Citations

Purpose: When nonnative speaking patterns emerge following neurologic insult or injury, it has often been referred to as foreign, or nonnative, accent syndrome (FAS). In many other cases, FAS can occur as a manifestation of a functional speech disorder (FSD). The purpose of this clinical focus article is to (a) describe the methods utilized in identifying and managing a functional communication disorder characterized by nonnative speech, language, and nonverbal communication intrusions, accompanied by a functional movement disorder and (b) describe the patient's lived experience. Method and Results: The patient is a 59-year-old monolingual English-speaking gentleman with a remote history of episodic stuttering, who later developed an abrupt onset of a nonnative speaking style that changed in quality over time. He also developed gait difficulties that, after visiting with several neurologists, were judged to reflect a functional neurological disorder. During the speech evaluation session, an “Eastern European accent” was noted, along with nonnative word use and word order. A diagnosis of FSD characterized by foreign accent was made, and intervention was undertaken as detailed in the clinical focus article and demonstrated in the accompanying video. With behavioral intervention, the patient was able to correct sound-level changes in structured tasks such as reading and repetition and then in conversation. Language dialect–like changes resolved without direct intervention. The nonspeech movement disorder was addressed with intensive occupational and physical therapy. Conclusions: The patient presented with functional FAS, which was effectively managed with symptomatic, targeted behavioral intervention. His experience demonstrates the importance of patient advocacy in acquiring an accurate diagnosis and appropriate management recommendations. Supplemental Material: https://doi.org/10.23641/asha.24653430

Background: The agrammatic variant of primary progressive aphasia (PAA), primary progressive apraxia of speech (PPAOS), or a combination of both (AOS-PAA) are neurodegenerative disorders characterized by speech-language impairments and together compose the AOS-PAA spectrum disorders. These patients typically have an underlying 4-repeat tauopathy, although they sometimes show evidence of amyloid-β and tau deposition on PET, suggesting Alzheimer’s disease (AD). Given the growing number of pharmacologic treatment options for AD, it is important to better understand the incidence of AD pathology in these patients. Objective: This study aimed to evaluate the frequency of amyloid-β and tau positivity in AOS-PAA spectrum disorders. Sixty-five patients with AOS-PAA underwent a clinical speech-language battery and PiB PET and flortaucipir PET imaging. Methods: Global PiB PET standardized uptake value ratios (SUVRs) and flortaucipir PET SUVRs from the temporal meta region of interest were compared between patient groups. For 19 patients who had died and undergone autopsy, their PET and pathology findings were also compared. Results: The results showed that although roughly half of the patients are positive for at least one biomarker, their clinical symptoms and biomarker status were not related, suggesting that AD is not the primary cause of their neurodegeneration. All but one patient in the autopsy subset had a Braak stage of IV or less, despite four being positive on tau PET imaging. Conclusions: Inclusion criteria for clinical trials should specify clinical presentation or adjust the evaluation of such treatments to be specific to disease diagnosis beyond the presence of certain imaging biomarkers.

Purpose: This case study details the approach to differential diagnosis in characterizing a progressive communication difficulty in a nonnative English-speaking patient. Method: The patient is a multilingual gentleman in his late 60s with a 3-year history of progressive difficulty speaking clearly who presented for evaluation of communication difficulties. Prior to speech evaluation, he was diagnosed with stress-related speech changes by one provider, parkinsonism by another, and primary progressive aphasia by a third provider. A thorough speech and language evaluation, including a detailed case history, was conducted. Results: The patient described a progressive difficulty in enunciating, but not coming up with words; this was present across languages spoken. On exam, the patient exhibited phonetic predominant apraxia of speech, nonverbal oral apraxia, and no more than equivocal aphasia, yielding an overall shared speech and neurologic diagnosis of primary progressive apraxia of speech. Conclusion: This case demonstrates the importance of differential diagnosis of motor speech disorders and a detailed case history, particularly when speech difficulties are the first and only neurological symptom.

AbstractBackgroundThe semantic fluency task involves naming as many items as possible in a given category (e.g., animals) in one minute. It is often interpreted as a measure of word retrieval, but also draws on executive function abilities to organize responses and develop task‐specific strategies. Indeed, both language skills and dementia severity ratings have recently been found to correlate with semantic fluency performance in patients with primary progressive aphasia (Riello et al., 2022; Scheffel et al., 2021). Here, we focused instead on how performance on language and executive function measures predicts longitudinal change in semantic fluency performance in patients with progressive agrammatic aphasia and/or apraxia of speech (AOS).MethodPatients with isolated agrammatic aphasia (n = 10), isolated AOS (n = 42), or a combination of the two (n = 59) with 1‐7 visits (mean = 2.19) were included. We used Bayesian hierarchical linear models to investigate whether baseline measures of executive function or language were associated with the rate at which semantic fluency performance changes over time while accounting for baseline diagnosis group, sex, education, age at baseline, and baseline disease duration.ResultSemantic fluency performance declined over time, at a similar rate irrespective of diagnostic group (isolated or combined aphasia or AOS; Figure 1). Across all patients, higher executive function abilities at baseline, as indicated by faster Trails Making Test B completion times, predicted higher baseline semantic fluency performance and also a steeper annual rate of decline (Figure 2). Similarly, higher fluency ratings during the Western Aphasia Battery‐Revised picture description at baseline were associated with higher baseline semantic fluency performance and faster decline. Baseline performance on the Boston Naming Test and Token Test were also associated with baseline semantic fluency, but not with longitudinal change.ConclusionThese results better delineate how language and executive function abilities support performance in the semantic fluency task. Higher abilities in those areas at baseline do not appear to be protective over time. Rather, the differences in semantic fluency that are present at baseline disappear as the disease progresses.