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.     

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.     

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.     

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.     

Motor speech disorders associated with primary progressive aphasia

Background: Primary progressive aphasia (PPA) and conditions that overlap with it can be accompanied by motor speech disorders. Recognition and understanding of motor speech disorders can contribute to a fuller clinical understanding of PPA and its management as well as its localisation and underlying pathology.

Aims: To review the types of motor speech disorders that may occur with PPA, its primary variants, and its overlap syndromes (progressive supranuclear palsy syndrome, corticobasal syndrome, motor neuron disease), as well as with primary progressive apraxia of speech.

Main Contribution: The review should assist clinicians’ and researchers’ understanding of the relationship between motor speech disorders and PPA and its major variants. It also highlights the importance of recognising neurodegenerative apraxia of speech as a condition that can occur with little or no evidence of aphasia.

Conclusion: Motor speech disorders can occur with PPA. Their recognition can contribute to clinical diagnosis and management of PPA and to understanding and predicting the localisation and pathology associated with PPA variants and conditions that can overlap with them.     

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.