after 20 days his delirium had disappeared and his temper had become very calm and mild. After 40 days, his intelligence level increased substantially as measured by various neuropsychological tests.     

Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

Tourette综合征的神经心理和血浆催乳素含量及其关系的初步研究

本研究采用中国修订的韦氏儿童智力量表（ＷＩＳＣ－ＣＲ）、韦氏儿童记忆量表（ＷＭＳＣ－ＣＲ），艾森克个性问卷（ＥＰＱ）和Ａｃｈｅｎｂａｃｈ儿童行为量表（ＡＣＢＣ），对３９例（未用药组２２例，用药组１７例）７～１４岁Ｔｏｕｒｅｔｔｅ综合征（ＴＳ）患儿进行测试，同时采用放射免疫方法测定其血浆催乳素（ＰＲＬ）含量，并与１８例正常对照组比较。结果表明：ＴＳ患儿智力测验和记忆测验中的背数、译码和视觉再生分测验量表分低于对照组。其个性测验中的神经质（Ｎ）和精神质（Ｐ）量表Ｔ分高于对照组，掩饰性（Ｌ）量表Ｔ分低于对照组。其ＡＣＢＣ总分高于对照组。用药组的记忆商（ＭＱ）、理解记忆和累加分测验量表分低于未用药组，ＴＳ患儿的血浆催乳素含量高于对照组。相关分析表明血浆催乳素含量与ＡＣＢＣ总分和精神抑制药物如氟哌啶醇和泰必利等因素有关。我们的初步研究提示ＴＳ患儿存在不同程度的神经心理缺陷，催乳素可能参与ＴＳ发病过程，氟哌啶醇和泰必利等精神抑制药物可能对ＴＳ患儿的记忆功能有影响以及可以导致血浆催乳素分泌增加，为ＴＳ的防治干预提供了一定的理论依据。     

Brain dysfunction in social drinkers

Thirty-four social drinkers who had referred themselves to the Regional Brain Damage Unit for assessment of the effects of drinking alcohol were compared with 42 volunteer control subjects of equivalent age but with low alcohol intake, using two 'learning' tests — the Rey Auditory Verbal Learning Test (RAVLT) and the Austin Button Maze. The Maze Test gave no evidence of disorder, but the two groups were significantly different on the RAVLT. No abnormalities in standard cognitive tests were apparent. These results suggest that a deficit in learning ability may be an early feature of the brain dysfunction associated with excessive alcohol consumption.     

Neuropsychological correlates of intractable anxiety disorder before and after capsulotomy

A comprehensive neuropsychological test battery was administered to 10 consecutive patients undergoing neurosurgical intervention, capsulotomy, as a last resort treatment for chronic, incapacitating, and otherwise intractable illness, either obsessive-compulsive disorder (OCD; n= 5) or non-OCD anxiety disorder (n= 5). The aim was to study the neuropsychology of severe anxiety disorders before and after a defined neurosurgical intersection of connections between the frontal lobes and related brain regions. Although extremely disabled by their illness before surgery, the patients performed within the normal range on most tests. After capsulotomy, there was significant improvement on measures of clinical morbidity and of psychosocial functioning, and the general neuropsychological performance remained remarkably intact. In a subgroup of 5 patients, however, perseverative responses were more common postoperatively, possibly indicating dysfunction of systems involving the frontal lobes. Although admittedly sparse, these data can be interpreted as suggesting that in vulnerable individuals, capsulotomy may give rise to increased perseverative behaviour in the laboratory, and possibly in the real world as well. This risk must be weighed against the potential clinical benefit of capsulotomy in this extremely disabled, sometimes suicidal patient population.     

A critical survey of hypotheses regarding Post-traumatic Stress Disorders in light of recent research findings

This paper reviews briefly recent findings from psychobiological research upon Post-traumatic Stress Disorders. A critical assay is provided of the various hypotheses advanced as the result of this research data. The author suggests neuropsychological theory provides the best explanation for understanding and classifying both the clinical phenomenology and the biological abnormalities discovered in the chronic forms of PTSD.Initial draft presented at a workshop Delayed Effects of Post Traumatic Stress Disorders, sponsored by the Emergency Services Branch of the National Institutes of Mental Health April 14–15, 1986.     

Declining immune function in children and adolescents with hemophilia and HIV infection: effects on neuropsychological performance. Hemophilia Growth and Development Study

OBJECTIVE: To determine whether declines in immune functioning are associated with changes in neuropsychological performance in children and adolescents with hemophilia who are infected with the human immunodeficiency virus (HIV). METHODS: Participants were 333 males with hemophilia, ages 6-19 years at entry. A baseline and four annual neuropsychological evaluations were given. A longitudinal growth curves analysis of data was performed to detect changes associated with declining immune function. The cohort was stratified into four groups: (1) HIV- (n = 126); (2) HIV+, average of first two and last two CD4 counts > or = 200, (n = 106; High CD4 group); (3) HIV+, average first two counts > or = 200, average last two counts < 200 (n = 41; CD4 Drop group); and (4) HIV+, average first two and last two counts < 200 (n = 60; Low CD4 group). RESULTS: There were significant differences among the four groups over time in nonverbal intelligence, perceptual/performance skills, nonverbal memory, academic achievement, and language. The Low CD4 group consistently showed the greatest decrement in performance. On measures showing a practice effect for repeated measurements, the Low CD4 group participants' scores remained stable over time, suggesting opposing effects of practice and HIV-related declines. Lowered academic performance relative to IQ was found in all groups. CONCLUSIONS: Declines in neuropsychological functioning are directly related to declines in immune functioning in HIV+ children, adolescents, and young adults with hemophilia. Hemophilia itself may be a risk factor for academic underachievement.     

胼胝体分离综合征神经心理学特征的诊疗流程及应用

目的:本研究拟通过标准的诊断流程,分析胼胝体分离综合征(CDS)的神经心理学特征,为临床诊疗提供客观依据。方法:回顾性分析8例CDS患者的病因、神经心理学表现及影像学特点。结果:影像学提示患者均为缺血性卒中,病灶均累及胼胝体,其中膝部6例、体部8例、压部4例;患者均伴有认知障碍,MoCA评分为(19.00±4.11),均26分;神经心理学表现复杂多样,评估显示偏瘫(8/8例)、异己手综合征(8/8例)、失语症(3/8例)、触觉失认(4/8例)、失用症(5/8例)、失读症(4/8例)、失写症(4/8例)、偏侧忽略(3/8例)、精神障碍(4/8例)。结论:CDS临床表现和神经心理学特征复杂多变,标准的评估流程有助于明确诊断,为临床诊疗提供可靠依据。     

老年原发性失眠的神经心理学测验与P300检测的关系及其意义

目的 了解老年原发性失眠患者认知功能有无损害及其程度. 方法 用神经心理学检测(MMSE、MoCA量表)、P300检测52例老年原发性失眠患者及60例正常体检者. 结果老年原发性失眠患者MoCA量表中视空间与执行功能、注意、延迟回忆得分及总分较对照组显著下降,差异有统计学意义(P＜0.01).MMSE量表评分亦有下降趋势,但差异无统计学意义.P300结果显示无论是靶刺激还是非靶刺激,其诱发出的波明显低平及后移,差异有统计学意义(P＜0.01). 结论 老年原发性失眠患者存在认知功能损害,联合MoCA量表评估及P300检测有助于老年原发性失眠患者早期认知功能损害的诊断.