Narrative review of the psychometric properties of language tests used in anomia treatment for primary progressive aphasia (PPA)

Background: Primary progressive aphasia (PPA) is a neurodegenerative dementia in which language decline is the first and most prominent symptom. Among several interventions for PPA, language rehabilitation has been the most frequently used.

Aims: This narrative review aimed to evaluate existing standardised language tests used in the assessment of PPA, in order to determine whether they are appropriate and psychometrically adequate to detect change over time in the treatment of anomia.

Main Contribution: The present findings highlight the scarcity of psychometrically robust instruments used to measure therapy-induced gains in PPA. Additionally, most of these instruments were not validated for use with the PPA population, which consequently might bias the results. There is a need for population-based norms for existing instruments.

Conclusions: The accurate assessment of lexico-semantic deficits in PPA should rely on objective, reliable, valid, and responsive outcome measures. Psychometric studies are needed to evaluate and eventually improve the quality of language tests used in clinical practice.     

A MEG study of the neural substrates of semantic processing in semantic variant primary progressive aphasia

Despite a well-documented pattern of semantic impairment, the patterns of brain activation during semantic processing in semantic variant primary progressive aphasia (svPPA) still remain poorly understood. In the current study, one svPPA patient (EC) and six elderly controls carried out a general-level semantic categorization task while their brain activity was recorded using magnetoencephalography (MEG). Despite similar behavioral performance, EC showed hyperactivation of the left inferior temporal gyrus (ITG) and right anterior temporal lobe (ATL) relative to controls. This suggests that periatrophic regions within the ATL region may support preserved semantic abilities in svPPA.     

Fundamental or forgotten? Is Pierre Paul Broca still relevant in modern neuroscience?

The ability to speak is a unique human capacity, but where is it located in our brains? This question is closely connected to the pioneering work of Pierre Paul Broca in the 1860s. Based on post-mortem observations of aphasic patients' brains, Broca located language production in the 3rd convolution of the left frontal lobe and thus reinitiated the localizationist view of brain functions. However, contemporary neuroscience has partially rejected this view in favor of a network-based perspective. This leads to the question, whether Broca's findings are still relevant today. In this mini-review, we discuss current and historical implications of Broca's work by focusing on his original contribution and contrasting it with contemporary knowledge. Borrowing from Broca's famous quote, our review shows that humans indeed “speak with the left hemisphere”– but Broca's area is not the sole “seat of articulatory language”.     

How clerks understand the requests of people living with aphasia in service encounters

ABSTRACT

Aphasia often restricts participation. People living with aphasia (PLWA) engage in fewer activities, which leads to fewer interactions than before aphasia. Analyses of interactions with non-familiar people in activities of daily life could provide knowledge about how to integrate these situations in rehabilitation and facilitate ongoing PLWA participation post-rehabilitation. This qualitative study is the first to examine how PLWA make their requests understood in service encounters despite aphasia. Six people living with moderate or severe aphasia were video-recorded in situations of service encounters, e.g., pharmacies, specialised shops, restaurants, and others. We identified fifty-nine occurrences with one or several difficulties in the formulation of the request. They were examined, including the clerks’ responses and ensuing interaction using multimodal conversation analysis. Results showed that PLWA used nonverbal communication within the physical environment and the context of the interaction to support verbal production. In the majority of situations, the clerks understood the request promptly. In other situations, they both collaborated to achieve a clear understanding of the request. Moreover, the findings attest to the competence of people living with moderate or severe aphasia in engaging in service encounters and add to the knowledge base about interaction and social participation in aphasia.     

多模态技术联合术中唤醒麻醉在Broca区胶质瘤患者语言功能区定位中的作用

目的探讨多模态技术联合术中唤醒麻醉在Broca区胶质瘤患者语言功能区定位中的作用和手术疗效。方法回顾性分析2011年1月至2017年12月复旦大学附属华山医院神经外科行手术切除的Broca区胶质瘤患者的临床资料,共42例。术前采用功能磁共振成像和弥散张量成像技术重建语言皮质激活区域和皮质下传导通路,术中在唤醒麻醉下通过直接电刺激技术定位语言皮质和皮质下传导通路,并在术中磁共振、实时神经影像导航系统引导下行肿瘤切除。根据肿瘤是否侵犯中央前回腹侧部(vPMC)将患者分为未侵犯vPMC(仅侵犯额下回后部)组24例,侵犯vPMC组18例,评估并比较两组患者语言功能区定位情况和疗效。结果42例患者均完成术中语言功能区定位,34例(81.0%)至少有1个阳性定位点;3例(7.1%)在定位过程中出现癫痫局灶性发作。25例患者肿瘤为全切除,17例为非全切除。术后病理学证实世界卫生组织(WHO)肿瘤分级Ⅱ级者23例,Ⅲ级者14例,Ⅳ级者5例。术后1个月内(近期)16例(38.1%)患者有语言功能障碍,其中12例3个月内恢复,4例(9.5%)未恢复。术后所有患者随访6~84个月(中位数为24个月),14例出现肿瘤进展,其中11例死亡。与未侵犯vPMC组比较,侵犯vPMC组肿瘤的WHO级别高(P=0.011),术后近期语言功能障碍的发生比率高[分别为10/18、25.0%(6/24),P=0.044],无进展生存期[分别为(28.4±5.2)个月、(80.7±3.2)个月]和总生存期[分别为(38.8±5.8)个月、(80.8±3.2)个月]均短。差异均有统计学意义(均P<0.001)。结论多模态技术联合术中唤醒麻醉治疗Broca区胶质瘤有助于在最大化切除肿瘤的同时,保护患者的语言功能。其中肿瘤侵犯vPMC比仅侵犯额下回后部者引起近期语言功能障碍率更高,预后更差。     

Priming sentence comprehension in aphasia: effects of lexically independent and specific structural priming

Background & Aims: Impaired message-structure mapping results in deficits in both sentence production and comprehension in aphasia. Structural priming has been shown to facilitate syntactic production for persons with aphasia (PWA). However, it remains unknown if structural priming is also effective in sentence comprehension. We examined if PWA show preserved and lasting structural priming effects during interpretation of syntactically ambiguous sentences and if the priming effects occur independently of or in conjunction with lexical (verb) information.

Methods & Procedures: Eighteen PWA and 20 healthy older adults (HOA) completed a written sentence-picture matching task involving the interpretation of prepositional phrases (PP; the chef is poking the solider with an umbrella) that were ambiguous between high (verb modifier) and low attachment (object noun modifier). Only one interpretation was possible for prime sentences, while both interpretations were possible for target sentences. In Experiment 1, the target was presented immediately after the prime (0-lag). In Experiment 2, two filler items intervened between the prime and the target (2-lag). Within each experiment, the verb was repeated for half of the prime-target pairs, while different verbs were used for the other half. Participants’ off-line picture matching choices and response times were measured.

Results: After reading a prime sentence with a particular interpretation, HOA and PWA tended to interpret an ambiguous PP in a target sentence in the same way and with faster response times. Importantly, both groups continued to show this priming effect over a lag (Experiment 2), although the effect was not as reliable in response times. However, neither group showed lexical (verb-specific) boost on priming, deviating from robust lexical boost seen in the young adults of prior studies.

Conclusions: PWA demonstrate abstract (lexically-independent) structural priming in the absence of a lexically-specific boost. Abstract priming is preserved in aphasia, effectively facilitating not only immediate but also longer-lasting structure-message mapping during sentence comprehension.     

汉语失语症测评软件在脑出血后失语评定中的应用

目的探讨中康法汉语标准失语症电脑软件测评系统(CRRCAE)在左侧基底节区脑出血患者语言功能评定及疗效观察中的应用价值。方法对24例左侧基底节区脑出血患者行微创手术治疗,术后行语言康复训练等治疗,并在术前、术后4 w,8 w应用CRRCAE(电脑版)行语言功能评定。结果治疗后与治疗前相比,CRRCAE测评系统各项评分均有明显提高,且随着时间的推移,患者的语言功能明显提高。结论CRRCAE电脑软件测评系统可用于评定脑出血患者语言恢复的程度和特点,能有效的鉴别其改善的程度,具有较好的临床应用价值。     

Persistent verbal and behavioral deficits after resection of the left supplementary motor area in epilepsy surgery

An 8-year-old boy underwent a resection for focal cortical dysplasia at the left supplementary motor area (SMA) for the treatment of intractable epilepsy. The manifestations of SMA syndrome, such as transient mutism and right hemiparesis, resolved within a few weeks. Verbal disfluency and impaired executive function, accompanied by impulsivity and distractibility, persisted for more than 12 months. The verbal and behavioral problems caused serious difficulties in the school life of the patient, until they became less evident at 18 months after surgery. Tractography performed 18 months after surgery revealed a defect in the subportion of fronto-parietal association fibers within the left superior longitudinal fascicles. Verbal influency can persist with unusually long duration after resection of SMA during childhood. Although not discernible on the routine neuroimaging, white matter damage beneath the SMA region could result in serious disabilities in executive function. These complications should be recognized for the prediction and assessment of deficits in children after surgical intervention involving this region.     

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.