Language-Independent Acoustic Biomarkers for Quantifying Speech Impairment in Huntington's Disease.

PURPOSE: Changes in voice and speech are characteristic symptoms of Huntington's disease (HD). Objective methods for quantifying speech impairment that can be used across languages could facilitate assessment of disease progression and intervention strategies. The aim of this study was to analyze acoustic features to identify language-independent features that could be used to quantify speech dysfunction in English-, Spanish-, and Polish-speaking participants with HD. METHOD: Ninety participants with HD and 83 control participants performed sustained vowel, syllable repetition, and reading passage tasks recorded with previously validated methods using mobile devices. Language-independent features that differed between HD and controls were identified. Principal component analysis (PCA) and unsupervised clustering were applied to the language-independent features of the HD data set to identify subgroups within the HD data. RESULTS: Forty-six language-independent acoustic features that were significantly different between control participants and participants with HD were identified. Following dimensionality reduction using PCA, four speech clusters were identified in the HD data set. Unified Huntington's Disease Rating Scale (UHDRS) total motor score, total functional capacity, and composite UHDRS were significantly different for pairwise comparisons of subgroups. The percentage of HD participants with higher dysarthria score and disease stage also increased across clusters. CONCLUSION: The results support the application of acoustic features to objectively quantify speech impairment and disease severity in HD in multilanguage studies. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25447171.

Progressive midbrain clefts after head trauma and decompressive surgery: a report of two patients.

This report describes two patients with acute-onset ptosis, oculomotor dysfunction, ataxia and drowsiness, referable to the midbrain tegmentum. Both patients had previously suffered severe closed head injuries requiring craniotomy for cerebral decompression. Serial brain scans in both cases revealed a newly developing cleft in the midbrain, with features suggestive of abnormal cerebrospinal fluid (CSF) flow across the aqueduct. A trial of acetazolamide was initiated to reduce CSF production, followed by a third ventriculostomy for CSF diversion in one patient, which resulted in arrested disease progression and partial recovery. There are only two previous reports in the literature of midbrain clefts that developed as remote sequelae of head trauma. We postulate that altered CSF flow dynamics in the aqueduct, possibly related to changes in brain compliance, may be contributory. Early recognition and treatment may prevent irreversible structural injury and possible death.

Metronidazole-induced reversible cerebellar dysfunction.

A 73-year-old man who presented with fever and abdominal discomfort was diagnosed to have a liver abscess. He was treated with antimicrobials which included metronidazole. One month into treatment, he developed neurological symptoms and signs that were suggestive of cerebellar pathology. MRI of the brain showed T2/fluid attenuated inversion recovery hyperintensities involving bilateral dentate, fastigial and interpositus nuclei. After excluding common aetiologies, the possibility of metronidazole-induced neurotoxicity was considered. After stopping metronidazole, his symptoms and signs resolved. A subsequent MRI scan of the brain showed reversal of changes. Neurotoxicity caused by metronidazole is an uncommon adverse effect of a commonly used antimicrobial drug and should be considered in the appropriate clinical scenario.

Isolated dysarthria due to hypoglossal palsy caused by supratentorial ischemic stroke: Radiological and electropsyological analysis.

OBJECTIVES: Isolated dysarthria caused by stroke is a rare condition and generally seen in infratentorial lesions, especially in cerebellar lesions. Isolated dysarthria associated with supratentorial ischemic lesions are also very rare and, these conditions were shown in only a few cases. In this study, six patients characterized by IHP due to supratentorial ischemic lesions are presented. PATIENTS AND METHODS: Physical examinations of patients were done by two different neurologists. The risk factors for stroke were determined. Localisations of lesions were identified by using magnetic resonance images(MRI). Corticolingual tract affection without sensorial and other corticospinal tract involvement was confirmed using Somatosensory evoked potentials(SEPs) and Transcranial Magnetic Stimulation(TMS). RESULTS: Unilateral lingual paresis was detected in all of the patients. The lesions were demonstrated on MRI slices. All lesions were on the same localization. The affection of the corticolingual tract without any other motor and sensory tract involvement was proven electrophysiologically. CONCLUSION: Corticospinal and corticobulbar fibers are very close to each other. Therefore common involvement is expected in cerebrovascular diseases. However, six patients with IHP caused by supratentorial ischemic lesions were reported in this study. Thus, a possible map of the corticolingual tract was drawn.

Articulatory network reorganization in Parkinson's disease as assessed by multimodal MRI and acoustic measures.

INTRODUCTION: Hypokinetic dysarthria (HD) is common in Parkinson's disease (PD). Our objective was to evaluate articulatory networks and their reorganization due to PD pathology in individuals without overt speech impairment using a multimodal MRI protocol and acoustic analysis of speech. METHODS: A total of 34 PD patients with no subjective HD complaints and 25 age-matched healthy controls (HC) underwent speech task recordings, structural MRI, and reading task-induced and resting-state fMRI. Grey matter probability maps, task-induced activations, and resting-state functional connectivity within the regions engaged in speech production (ROIs) were assessed and compared between groups. Correlation with acoustic parameters was also performed. RESULTS: PD patients as compared Tto HC displayed temporal decreases in speech loudness which were related to BOLD signal increases in the right-sided regions of the dorsal language pathway/articulatory network. Among those regions, activation of the right anterior cingulate was increased in PD as compared to HC. We also found bilateral posterior superior temporal gyrus (STG) GM loss in PD as compared to HC that was strongly associated with diadochokinetic (DDK) irregularity in the PD group. Task-induced activations of the left STG were increased in PD as compared to HC and were related to the DDK rate control. CONCLUSIONS: The results provide insight into the neural correlates of speech production control and distinct articulatory network reorganization in PD apparent already in patients without subjective speech impairment.

Dysarthria syndromes in children with cerebral palsy.

AIM: To investigate whether dysarthria syndromes acquired in adulthood can also be observed in children with cerebral palsy (CP) and, if so, whether they align with children's CP subtypes. METHOD: Twenty-six children with CP participated (mean age 7y 8mo [SD 1y 2mo], 5y 1mo-9y 10mo; 16 males and 10 females). Speech samples were elicited in a computer-based game and were analysed using the auditory perceptual criteria of the Bogenhausen Dysarthria Scales (BoDyS). For statistical classification, three comparison groups of adults with standard dysarthria syndromes (i.e. spastic, hyperkinetic, and ataxic) were used. Their BoDyS data were entered into a mixture discriminant analysis, with data from the comparison groups as the training sample and those from the children with CP as the test sample. Results were related to findings in a group of adults with CP. RESULTS: Among the children with CP, most had spastic (n=14), while fewer had ataxic (n=9) or hyperkinetic (n=3), dysarthria. However, syndrome allocations were significantly more ambiguous than in adults with CP. For 11 children, their dysarthria syndromes did not align with their CP subtype. INTERPRETATION: Dysarthria syndromes are less clear cut in children than in adults with CP because of a number of developmental factors. WHAT THIS PAPER ADDS: Children with cerebral palsy (CP) show diverse patterns of dysarthric symptoms. Dysarthria syndromes do not seem to manifest fully during childhood. Dysarthria syndrome and CP subtype may not align in children with CP.

Network Fingerprint of Stimulation-Induced Speech Impairment in Essential Tremor.

OBJECTIVE: This study was undertaken to gain insights into structural networks associated with stimulation-induced dysarthria (SID) and to predict stimulation-induced worsening of intelligibility in essential tremor patients with bilateral thalamic deep brain stimulation (DBS). METHODS: Monopolar reviews were conducted in 14 essential tremor patients. Testing included determination of SID thresholds, intelligibility ratings, and a fast syllable repetition task. Volumes of tissue activated (VTAs) were calculated to identify discriminative fibers for stimulation-induced worsening of intelligibility in a structural connectome. The resulting fiber-based atlas structure was then validated in a leave-one-out design. RESULTS: Fibers determined as discriminative for stimulation-induced worsening of intelligibility were mainly connected to the ipsilateral precentral gyrus as well as to both cerebellar hemispheres and the ipsilateral brain stem. In the thalamic area, they ran laterally to the thalamus and posteromedially to the subthalamic nucleus, in close proximity, mainly anterolaterally, to fibers beneficial for tremor control as published by Al-Fatly et al in 2019. The overlap of the respective clinical stimulation setting's VTAs with these fibers explained 62.4% (p < 0.001) of the variance of stimulation-induced change in intelligibility in a leave-one-out analysis. INTERPRETATION: This study demonstrates that SID in essential tremor patients is associated with both motor cortex and cerebellar connectivity. Furthermore, the identified fiber-based atlas structure might contribute to future postoperative programming strategies to achieve optimal tremor control without speech impairment in essential tremor patients with thalamic DBS. ANN NEUROL 2021;89:315-326.

Diagnosis of Transient Ischemic Attack: Sex-Specific Differences From a Retrospective Cohort Study.

BACKGROUND AND PURPOSE: Research suggests that women and men may present with different transient ischemic attack (TIA) and stroke symptoms. We aimed to explore symptoms and features associated with a definite TIA/stroke diagnosis and whether those associations differed by sex. METHODS: We completed a retrospective cohort study of patients referred to The Ottawa Hospital Stroke Prevention Clinic in 2015. Exploratory multinomial logistic regression was used to evaluate candidate variables associated with diagnosis and patient sex. Backwards elimination of the interaction terms with a significance level for staying in the model of 0.25 was used to arrive at a more parsimonious model. RESULTS: Based on 1770 complete patient records, sex-specific differences were noted in TIA/stroke diagnosis based on features such as duration of event, suddenness of symptom onset, unilateral sensory loss, and pain. CONCLUSIONS: This preliminary work identified sex-specific differences in the final diagnosis of TIA/stroke based on common presenting symptoms/features. More research is needed to understand if there are biases or sex-based differences in TIA/stroke manifestations and diagnosis.

Swallowing, Chewing and Speaking: Frequently Impaired in Oculopharyngeal Muscular Dystrophy.

BACKGROUND: Oculopharyngeal muscular dystrophy (OPMD) is a late onset progressive neuromuscular disorder. Although dysphagia is a pivotal sign in OPMD it is still not completely understood. OBJECTIVE: The aim of this study was to systematically investigate oropharyngeal functioning in a large OPMD population. METHODS: Forty-eight genetically confirmed OPMD patients completed questionnaires, performed clinical tests on swallowing, chewing, speaking, tongue strength and bite force, and underwent videofluoroscopy of swallowing. Descriptive statistics was used for all outcomes and logistic regression to investigate predictors of abnormal swallowing. RESULTS: Eighty-two percent reported difficulties with swallowing, 27% with chewing and 67% with speaking. Patients performed significantly worse on all oropharyngeal tests compared to age-matched controls except for bite force. Also asymptomatic carriers performed worse than controls: on chewing time, swallowing speed and articulation rate. During videofluoroscopy, all patients (except one asymptomatic) had abnormal residue and 19% aspirated. Independent predictors of abnormal residue were reduced swallowing capacity for thin liquids (OR 10 mL = 0.93; 20 mL = 0.95) and reduced tongue strength for thick liquids (OR 10 mL = 0.95); 20 mL = 0.90). Aspiration of thin liquids was predicted by disease duration (OR = 1.11) and post-swallow residue with 20 mL (OR = 4.03). CONCLUSION: Next to pharyngeal dysphagia, chewing and speaking are also frequently affected in OPMD patients, even in asymptomatic carriers. Residue after swallowing is a very early sign, while aspiration is a later sign in OPMD. For clinical follow-up monitoring of subjective complaints, swallowing capacity and tongue strength seems relevant.

Mapping typical and hypokinetic dysarthric speech production network using a connected speech paradigm in functional MRI.

We developed a task paradigm whereby subjects spoke aloud while minimizing head motion during functional MRI (fMRI) in order to better understand the neural circuitry involved in motor speech disorders due to dysfunction of the central nervous system. To validate our overt continuous speech paradigm, we mapped the speech production network (SPN) in typical speakers (n = 19, 10 females) and speakers with hypokinetic dysarthria as a manifestation of Parkinson disease (HKD; n = 21, 8 females) in fMRI. We then compared it with the SPN derived during overt speech production by 15O-water PET in the same group of typical speakers and another HKD cohort (n = 10, 2 females). The fMRI overt connected speech paradigm did not result in excessive motion artifacts and successfully identified the same brain areas demonstrated in the PET studies in the two cohorts. The SPN derived in fMRI demonstrated significant spatial overlap with the corresponding PET derived maps (typical speakers: r = 0.52; speakers with HKD: r = 0.43) and identified the components of the neural circuit of speech production belonging to the feedforward and feedback subsystems. The fMRI study in speakers with HKD identified significantly decreased activity in critical feedforward (bilateral dorsal premotor and motor cortices) and feedback (auditory and somatosensory areas) subsystems replicating previous PET study findings in this cohort. These results demonstrate that the overt connected speech paradigm is feasible during fMRI and can accurately localize the neural substrates of typical and disordered speech production. Our fMRI paradigm should prove useful for study of motor speech and voice disorders, including stuttering, apraxia of speech, dysarthria, and spasmodic dysphonia.

Korean language specific dysarthria associated with idiopathic peripheral facial palsy.

To investigate the patterns of dysarthria in Korean patients with idiopathic peripheral facial palsy.Seventy-eight patients diagnosed with idiopathic peripheral facial palsy within the onset of symptom to 7 day time frame were prospectively enrolled. The initial symptom of facial palsy was examined by the House-Brackmann scale. All patients were tested by Urimal-Test of Articulation and Phonology-2 (U-TAP-2), which is specialized for the evaluation of dysarthria in Korean language - Hangeul - when the patients first visited and were followed up at 4 weeks after the onset, respectively. The facial electromyography was performed after 7 days, since the presentation of the first symptom. Electric stimulation therapy and simple facial exercise education were performed in all patients as routine treatments for facial palsy with or without dysarthria. The patterns of dysarthria were analyzed by initial and follow-up U-TAP-2 results, respectively.Among 78 patients, 50 patients (64.1%) had dysarthria in the first assessment. The 6 consonants and 3 vowels were errored in U-TAP-2 test. The bilabial consonants "ㅃ"[p] or "ㅍ" [p] were substituted with labiodental consonant [f], and palate-alveolar consonants were replaced by alveolar consonants - "ㅊ"[t(Equation is included in full-text article.)] to "ㅌ"[t]. Bilabial consonant "ㅁ"[m] was replaced by velar nasal consonant "ㅇ"[ŋ]. Liquid consonant was altered to nasal sound. For example, "ㄹ"[r] is replace by "ㄴ"[n]. The velar consonant "ㄲ"[k] was pronounced as "ㅋ" [k]. The diphthong vowels "ㅟ"[[Latin Small Letter Turned H]i], "ㅚ"[ø], or "ㅘ"[wa] were pronounced as monothong "ㅣ" [i], "ㅐ"[ε], or "ㅏ"[a], and "못"[mot] is slowly pronounced. After 4 weeks, 14 patients still showed pronunciation errors in 5 consonants and 3 vowels. The most common error was substitution.Among 78 patients with idiopathic peripheral facial palsy, 50 patients had dysarthria and 14 out of 50 patients with dysarthria lasted more than 4 weeks. Five consonants ("ㅁ", "ㅊ", "ㅍ", "ㄹ", "ㄲ") and 3 vowels ("ㅘ", "ㅗ", "ㅟ or ㅚ") were still mispronounced after 4 weeks, and most common error was substitution. Therefore, speech evaluation and speech therapy specialized for errors in high frequency of consonants and vowels are needed in patients with idiopathic peripheral facial palsy, in Korea.

A telephonic single breath count test for screening of exacerbations of myasthenia gravis: A pilot study.

BACKGROUND: Patients with myasthenia gravis (MG) may experience worsening symptoms outside of a clinical setting. A method of diagnosing and triaging such individuals would be valuable. This study gauged the viability of a nurse-administered single breath count test (SBCT) over the telephone for assessing MG exacerbations. METHODS: This was a retrospective, single-center review of a pilot study of 45 telephone calls from patients with MG who had worsening baseline symptoms. SBCTs were administered over the telephone to patients by trained nurses. Patients with a breath count of 25 or less were sent to the emergency department. RESULTS: Using a cutoff count of 25, the nurse-administered telephonic SBCT had a positive predictive value of 71%, sensitivity of 80%, and specificity of 60% in diagnosing an MG exacerbation. CONCLUSIONS: SBCT administered by trained nurses by means of telephone may be a useful screening tool for assessing decreased respiratory function in patients with MG.

Respiratory muscle training in stroke patients with respiratory muscle weakness, dysphagia, and dysarthria - a prospective randomized trial.

OBJECTIVE: To examine the efficacy of combined inspiratory and expiratory respiratory muscle training (RMT) with respect to the swallowing function, pulmonary function, functional performance, and dysarthria in patients with stroke. DESIGN: Prospective, randomized controlled trial. SETTING: Tertiary hospital. PARTICIPANTS: The trial included 21 subjects (12 men, 9 women) aged 35 to 80 years presenting with 6 months history of unilateral stroke, respiratory muscle weakness (≥70% predicted maximal inspiratory pressure (MIP) and/or ≤70% maximal expiratory pressure (MEP)), dysphagia, or dysarthria. These subjects were randomly assigned to the control (n = 10, rehabilitation) and experimental (n = 11, rehabilitation with RMT) groups. INTERVENTION: Inspiratory RMT starting from 30% to 60% of MIP and expiratory RMT starting from 15% to 75% of MEP for 5 days/week for 6 weeks. MAIN OUTCOME MEASURES: MIP, MEP, pulmonary function, peak cough flow, perception of dyspnea, Fatigue Assessment Scale, Modified Rankin Scale, Brunnstrom stage, Barthel index, Functional Oral Intake Scale (FOIS), and parameters of voice analysis. RESULTS: Significant differences were observed between both groups in terms of MIP, forced vital capacity (FVC), and forced expiratory volume per second (FEV1) of the percentage predicted. Significant difference was found with respect to the change in fatigue, shimmer percent, amplitude perturbation quotient, and voice turbulence index (VTI) according to the acoustic analysis in the RMT group. The FEV1/FVC ratio was negatively correlated with jitter percent, relative average perturbation, pitch perturbation quotient, and VTI; the maximum mid-expiratory flow (MMEF) and MMEF% were also negatively correlated with VTI. Significant differences among participants of the same group were observed while comparing the Brunnstrom stage before and after training of the affected limbs and the Barthel scale and FOIS scores in both the groups. CONCLUSIONS: Altogether, 6-week combined inspiratory and expiratory RMT is feasible as adjuvant therapy for stroke patients to improve fatigue level, respiratory muscle strength, lung volume, respiratory flow, and dysarthria.Clinical trial registration number (Clinical Trial Identifier): NCT03491111.

Right fronto-parietal white matter disruption contributes to speech impairments in amyotrophic lateral sclerosis.

INTRODUCTION: Non-linguistic properties of speech are widely heterogeneous and require complex neurological integration. The association between white matter integrity and the severity of dysarthria was investigated in a group of patients diagnosed with amyotrophic lateral sclerosis (ALS). METHODS: Thirty-six patients diagnosed with amyotrophic lateral sclerosis completed a magnetic resonance imaging protocol inclusive of diffusion-weighted images. A clinical assessment of pneumo-phono-articulatory abilities was conducted for each patient, and a composite score of residual speech capacity was calculated. Tract-Based Spatial Statistics was carried out to model the potential association between residual speech capacity and microstructural properties of white matter (fractional anisotropy, mean and radial diffusivity). RESULTS: A significant negative association was found between residual speech capacity and mean diffusivity in a large white matter cluster located in frontal, parietal and right temporal regions. These subcortical areas were characterised by pathological microstructural disruption, as revealed by post hoc analyses. CONCLUSIONS: Non-linguistic aspects of speech are associated with microstructural integrity of frontal, parietal and right temporal white matter in amyotrophic lateral sclerosis. Such mapping is consistent with the centres responsible of volitional control of speech and sensory feedback during non-linguistic speech production.

Direction-Specific Jaw Dysfunction and Its Impact on Tongue Movement in Individuals With Dysarthria Secondary to Amyotrophic Lateral Sclerosis.

Purpose The current study tested jaw movement characteristics and their impact on tongue movement for speech production in individuals with amyotrophic lateral sclerosis (ALS). Specifically, the study examined tongue and jaw movement in multiple directions during jaw opening and closing strokes in individuals with ALS and controls. Method Twenty-two individuals with ALS and 22 controls participated in the current study. Tongue and jaw movements during the production of the words "Iowa" and "Ohio" (produced in a carrier phrase) were recorded using electromagnetic articulography. Tongue and jaw distances were measured for jaw opening and closing strokes. Distance was measured in the anterior-posterior and superior-inferior dimensions (retraction, advancement, lowering, and raising). Results Findings revealed that individuals with ALS exaggerated their jaw opening movements, but not their jaw closing movements, compared to controls. Between the groups, a comparable tongue lowering distance was observed during jaw opening movements. In contrast, reduced tongue raising was observed during the jaw closing movements in individuals with ALS compared to controls. Conclusion The findings suggest that individuals with ALS produce excessive jaw opening movements in the absence of excessive jaw closing movements. The lack of excessive jaw closing movements results in reduced tongue raising in these individuals. Excessive jaw opening movements alone suggest a direction-specific jaw dysfunction. Future studies should examine whether excessive jaw raising can be facilitated and if it enhances tongue raising movement for speech production in individuals with dysarthria secondary to ALS.