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.     

Long-term treatment with tacrine (THA) in Alzheimer's disease—evaluation of neuropsychological data

Long-term effects of tacrine (THA) on cognitive functions of very mild AD patients were studied. The stability of possible positive changes following prolonged treatment and the effect of increased dose was also studied. Three patients were treated with tacrine (80 mg/day) and the effect on cognitive functions was measured with a neuropsychological test battery. Two of the patients (Pats 1 and 4) showed clear positive changes in all parameters measured. The third patient (Pat 5) did not show as clear positive responses. The effect of the initial treatment dose diminished over time. After raising the dose two of the patients showed improvement in cognitive tests reaching their initial level of performance or even better in most of the tests. This positive effect was not as clear in patient 5. After 13 months of tacrine all patients still showed positive changes in some of the tests. Compared to a hypothetical progression curve for untreated AD patients the patients treated with tacrine seemed to have slower progression. In conclusion, it seems that long-term positive effects on cognitive functions of AD patients can be reached with tacrine and it seems to be possible to slow down the progression of the disease. However, to reach long-term positive effects increasing doses seem to be needed. AD patients seem to differ in their response to tacrine.     

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。     

EEG reactivity correlates with neuropsychological test scores in Down's syndrome

Introduction – Down's syndrome patients express a neurodegenerative disorder and mental retardation. We studied the reactivity of EEG and its correlation with neuropsychological test score in Down's syndrome. Material and methods – We studied 32 patients with Down's syndrome and 31 controls for blocking of occipital EEG activity. The temporo-occipital EEG with eyes open (EO) was compared with resting EEG with eyes closed (EC), (EC/EO ratio). Results – Both Down patients and controls showed significant diminution of alpha, beta and theta activity and decrease of EEG frequency with EO. However, there was a significant impairment in Down patients in the EC/EO ratio in alpha band, compared to controls. The controls had no correlation of the alpha EC/EO ratio with age or gender. The Down patients showed a significant correlation of this variable with age which is in accordance with a gradually progressing disease. They had also significant correlations of the alpha EC/EO ratio and neuropsychological test scores which indicates that this ratio may be a more general measure of cerebral or hemispherical dysfunction than a mere impairment of visual activation. Down patients also showed significant differences in resting EEG variables, compared to the controls, even if the conventional EEG showed normal or mildly slowed dominant occipital rhythm in most of the patients. The correlation analysis between resting EEG and EC/EO ratio variables pointed out that they are relatively independent, representing different factors in the regulation of EEG. Conclusions – We believe that the alpha EC/EO ratio of EEG add a new domain in the assessment of cerebral dysfunction in Down's syndrome.     

癫(癎)患者认知功能影响因素的研究

目的:研究癫患者认知功能的影响因素。方法:对166例癫患者进行认知评定。认知评定工具:听觉词语测验、逻辑记忆测验、数字符号转换测验、Stoop字色干扰测验、连线测验、言语流畅性测验、Rey-Osterrieth复杂图片测验及Boston命名测验。影响因素与认知功能评分之间采用一元线性相关及多元逐步回归分析。结果:患者年龄、性别、文化程度、起病年龄、病程、发作频率、发作持续时间、全身强直阵挛发作(GTCS)、复杂部分性发作(CPS)及抗癫药物数量与患者的认知功能有相关性。结论:癫患者的起病年龄与其认知损害成正比,病程越长对癫患者的认知损害越明显,原发或继发全身性强直阵挛发作与言语功能损害、复杂部分性发作与言语记忆损害之间的关系密切,用药种类与记忆、注意力以及精神运动能力的损害有关。     

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.