Purpose: Prior studies have shown that communication-related participation restrictions in patients with degenerative disease do not always match clinician judgment or objective indices of symptom severity. Although there is a growing body of literature documenting that discrepancies between patients with dementia and their care partners' perception of participation restrictions exist, it is not known how care partner perceptions of communication participation restrictions specifically match or diverge from the patients' experiences, which may inform the use of care partner proxy in the context of degenerative diseases. Method: Thirty-eight patients with progressive neurologic conditions (progressive supranuclear palsy, corticobasal syndrome, and primary progressive aphasia or apraxia of speech) and, in most instances, focal cognitive-communication disorders were included. The patients and their accompanying care partners independently completed the Communicative Participation Item Bank, short form, a 10-question survey about communication participation restrictions in different contexts. Care partners were instructed to complete the form with their perception of the patient's experience. The difference between patient and care partner total scores were calculated and analyzed relative to clinical and demographic variables of interest. Results: Care partner ratings modestly tracked with patient experience and objective indices of symptom severity but did not exactly match patient ratings. The presence of aphasia increased, but did not fully account for, the likelihood of a discrepancy between care partner and patient ratings. Conclusion: Although careful consideration should be given prior to using care-partner report as a proxy for patient experience, it is worthwhile to include care partner ratings as a means of supporting conversations about differing perceptions, guiding joint intervention planning, and monitoring care-partner perceptions of change along with the implementation of supported conversation strategies.
The Apraxia of Speech Rating Scale: Reliability, Validity, and Utility
Purpose: The purpose of this study was to examine the interrater reliability and validity of the Apraxia of Speech Rating Scale (ASRS-3.5) as an index of the presence and severity of apraxia of speech (AOS) and the prominence of several of its important features. Method: Interrater reliability was assessed for 27 participants. Validity was examined in a cohort of 308 participants (120 with and 188 without progressive AOS) through item analysis; item-Total score correlations; correlations among ASRS Total score and component subscores and independent clinical ratings of AOS, dysarthria and aphasia severity, intelligibility, and articulatory errors, as well as years postonset and age; and regression models assessing item and Total score prediction of AOS presence. Results: Interrater reliability was good or excellent for most items and excellent for the Total score. Item and Total score analyses revealed good separation of participants with versus without AOS. Inter-item and item-Total score correlations were generally moderately high as were correlations between the ASRS Total score and independent ratings of AOS severity, intelligibility, and articulatory errors. The Total score was not meaningfully correlated with ratings of aphasia and dysarthria severity, years postonset, or age. Total scores below 7 and above 10 revealed excellent diagnostic sensitivity and specificity for AOS. The presence of eight or more abnormal features was also highly predictive of AOS presence. Conclusions: The ASRS-3.5 is a reliable and valid scale for identifying the presence and severity of AOS and its predominant features. It has excellent sensitivity to AOS presence and excellent specificity relative to aphasia and dysarthria in patients with neurodegenerative disease. Supplemental Material: https://doi.org/10.23641/asha.21817584
A Preliminary Report of Network Electroencephalographic Measures in Primary Progressive Apraxia of Speech and Aphasia
The objective of this study was to characterize network-level changes in nonfluent/agrammatic Primary Progressive Aphasia (agPPA) and Primary Progressive Apraxia of Speech (PPAOS) with graph theory (GT) measures derived from scalp electroencephalography (EEG) recordings. EEGs of 15 agPPA and 7 PPAOS patients were collected during relaxed wakefulness with eyes closed (21 electrodes, 10–20 positions, 256 Hz sampling rate, 1–200 Hz bandpass filter). Eight artifact-free, non-overlapping 1024-point epochs were selected. Via Brainwave software, GT weighted connectivity and minimum spanning tree (MST) measures were calculated for theta and upper and lower alpha frequency bands. Differences in GT and MST measures between agPPA and PPAOS were assessed with Wilcoxon rank-sum tests. Of greatest interest, Spearman correlations were computed between behavioral and network measures in all frequency bands across all patients. There were no statistically significant differences in GT or MST measures between agPPA and PPAOS. There were significant correlations between several network and behavioral variables. The correlations demonstrate a relationship between reduced global efficiency and clinical symptom severity (e.g., parkinsonism, AOS). This preliminary, exploratory study demonstrates potential for EEG GT measures to quantify network changes associated with degenerative speech–language disorders.
This study compared orofacial muscle strength between normal and dysarthric speakers and across types of dysarthria, and examined correlations between strength and dysarthria severity. Participants included 79 speakers with flaccid, spastic, mixed spastic–flaccid, ataxic, or hypokinetic dysarthria and 33 healthy controls. Maximum pressure generation (Pmax) by the tongue, lips, and cheeks represented strength. Pmax was lower for speakers with mixed spastic–flaccid dysarthria for all tongue and lip measures, as well as for speakers with flaccid or spastic dysarthria for anterior tongue elevation and lip compression. Anterior tongue elevation and cheek compression tended to be lower than normal for the hypokinetic group. Pmax did not differ significantly between controls and speakers with ataxic dysarthria on any measure. Correlations were generally weak between dysarthria severity and orofacial weakness but were stronger in the dysarthria groups with more prominent orofacial weakness. The results generally support predictions that orofacial weakness accompanies flaccid and/or spastic dysarthria but not ataxic dysarthria. The findings support including type of dysarthria as a variable of interest when examining orofacial weakness in motor speech disorders.
Characterizing Anarthria to Facilitate Neurological Diagnosis: A Case Illustration
Purpose: The purpose of this clinical focus article is to illustrate how speech-language pathologist (SLP) characterization of anarthria can contribute to neurological diagnosis and to highlight the challenges associated with such an endeavor. Method: Used in this study are a retrospective chart review and clinicians' experience-based reflections. Results: A 65-year-old man, who, in the context of a neurodegenerative disease, presented with near-complete-loss of speech, was referred by neurologists to SLPs for further characterization of his speech difficulty. Assessment of his limited speech output revealed anarthria with mixed features (spastic and hypokinetic) with superimposed apraxia of speech. Conclusions: SLP characterization of anarthria to facilitate neurological diagnosis is challenging but possible. Clinical lessons learned from this unusual scenario are discussed.
Assessing Change in Communication Limitations in Primary Progressive Apraxia of Speech and Aphasia: A 1-Year Follow-Up Study
Purpose Individuals with primary progressive apraxia of speech have apraxia of speech (AOS) as the initial and predominant symptom. Many develop aphasia and/or dysarthria later in the disease course. It was previously demonstrated that patients with neurodegenerative AOS experience reduced participation in communication that is further exacerbated by co-occurring language deficits ( Utianski et al., 2020 ). Measures of disease severity did not necessarily correlate with measures of participation restrictions. The aim of this follow-up study was to describe changes in communication limitations in these patients, again measured by (a) the patient via the Communicative Participation Item Bank (CPIB) and (b) the speech-language pathologist via the American Speech-Language-Hearing Association's (ASHA's) Functional Communication Measures (FCMs) and an adapted motor speech disorder (MSD) severity rating to determine if there are significant changes in these and other objective speech and language measures at follow-up after 1 year. Method Of the 24 patients reported in the study of Utianski et al. (2020) , 17 (10 men, seven women) returned for a second visit approximately 1 year following the first visit. Identical procedures were utilized; the communication measures collected at each visit were statistically compared. Correlations were calculated between the participation ratings and other clinical assessment measures at the second visit and for the change in scores on those measures between the first and second visits. Results There were statistically significant differences in AOS and aphasia severity between visits. There were significant changes in clinical assessments, MSD severity rating, and all ASHA FCMs between visits, but not the CPIB. Correlation analyses suggest the relationships among clinical and participation measures are complex; overall, patients with more severe changes in AOS experienced greater changes in participation restrictions. Conclusions The findings of this study support the use of patient-reported outcome measures as they may better reflect the patient experience, including the influence of factors such as ongoing speech therapy and the emergence of neuropsychiatric features, and associated changes in day-to-day functioning, when other measures may simply index the progression of the disease. Supplemental Material https://doi.org/10.23641/asha.16528512
Distinguishing Spastic and Dystonic Features in a Patient With a History of Brainstem Encephalitis: A Clinical Case Study
Purpose One role of a speech-language pathologist (SLP) is to differentially diagnose motor speech disorders to aid physicians, typically neurologists, in establishing a medical diagnosis. The present case study describes the clinical problem solving undertaken by the SLP to characterize the nature of neurological deficits to develop an appropriate management plan. Method The patient is a 29-year-old man with a history of brainstem encephalitis who presented for evaluation of dysarthria, dysphagia, and gait difficulties. The neurological exam characterized a spastic dysarthria. The SLP conducted a motor speech examination expressly for the purpose of differential diagnosis. Results The patient exhibited features of spastic dysarthria including strained phonation and hypernasality, equivocally slow rate associated with intermittent interword pauses rather than lengthened segments, and articulatory imprecision in the absence of weakness or slowness of the articulators. Defining features of spastic dysarthria absent from his speech were monopitch and monoloudness, as well as slow alternate motion rates. A diagnosis of hyperkinetic dysarthria associated with dystonia was informed by (a) hypernasality disproportionate to other deviant speech features, with preserved reflexive velar movements and nonspeech movements; (b) adventitious lip pursing; (c) modest improvement in speech with sensory tricks; (d) mild and intermittent phonatory strain; and (e) normal nonspeech oral motor function. Conclusions This case demonstrates the role speech-language pathology can play in the neurological assessment and management process. The recognition by the SLP of hyperkinetic dysarthria led to additional diagnostic tests and to a broader range of options for medical management.
Facilitating End-of-Life Interaction Between Patients With Severe Communication Impairment and Their Families
Purpose The purpose of this report is to promote conversation among medical speech-language pathologists (SLPs) about their role in facilitating family–patient interaction involving patients who acutely encounter the end-of-life (EOL) phase and have severe communication impairment. Case Report We provide self-reflections from our own clinical practice, pertinent literature review, and medical chart review of a relevant patient to serve as a catalyst for such discussion. Reflections We share a preliminary handout containing strategies that SLPs could provide family members of patients who have encountered an EOL phase and have severe communication impairment (e.g., global aphasia) and reduced alertness level. Clinical Implications SLPs can play an important role in facilitating EOL patient–family interaction and alleviate suffering even when patients have severe communication impairment.
Motor Speech Disorders and Communication Limitations in Progressive Supranuclear Palsy
Purpose This study describes motor speech disorders and associated communication limitations in six variants of progressive supranuclear palsy (PSP). Method The presence, nature, and severity of dysarthria and apraxia of speech (AOS) were documented, along with scores on the Apraxia of Speech Rating Scale–Version 3 (ASRS-3) for 77 (40 male and 37 female) patients with PSP. Clinician-estimated and patient-estimated communication limitations were rated using the Motor Speech Disorders Severity Rating (MSDSR) Scale and the Communicative Effectiveness Survey (CES), respectively. Descriptive statistics were calculated for each of these dependent variables. One-tailed t tests were conducted to test mean differences in ASRS-3 and CES between participants with and without AOS and between participants with and without dysarthria. Spearman rank correlations were calculated between ASRS-3 scores and clinical judgments of AOS and dysarthria severity and between MSDSR and CES ratings. Results Nine participants (12%) had normal speech. Eighty-seven percent exhibited dysarthria; hypokinetic and mixed hypokinetic–spastic dysarthria were observed most frequently. AOS was observed in 19.5% of participants across all variants, but in only 10% exclusive of the PSP speech and language variant. Nearly half presented with AOS in which neither phonetic nor prosodic features clearly predominated. The mean ASRS-3 score for participants with AOS was significantly higher than for those without and correlated strongly with clinician judgment of AOS severity. Mean ASRS-3 was higher for participants with dysarthria than for those without but correlated weakly with dysarthria severity. Mean MSDSR and CES ratings were lower in participants with AOS compared to those without and moderately correlated with each other. Conclusions Motor speech disorders that negatively impact communicative effectiveness are common in PSP and occur in many variants. This is the first description of motor speech disorders across PSP variants, setting the stage for future research characterizing neuroanatomical correlates, progression of motor speech disorders, and benefits of targeted interventions. Supplemental Material https://doi.org/10.23641/asha.14111837
A Longitudinal Evaluation of Speech Rate in Primary Progressive Apraxia of Speech
Purpose Individuals with primary progressive apraxia of speech (PPAOS) have apraxia of speech (AOS) in which disruptions in articulation or prosody predominate the speech pattern, referred to, respectively, as phonetic or prosodic subtypes. Many develop aphasia and/or dysarthria. Past research has demonstrated that simple temporal acoustic measures are sensitive to the presence of AOS. The aim of this study was to describe the change in temporal acoustic measures over time and assess if specific patterns of AOS or co-occurring aphasia or dysarthria impact the rate of change over time. Method Durations for multiple productions of the words cat , catnip , catapult , and catastrophe , in an imitative speech task, were recorded for 73 patients, with two to six visits each. A linear mixed-effects model was used to assess the cross-sectional differences and longitudinal influence of AOS subtype and presence of aphasia/dysarthria on speech rate. Pearson correlations were calculated between rate measures and performance on other clinical measures. Results Cross-sectionally, patients with prosodic-predominant PPAOS produced words more slowly than those with phonetic-predominant PPAOS. Patients with either aphasia or dysarthria produced words more slowly than those without. Longitudinally, the speech rate of patients with phonetic-predominant PPAOS had a reduction of 0.5 syllables per second per year. Patients with prosodic-predominant AOS changed less quickly, as did those who developed aphasia. Dysarthria did not impact rate of change. There were strong associations between speech rate measures and other clinical indices of speech and language functioning. Conclusion Simple temporal acoustic measures may reflect the subtype of AOS (phonetic or prosodic predominant), serve as an index of progression of AOS, and inform prognostication relative to the presenting combination of speech and language features. Supplemental Material https://doi.org/10.23641/asha.13564724