Smartphone use as an efficient tool to improve anomia in primary progressive aphasia.

Cognitive interventions are helpful in the non-pharmacological management of Primary progressive aphasia (PPA) and other neurodegenerative disorders of cognition, by helping patients to compensate for their cognitive deficits and improve their functional independence. In this study, we examined the effectiveness of cognitive rehabilitation based on the use of mobile device technology in PPA. The aim of this research study was to determine if BL, a patient with semantic variant PPA (svPPA) and severe anomia, was able to learn using specific smartphone functions and an application to reduce her word finding difficulties. She was trained during the intervention sessions on a list of target pictures to measure changes in picture naming performance. Errorless learning was applied during learning. BL quickly learned to use smartphone functions and the application over the course of the intervention. She significantly improved her anomia for trained pictures, and to a lesser extent for untrained semantically related pictures. Picture naming performance was maintained six months after the intervention, and she continued to use her smartphone regularly to communicate with family members and friends. This study confirms that smartphone use can be learned in PPA, which can help reduce the symptoms of anomia and improve communication skills.

White Matter Integrity Predicts Electrical Stimulation (tDCS) and Language Therapy Effects in Primary Progressive Aphasia.

BACKGROUND: Transcranial direct current stimulation (tDCS), in conjunction with language therapy, improves language therapy outcomes in primary progressive aphasia (PPA). However, no studies show whether white matter integrity predicts language therapy or tDCS effects in PPA. OBJECTIVE: We aimed to determine whether white matter integrity, measured by diffusion tensor imaging (DTI), predicts written naming/spelling language therapy effects (letter accuracy on trained and untrained words) with and without tDCS over the left inferior frontal gyrus (IFG) in PPA. METHODS: Thirty-nine participants with PPA were randomly assigned to tDCS or sham condition, coupled with language therapy for 15 daily sessions. White matter integrity was measured by mean diffusivity (MD) and fractional anisotropy (FA) in DTI scans before therapy. Written naming outcomes were evaluated before, immediately after, 2 weeks, and 2 months posttherapy. To assess tDCS treatment effect, we used a mixed-effects model with treatment evaluation and time interaction. We considered a forward model selection approach to identify brain regions/fasciculi of which white matter integrity can predict improvement in performance of word naming. RESULTS: Both sham and tDCS groups significantly improved in trained items immediately after and at 2 months posttherapy. Improvement in the tDCS group was greater and generalized to untrained words. White matter integrity of ventral language pathways predicted tDCS effects in trained items whereas white matter integrity of dorsal language pathways predicted tDCS effects in untrained items. CONCLUSIONS: White matter integrity influences both language therapy and tDCS effects. Thus, it holds promise as a biomarker for deciding which patients will benefit from language therapy and tDCS.

Errorless Learning Therapy in Semantic Variant of Primary Progressive Aphasia.

BACKGROUND: The semantic variant of primary progressive aphasia (svPPA) is characterized by a progressive loss of semantic knowledge impairing the ability to name and to recognize the meaning of words. OBJECTIVE: We aimed to evaluate the immediate and short-term effect of errorless learning speech therapy on the naming and recognition of commonly used words in patients with svPPA. METHODS: Eight participants diagnosed with svPPA received 16 sessions of intensive errorless learning speech therapy. Naming and word comprehension tasks were evaluated at baseline, immediately postintervention, and at follow-up after 1, 3, and 6 months. These evaluations were performed using two item sets (a trained list and an untrained list). RESULTS: In the naming tasks, patients showed a significant improvement in trained items immediately after the intervention, but that improvement decayed progressively when therapy ended. No improvements were found either in trained comprehension or in untrained tasks. CONCLUSION: Errorless learning therapy could improve naming ability in patients with svPPA. This effect may be due to the relative preservation of episodic memory, but the benefit is not maintained over time, presumably because there is no consolidation.

Improvement in functional vocabulary and generalization to conversation following a self-administered treatment using a smart tablet in primary progressive aphasia.

Currently, public services in speech-language pathology for primary progressive aphasia (PPA) are very limited, although several interventions have been shown to be effective. In this context, new technologies have the potential to enable people with PPA to improve their communication skills. The main aim of this study was to investigate the efficacy of a self-administered therapy using a smart tablet to improve naming of functional words and to assess generalization to an ecological conversation task. Five adults with PPA completed the protocol. Using an ABA design with multiple baselines, naming performance was compared across four equivalent lists: (1) trained with functional words; (2) trained with words from a picture database; (3) exposed but not trained; and (4) not exposed (control). Treatment was self-administered four times a week for a period of four consecutive weeks. A significant improvement for trained words was found in all five participants, and gains were maintained two months post-treatment in four of them. Moreover, in three participants, evidence of generalization was found in conversation. This study supports the efficacy of using a smart tablet to improve naming in PPA and suggests the possibility of generalization to an ecological context.

Treatment for spoken and written word retrieval in the semantic variant of primary progressive aphasia.

This study investigated whether a treatment programme for spoken word retrieval, supplemented with written naming, was beneficial for an individual with right-hemisphere dominant semantic variant of PPA (svPPA). Assessment and treatment were delivered remotely through Skype. Treatment consisted of two phases of lexical retrieval therapy (Repetition and Reading in the Presence of a Picture: RRIPP), with and without written responses (Phases 1 and 2 respectively), and a third treatment phase based on the procedures of Conceptual Enrichment (COEN) therapy. The first two phases of treatment resulted in short-lasting improvements in spoken and written word retrieval, with greater improvement in Phase 2 when written production was also required. Both treatment phases resulted in gains only for treated items, but generalised to different depictions to those treated. However, Phase 2 also resulted in significant improvement of treated items on a comprehension task. COEN treatment did not result in significant gains in word retrieval or comprehension. This study reinforces the value of a simple lexical retrieval treatment delivered remotely. It adds to the current evidence that anomia in svPPA can be responsive to treatment, but also shows that challenges remain regarding maintenance effects and the generalisation of treatment effects to connected speech.

Lexical retrieval treatment in primary progressive aphasia: An investigation of treatment duration in a heterogeneous case series.

Word-finding difficulty is typically an early and frustrating symptom of primary progressive aphasia (PPA), prompting investigations of lexical retrieval treatment in PPA. This study aimed to investigate immediate treatment gains following two versus four weeks of treatment, item generalisation, and maintenance of gains with ongoing treatment in a single case series of eight individuals with heterogeneous PPA presentations (three non-fluent/agrammatic, two logopenic, two semantic, and one mixed PPA). Three individuals made initial gains in picture naming and maintained them over 6 months or more with ongoing treatment. By contrast, three individuals made marginal initial gains but were unable to continue treatment, and two individuals did not make the typically-reported initial gains with two or four weeks of treatment. There was little evidence of generalisation to untreated items. Our results add to the evidence that daily home practice of Repetition and Reading in the Presence of a Picture over extended periods can increase and maintain retrieval of personally-relevant words in picture naming for some individuals with semantic or nonfluent/agrammatic variant PPA. Further research is needed into the factors associated with long-term treatment adherence and gains, and the factors associated with nonadherence to treatment.

Long-Term maintenance of anomia treatment effects in primary progressive aphasia.

This study examined the maintenance of anomia treatment effects in primary progressive aphasia (PPA). Following baseline testing, a phonological treatment and an orthographic treatment were administered over the course of six months. The treatment stimuli consisted of nouns that were consistently named correctly at baseline (Prophylaxis items) and/or nouns that were consistently named incorrectly at baseline (Remediation items). Naming accuracy was measured at baseline, and it was measured at 1 month, 8 months, and 15 months post-treatment. The change in naming accuracy from baseline to each post-treatment evaluation was calculated within each treatment condition, and within a matched untrained condition. The change in naming accuracy was then compared between the three conditions. The results of these analyses indicate that phonological and orthographic treatments are both effective in the Prophylaxis and Remediation of anomia in all three variants of PPA. For Prophylaxis items, some of the effects of each treatment can persist for as long as 15 months post-treatment. These long-term treatment effects were more robust in the orthographic treatment condition and for participants with the semantic variant of PPA.

Comparing the effects of clinician and caregiver-administered lexical retrieval training for progressive anomia.

There is a growing body of literature indicating that lexical retrieval training can result in improved naming ability in individuals with neurodegenerative disease. Traditionally, treatment is administered by a speech-language pathologist, with little involvement of caregivers or carry-over of practice into the home. This study examined the effects of a lexical retrieval training programme that was implemented first by a clinician and, subsequently, by a trained caregiver. Two dyads, each consisting of one individual with anomia caused by neurodegenerative disease (one with mild cognitive impairment and one with logopenic primary progressive aphasia) and their caregiver, participated in the study. Results indicated medium and large effect sizes for both clinician- and caregiver-trained items, with generalisation to untrained stimuli. Participants reported improved confidence during communication as well as increased use of trained communication strategies after treatment. This study is the first to document that caregiver-administered speech and language intervention can have positive outcomes when paired with training by a clinician. Caregiver-administered treatment may be a viable means of increasing treatment dosage in the current climate of restricted reimbursement, particularly for patients with progressive conditions.

Retraining speech production and fluency in non-fluent/agrammatic primary progressive aphasia.

The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) presents with a gradual decline in grammar and motor speech resulting from selective degeneration of speech-language regions in the brain. There has been considerable progress in identifying treatment approaches to remediate language deficits in other primary progressive aphasia variants; however, interventions for the core deficits in nfvPPA have yet to be systematically investigated. Further, the neural mechanisms that support behavioural restitution in the context of neurodegeneration are not well understood. We examined the immediate and long-term benefits of video implemented script training for aphasia (VISTA) in 10 individuals with nfvPPA. The treatment approach involved repeated rehearsal of individualized scripts via structured treatment with a clinician as well as intensive home practice with an audiovisual model using 'speech entrainment'. We evaluated accuracy of script production as well as overall intelligibility and grammaticality for trained and untrained scripts. These measures and standardized test scores were collected at post-treatment and 3-, 6-, and 12-month follow-up visits. Treatment resulted in significant improvement in production of correct, intelligible scripted words for trained topics, a reduction in grammatical errors for trained topics, and an overall increase in intelligibility for trained as well as untrained topics at post-treatment. Follow-up testing revealed maintenance of gains for trained scripts up to 1 year post-treatment on the primary outcome measure. Performance on untrained scripts and standardized tests remained relatively stable during the follow-up period, indicating that treatment helped to stabilize speech and language despite disease progression. To identify neural predictors of responsiveness to intervention, we examined treatment effect sizes relative to grey matter volumes in regions of interest derived from a previously identified speech production network. Regions of significant atrophy within this network included bilateral inferior frontal cortices and supplementary motor area as well as left striatum. Volumes in a left middle/inferior temporal region of interest were significantly correlated with the magnitude of treatment effects. This region, which was relatively spared anatomically in nfvPPA patients, has been implicated in syntactic production as well as visuo-motor facilitation of speech. This is the first group study to document the benefits of behavioural intervention that targets both linguistic and motoric deficits in nfvPPA. Findings indicate that behavioural intervention may result in lasting and generalized improvement of communicative function in individuals with neurodegenerative disease and that the integrity of spared regions within the speech-language network may be an important predictor of treatment response.

Behavioural and neuroimaging changes after naming therapy for semantic variant primary progressive aphasia.

The objectives of this study were to examine the effects of a successful naming intervention on naming performance and brain activity in individuals with the semantic variant of primary progressive aphasia (svPPA). Four participants with svPPA were scanned while performing phonologically- and semantically-based tasks before and after an intense, 20-h naming therapy that followed the principles of errorless learning whereby errors were eliminated from the learning process. Five healthy control participants were scanned at the outset of the study and did not receive treatment. The results showed that in svPPA participants, successful re-learning of forgotten vocabulary was accompanied by activation of a larger network in bilateral brain regions and that the level of activation in the left anterior lobe may be inversely correlated with severity of semantic impairment. Our findings have implications for treatment in svPPA patients and suggest that semantic cues can improve naming, in spite of significant semantic impairment. The results indicate that intensive language therapy can lead to behavioural gains and neuroplastic changes even in individuals with more advanced anterior temporal lobe atrophy.

Exploring generalisation processes following lexical retrieval intervention in primary progressive aphasia.

PURPOSE: Within the current literature, positive intervention effects demonstrate the significant potential for people with Primary Progressive Aphasia (PPA) to learn/relearn words. Generalisation of intervention effects to other words and/or other contexts, however, remains unclear. METHOD: This multiple baseline, case-series design investigated the effects of a self-cueing lexical retrieval intervention across word classes (nouns, verbs and adjectives) on four individuals with PPA, three suggestive of the semantic variant and one of the logopenic variant. The intervention integrated semantic, phonological and orthographic levels of language production and drew on autobiographical memory. Changes in accuracy in retrieving treated and untreated items (pre-intervention, post-intervention and 4-weeks maintenance) were determined using the Cochran's Q test, with follow-up McNemar pairwise comparisons. RESULT: All participants showed significant improvements in naming treated items, across all word classes. Different patterns of generalised improvement to untreated words were found for each participant. In discourse, the semantic variant participants demonstrated a significant increase in correct information units, in contrast to the participant with the logopenic variant who remained stable. CONCLUSION: This study provides evidence that people with PPA can show improved lexical retrieval following intervention. The findings suggest possible differences in generalisation across word classes and according to underlying deficit.

Novel integrative virtual rehabilitation reduces symptomatology of primary progressive aphasia--a case report.

PURPOSE: BrightBrainer™ integrative cognitive rehabilitation system evaluation in an Adult Day Program by a subject with Primary Progressive Aphasia (PPA) assumed to be of the mixed nonfluent/logopenic variant, and for determination of potential benefits. METHODS: The subject was a 51-year-old Caucasian male diagnosed with PPA who had attended an Adult Day Program for 18 months prior to BrightBrainer training. The subject interacted with therapeutic games using a controller that measured 3D hand movements and flexion of both index fingers. The computer simulations adapted difficulty level based on task performance; results were stored on a remote server. The clinical trial consisted of 16 sessions, twice/week for 8 weeks. The subject was evaluated through neuropsychological measures, therapy notes and caregiver feedback forms. RESULTS: Neuropsychological testing indicated no depression (BDI 0) and severe dementia (BIMS 1 and MMSE 3). The 6.5 h of therapy consisted of games targeting Language comprehension; Executive functions; Focusing; Short-term memory; and Immediate/working memory. The subject attained the highest difficulty level in all-but-one game, while averaging 1300-arm task-oriented active movement repetitions and 320 index finger flexion movements per session. While neuropsychological testing showed no benefits, the caregiver reported strong improvements in verbal responses, vocabulary use, speaking in complete sentences, following one-step directions and participating in daily activities. This corroborated well with therapy notes. CONCLUSIONS: Preliminary findings demonstrate a meaningful reduction of PPA symptoms for the subject, suggesting follow-up imaging studies to detail neuronal changes induced by BrightBrainer system and controlled studies with a sufficiently large number of PPA subjects.

Improved verbal learning in the semantic variant of primary progressive aphasia when using semantic cues.

The semantic variant of primary progressive aphasia (PPA-S) is characterized by impairments in confrontation naming and single word comprehension. Although episodic memory may be relatively spared, there can be impairment in verbal learning tasks. We report a patient with PPA-S and impaired verbal learning who was tested to learn if when provided with semantic categories, her learning would improve. A 70-year-old right-handed woman with a 2-year history of progressive difficulties with word finding, naming, and memory was tested for language and memory deficits using the Hopkins Verbal Learning Test-Revised (HVLT-R). She was then retested with the HVLT-R after being provided with the three semantic categories to which these words belonged. Confrontation naming was impaired on the Boston Naming Test. Sentence repetition was normal. Comprehension testing with word picture matching and sentence comprehension was normal. On a test of semantic associations, Pyramids and Palm Trees, she was impaired. She was also impaired on tests of verbal learning (HVLT-R) (total: 13) but not recall. When a different version of the HVLT-R was given with the semantic categories of the words given beforehand, her scores improved (total: 26). This patient with PPA-S had an impairment of verbal learning, but not delayed recall. When given a semantic category cue beforehand, her verbal learning performance improved. This observation suggests that this patient did not spontaneously use semantic encoding. Using a semantic cueing strategy may help other patients with PPA-S improve their capacity for verbal learning.

Examining the value of lexical retrieval treatment in primary progressive aphasia: two positive cases.

Individuals with primary progressive aphasia (PPA) suffer a gradual decline in communication ability as a result of neurodegenerative disease. Language treatment shows promise as a means of addressing these difficulties but much remains to be learned with regard to the potential value of treatment across variants and stages of the disorder. We present two cases, one with semantic variant of PPA and the other with logopenic PPA, each of whom underwent treatment that was unique in its focus on training self-cueing strategies to engage residual language skills. Despite differing language profiles and levels of aphasia severity, each individual benefited from treatment and showed maintenance of gains as well as generalization to untrained lexical items. These cases highlight the potential for treatment to capitalize on spared cognitive and neural systems in individuals with PPA, improving current language function as well as potentially preserving targeted skills in the face of disease progression.

Positive effects of language treatment for the logopenic variant of primary progressive aphasia.

Despite considerable recent progress in understanding the underlying neurobiology of primary progressive aphasia (PPA) syndromes, relatively little attention has been directed toward the examination of behavioral interventions that may lessen the pervasive communication problems associated with PPA. In this study, we report on an individual with a behavioral profile and cortical atrophy pattern consistent with the logopenic variant of PPA. At roughly two-and-a-half years post onset, his marked lexical retrieval impairment prompted administration of a semantically based intervention to improve word retrieval. The treatment was designed to improve self-directed efforts to engage the participant's relatively preserved semantic system in order to facilitate word retrieval. His positive response to an intensive (2-week) dose of behavioral treatment was associated with improved lexical retrieval of items within trained categories, and generalized improvement for naming of untrained items that lasted over a 6-month follow-up interval. These findings support the potential value of intensive training to achieve self-directed strategic compensation for lexical retrieval difficulties in logopenic PPA. Additional insight was gained regarding the neural regions that supported improved performance by the administration of a functional magnetic resonance imaging protocol before and after treatment. In the context of a picture-naming task, post-treatment fMRI showed increased activation of left dorsolateral prefrontal regions that have been implicated in functional imaging studies of generative naming in healthy individuals. The increased activation in these frontal regions that were not significantly atrophic in our patient (as determined by voxel-based morphometry) is consistent with the notion that neural plasticity can support compensation for specific language loss, even in the context of progressive neuronal degeneration.

Effects of alternative communication on the communicative effectiveness of an individual with a progressive language disorder.

This study was designed to investigate the effects of two different modes of communication on the communicative output of an individual who is no longer able to communicate verbally, presenting with a primary progressive aphasia and apraxia of speech. The two treatment approaches included training the patient with a text-to-speech alternative communication device and with American sign language. An alternating treatment design was used to compare two communicative approaches (an alternative communication device and American sign language) on the subject's communicative effectiveness. Communicative effectiveness was measured in terms of number of words, correct information units and percentage correct information units, using a protocol that was adapted to quantify the output generated by the alternative communication device and American sign language. Increases across all three measures resulted for both the alternative communication device and American sign language. The clinical implications are explored, and the results add to existing studies regarding treatment possibilities using alternative communication for individuals who present with a progressive speech and language disorder, without concomitant cognitive deficits.