The patterns of progression in primary progressive aphasia—Implications for assessment and management

Background: Primary progressive aphasia (PPA) is a progressive language disorder with preserved cognitive function for at least 2 years from onset. The main variants currently distinguished are: non-fluent/agrammatic (nfvPPA), semantic (svPPA), and logopenic (lvPPA). Patients with initial language presentation may subsequently develop other symptoms, such as behavioural dysfunction or apraxia. The clinical pattern of PPA depends on the location of atrophy, the underlying pathology, and the stage of the disease.

Aims: This review aims at characterising longitudinal changes in clinical presentations of different PPA variants and at presenting implications of these changes for the assessment, diagnosis, and management.

Main contribution: The three PPA variants differ not only in terms of language impairment, but also with regard to cognitive and behavioural profile. Apraxia and rigidity frequently occur in the course of nfvPPA. Patients with lvPPA seem to follow the pattern of aphasic Alzheimer’s disease, where language impairment is accompanied by episodic memory deficit. Individuals diagnosed with svPPA often develop behavioural dysfunction similar to that observed in behavioural variant of frontotemporal dementia.

Conclusions: Implications for patient care are dependent on PPA variant and on the stage of the disease. In svPPA, emphasis should be on the management of semantic and behavioural problems in daily life. Caregivers of nfvPPA patients should be informed about the possible emergence of apraxia and other movement disorders. In contrast, families of individuals with lvPPA should be made aware of and trained to cope with an episodic memory decline and possible progression to other varieties of PPA.     

Pediatric Anti-NMDA (N-methyl D-Aspartate) Receptor Encephalitis

BackgroundWe report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis.MethodsSingle-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid.ResultsThree girls with anti-NMDA-R encephalitis were identified. All presented with an acute hyperkinetic movement disorder and seizures, expressive aphasia, and emotional lability requiring inpatient treatment for 1-3 months. Imaging and electroencephalogram findings were nondiagnostic. None had an underlying tumor or ovarian teratoma. All received immune-modulatory therapy, including one or more of the following: high-dose methyl-prednisolone, plasma exchange, intravenous immunoglobulin or mycophenolate mofetil. Two of the three patients relapsed within 6 months of presentation and required retreatment with plasma exchange. All have remained in subsequent remission, with two of the three requiring second-line immunotherapy with rituximab.ConclusionsHyperkinetic movements in pediatric patients presenting with acute encephalopathy and prominent psychiatric symptoms should elicit a search for NMDA-R antibodies early in the evaluation. Relapses require aggressive immunomodulatory treatment for remission. This series highlights a unique positron emission tomography scan finding of hypermetabolism in one of the patients that correlated with her clinical symptoms. Recovery and rehabilitation can be prolonged, often taking years after the initial diagnosis. Early identification and treatment is likely to reduce relapses and limit morbidity associated with this potentially devastating but treatable encephalitis.     

Cerebellar Mutism in Acute Disseminating Encephalomyelitis

BackgroundCerebellar mutism in children occurs after posterior fossa tumor resection and can have lasting effects on cognition, language, and behavior. Cerebellar mutism in acute disseminated encephalomyelitis is rare.PatientA 7-year-old boy with a 3-day history of fever, vomiting, and diarrhea presented with altered mental status and expressive aphasia. Magnetic resonance imaging showed new diffusion restriction in the bilateral dentate nuclei and right cerebellum. With treatment, he began to verbalize again but had long-term cognitive and language difficulties.ConclusionAcute disseminated encephalomyelitis is commonly a benign process, but its effect on the cerebellum can be long-lasting.     

非流畅性失语患者朗读两类成语的研究

目的 考查非流畅性失语患者在语音提示下朗读2种类型成语的能力,为确定其朗读训练方向提供依据.方法 对30例非流畅性失语患者实施自身对照研究,于言语训练前进行成语朗读能力测试.测试时,在语音提示成语前2个字后,分别朗读唯一搭配成语和双重搭配成语后2个字,2种类型成语测试满分分别为30、34分.结果 患者朗读唯一搭配成语成绩（21.12±11.65）分,双重搭配成语成绩（20.84±9.89）分,差异有统计学意义（P＜0.05）.结论 非流畅性失语患者朗读2种类型成语的能力存在差异,测试成绩可作为制定和调整言语训练的参考标准及训练内容.     

Neuroimaging in dementia

Over the last few years, advances in neuroimaging have generated biomarkers, which increase diagnostic certainty, provide valuable information about prognosis, and suggest a particular pathology underlying the clinical dementia syndrome. We aim to review the evidence for use of already established imaging modalities, along with selected techniques that have a great potential to guide clinical decisions in the future. We discuss structural, functional and molecular imaging, focusing on the most common dementias: Alzheimer's disease, fronto-temporal dementia, dementia with Lewy bodies and vascular dementia. Finally, we stress the importance of conducting research using representative cohorts and in a naturalistic set up, in order to build a strong evidence base for translating imaging methods for a National Health Service. If we assess a broad range of patients referred to memory clinic with a variety of imaging modalities, we will make a step towards accumulating robust evidence and ultimately closing the gap between the dramatic advances in neurosciences and meaningful clinical applications for the maximum benefit of our patients.     

旋律音调疗法治疗脑卒中后非流畅性失语的效果

目的 观察旋律音调疗法对脑卒中后非流畅性失语的临床康复疗效以及脑功能改变。方法 2017年3月至2019年8月,选取本院脑卒中后非流畅性失语患者40例,随机分为对照组和音乐组各20例,两组均接受言语治疗,音乐组在此基础上接受以旋律音调疗法为主的音乐治疗。治疗前和治疗4周后,采用中国康复研究中心汉语标准失语症检查表(CRRCAE)评定其听理解、复述、阅读、命名。两组各选3例患者,治疗前后行静息态fMRI,比较大脑半球局部一致性(ReHo)。结果 治疗后,对照组听理解(名词、动词、句子)、复述(名词、动词)、阅读(名词)和命名(动作说明)评分提高(t > 2.221, P < 0.05),音乐组听理解(名词、动词、句子)、复述(名词、动词、句子)、阅读(名词、动词)和命名(名词、动作说明)评分提高( t > 2.179, P < 0.05)。治疗前后差值比较,音乐组复述(名词、动词)评分增加更多( t > 2.299, P < 0.05),阅读(句子)评分有增加的趋势。治疗后,患者静息状态下左侧小脑、颞枕区的ReHo增强,双侧额叶、颞叶皮质ReHo降低。 结论 在言语训练基础上,联合旋律音调疗法可进一步改善脑卒中后非流畅性失语患者的言语功能,特别在句子阅读和单词复述方面。ReHo改变可能与脑损伤后神经修复有关。     

抑制性rTMS刺激联合镜像神经元训练系统对脑卒中后完全性失语的作用研究

目的：探讨抑制性重复经颅磁刺激(rTMS)联合镜像神经元训练系统(MNS)对脑卒中后完全性失语的临床疗效及其作用机制。方法：50例脑卒中后完全性失语患者按随机数字表法随机分为对照组16例、MNS组16例和联合组18例。对照组仅给予常规的言语康复训练,MNS组在对照组的基础上给予镜像神经元系统训练,联合组在MNS组的基础上给予右侧Broca同源区1Hz的rTMS刺激,于治疗前和治疗3周后评定3组患者西方失语成套测验(WAB)以及波士顿失语症程度分级（BDAE）评分。结果：治疗3周后,3组WAB各项评分和BDAE评分均较治疗前明显提高（P<0.05）;且联合组WAB各项评分及BDAE评分显著高于MNS组和对照组（P<0.05）,MNS组WAB评分中自发语评分及AQ评分亦高于对照组（P<0.05）,BDAE评分MNS组和对照组治疗后比较差异无统计学意义。结论：抑制性rTMS刺激右侧Broca同源区联合MNS训练系统对改善脑卒中后完全性失语患者的语言功能有一定作用。     

卒中后抑郁患者家庭功能及家庭护理干预的研究进展

卒中后抑郁(post-stroke depression,PSD)是指发生于卒中后,表现为一系列抑郁症状和相应躯体症状的综合征,是卒中后常见且可治疗的并发症之一,如未及时发现和治疗,将影响卒中后患者神经功能的恢复和日常生活能力,增加患者的致残率和死亡率[1-3]。     

脑卒中后抑郁的相关因素分析

目的:探讨脑卒中后抑郁的相关因素。方法:采用汉密尔顿抑郁量表(HAMD)对198例脑卒中患者进行现状调查。结果:73例脑卒中患者有抑郁表现,发生率为36.9%,经Logistic多元回归分析,脑卒中后抑郁的相关因素有负性生活事件、心血管病史、抑郁症史、病灶数目、病灶部位以及脑卒中史。结论:脑卒中后抑郁的相关因素有负性生活事件,心血管病史,抑郁症史、病灶数目,病灶部位以及脑卒中史。脑卒中后抑郁的发生可能是神经生物学因素和社会心理学因素共同作用的结果。     

卒中后失语静息态脑功能成像亚频段低频振幅脑活动研究

目的 探索卒中后失语(PSA)患者局部脑神经活动在不同频段的特征及西方失语成套测试(WAB)评分与特定频段的关系.方法 2015年3月至2018年5月,招募本院PSA患者15例(患者组)和年龄、性别、受教育程度等匹配的健康成年人15例(对照组),采用WAB测试;采集静息态功能性磁共振成像(rs-fMRI),计算低频振幅...