Acoustic analysis of dysarthria profile in ALS patients.

Dysarthria is a leading disability in ALS patients with motor neurone degeneration in the bulbar region. Although different approaches have been tried in the past, currently, no test is available to detect and follow the progression of dysarthria. We studied 53 patients with definite (n=27) or probable (n=26) ALS (the bulbar onset group n=15, the limb onset group n=38, mean age 53. 66/29-76 years/) according to El Escorial criteria. Each patient was seen by a neurologist every 10-12 weeks and clinical performance was assessed using the Norris scale. To evaluate dysarthria we developed a computer-based acoustic method. All patients had computer-analysed speech sound tests done three times. The most significantly affected vowels were selected for further studies. A method based on the Euclidian principle was used and the results were compared with 30 age, sex-matched, healthy control subjects. Our results demonstrated the existence of a specific dysarthria profile in ALS patients with most significantly affected vowels: 'B', 'O', 'I', 'W', 'T' in the bulbar group, and: 'B', 'I', 'T', 'W', 'O' in the limb group. This study suggests that it is possible to detect and monitor the progression of the disease based on the acoustic analysis of only several sounds. Abnormalities detected in the dysarthria profile may appear prior to any clinical symptoms of the disease.     

Assessment of dysarthria and dysphagia in ALS patients

Swallowing and speech disorders are the dramatic consequences of bulbar and pseudo-bulbar syndrome in ALS. Evaluation is necessary to guide speech therapy and to measure the effects of treatment. This article revues the different examinations used to assess bulbar and pseudobulbar involvement in an ALS patient: oromotor assessment, evaluation of the functions with self assessment, perceptive and objective evaluation of speech disorders, fiberoptic endoscopic evaluation of dysphagia (FEES) and videofluoroscopy.     

Evaluation of dysarthria with the assistance of acoustic speech analysis in patients with amyotrophic lateral sclerosis

The aim of the study was to assess dysarthria in ALS subjects using acoustic speech analysis. The study was performed in 47 definite or probable ALS patients aged 29-76 years (mean age 53.7 yr.) and in 30 age and sex matched healthy control subjects. Neurological examination showed 15 dysarthric ALS subjects. Acoustic speech analysis is a quantitative, computer-acoustic method estimating dysarthria and based on assessing of sound distance from speech sound tests. In both group the mean sound distance between chosen sounds was compared to a basic pattern and was measured on time-frequency computer acoustic analyses (delta f = 125 Hz, delta T = 9 ms, delta s = 0.5 dB). Our results demonstrated that all sounds were incorrect in all ALS subjects. These abnormalities were significantly increased in the dysarthric ALS subjects. The mean sound distances which separated ALS from control subjects is 0.2 (by Euclidian principle) in 4 out of 5 measured sounds. We suggest that it is possible to detect and measure dysarthria in ALS patients based on the acoustic speech analysis, also in the limb onset ALS subjects.     

Accuracy and inter-observer variation in the classification of dysarthria from speech recordings

Background   Dysarthria may be classified as flaccid, spastic, ataxic, hypokinetic, choreatic, dystonic, or mixed. We hypothesized that in routine neurological practice the reliability and accuracy of perceptual analysis alone in the classification of dysarthria is low and that this classification is mainly based on the clinical context rather than on the perception of speech. We therefore studied the accuracy and the inter- observer agreement in the classification of dysarthrias on the basis of perceptual analysis alone. Methods   Seventy two neurologists and neurological trainees classified recorded speech samples of 100 patients as flaccid, spastic, ataxic, extrapyramidal, or mixed dysarthria, or as not dysarthric. All observers were blinded to the patients’ final diagnosis, which was based on all clinical features and investigations. In the analysis the observers were arranged in eight groups of nine observers, or four paired groups with similar levels of clinical experience. Together, the observers in a given group rated all 100 recordings. Results   The accuracy of the classification was poor (35 % were classified correctly) and the inter-observer agreement between paired groups low (κ 0.16 to 0.32). The level of experience in neurology did not have a significant influence. Conclusion   Neurological trainees as well as experienced neurologists have great difficulty in identifying specific types of dysarthria on the basis of perceptual analysis alone. In clinical practice this probably means that most neurologists will classify dysarthria in the context of other features from neurological examination or ancillary investigations.     

Evaluation of motor speech function in the diagnosis of various forms of dysarthria

Perceptual analysis is not sufficient enough to identify specific dysarthria types. In order to improve the discrimination between dysarthria types, we developed a standardized evaluation of different functions controlling speech motor performances. This was applied to 90 patients suffering from hypokinetic, spastic or ataxic dysarthria and 15 control subjects. A discriminate analysis showed that 71.4 p. 100 of the cases were correctly classified. This model was validated within a new group of 21 patients and showed that the less severe dysarthric parkinsonian patients were difficult to distinguish from control subjects. The discriminate analysis was then used for 20 patients with atypical parkinsonism. Seven patients with progressive supranuclear palsy were considered to have hypokinetic dysarthria. The 6 patients with multisystem atrophy and 7 with corticobasal degeneration were classified among the 3 dysarthric types. We suggest that motor speech evaluation may contribute to differential diagnosis within groups of patients suffering from atypical parkinsonism.     

Communication disturbances in the natural speech of schizophrenic patients and non-schizophrenic parents of Patients

Subclinical communication disturbances in the relatives of schizophrenic patients appear to be heterogeneous in both form and origin. The Communication Disturbances Index (CDI) was applied to the natural speech of stable schizophrenic out-patients, non-schizophrenic parents of patients, and control subjects. The parents of schizophrenic patients displayed more frequent overall instances of communication failure in their speech than the controls. The specific types of disturbance that were more frequent were structural lack of clarity, vague and ambiguous word meanings. The parents did not differ significantly from the patients with regard to total CDI ratings. However, patients made more frequent missing-information reference than parents, and parents made more frequent vague reference than patients. High CDI scores in parents were associated with more severe lifetime core positive symptoms in their patient offspring.     

Affective reactivity of speech disturbances in schizotypy

Speech disturbances (SD) are a pernicious symptom of schizophrenia that increase when negative emotion is elicited. This increase is referred to as affective reactivity (AR). Although considerable research has examined SD in schizophrenia, few studies have investigated this symptom in individuals at risk for the disorder, who demonstrate schizophrenia-like, or schizotypic, traits. In the present study, we examined: (1) SD severity in schizotypy, (2) how SD varies as a function of stress reactivity in schizotypy, and (3) the relationship between SD/AR with Quality of Life (QOL). Individuals with psychometrically-defined schizotypy (n = 83) and controls (n = 22) completed a laboratory procedure in which they produced speech while viewing pleasant and stressful photographs. This speech was analyzed for subtle speech disorder using a well-validated measure. We found that the schizotypy group demonstrated significant increases in SD across both baseline and stressful conditions compared to the control group. AR was not significantly different between the groups. Within the schizotypy group, severity of disorganized schizotypy symptoms was associated with high levels of SD and AR while interpersonal schizotypy was associated with low levels of SD and AR. AR was also related to increased objective QOL in the schizotypy group. This study highlights the role of stress reactivity across the schizophrenia-spectrum. Moreover, the incongruous relationships between disorganized and interpersonal symptoms with SD underscore the marked heterogeneity in processes across schizotypy.     

The cognitive origins of specific types of schizophrenic speech disturbances

OBJECTIVE: Cognitive impairments associated with schizophrenia might be expected to have a marked impact on the ability to produce coherent speech, yet associations between cognitive performance and speech disorder have typically been weak. Findings on this question may have been limited by measurement methods and by the heterogeneity of speech disorder. This study examined the contributions of impairments in sustained attention and sequencing abilities to schizophrenic speech disorder, measured in terms of communication failures and divided into different types of disorder. METHOD: Samples of natural speech were collected from severely ill inpatients with schizophrenia and nonpsychiatric comparison subjects and rated for frequencies of six types of communication failures: four structural and two nonstructural types. Subjects also completed a battery of cognitive tests assessing several facets of attention and sequencing ability. Hierarchical regression was used to identify cognitive contributors to communication failures. RESULTS: Impaired sustained attention was associated with more frequent structural and nonstructural communication failures. As predicted, impaired sequencing, and in particular conceptual sequencing, also made a substantial contribution to the structural communication failures, but not to the nonstructural ones. These findings held when global level of impairment was controlled statistically. Performance on the tests of attention and sequencing explained 56% of the variance in structural speech disorder. CONCLUSIONS: Schizophrenic speech disorder is heterogeneous in form and in cognitive underpinnings. Impairments in attention and sequencing abilities are highly predictive of communication failures related to language structure.     

Timing disturbances in the speech of a language-delayed Thai adult

An acoustic-perceptual investigation was performed on various aspects of timing in the speech of a 21-year-old adult speaker of Thai who reportedly did not start speaking until the age of 7. Selected aspects of timing included: ( I ) the voicing contrast in Thai homorganic word-initial stops; (2) the duration contrast in Thai short and long vowels; and (3) the duration patterns of phrases and sentences in Thai connected speech. Measures of stop consonant voicing and vowel length were taken from monosyllabic citation forms; measures of syllables, phrases and sentences from an oral reading of a paragraph-sized passage. Findings indicated that speech timing skills relatcd to stop consonant voicing, vowel length, and rhythm can be differentially impaired, and moreover, that the pattern of impairment appears to be related to the size of the temporal planning unit.     

Sympathetic disturbances increase risk of sudden cardiac arrest in sporadic ALS

BACKGROUND: ALS exclusively involves motor neurons, however, accumulating evidence suggests involvement of sympathetic neurons, as in other diseases including Parkinson's disease and multiple system atrophy. In these diseases increased risk of sudden cardiac arrest is established, while that in ALS remains uncertain. METHODS: The authors retrospectively studied 12 pathologically confirmed sporadic ALS patients who received no assisted ventilation. Among them, two patients died of sudden cardiac arrest. Changes in QTc interval and dispersion, indices of sympathetic activities obtainable by routine electrocardiograms, were evaluated at the early stage and the terminal stage. Pathologically, intermediolateral nucleus (IML) sympathetic neurons in the upper thoracic cord were examined. RESULTS: The QTc intervals and dispersion were significantly increased at the terminal stage compared with that at the early stage (p<0.01). The numbers of IML neurons were significantly lower in ALS patients than in controls (p=0.017), and had linear inverse correlation with the rate of increases in maximum QTc interval and QTc dispersion (p=0.01, r=-0.915 and p=0.02, r=-0.884). Notably, two patients with sudden cardiac arrest showed longer QTc interval, larger QTc dispersion, and lower number of IML neurons than most of others. CONCLUSIONS: Patients with ALS had reduced sympathetic activities at the terminal stage of disease, presumably due to neuronal loss in IML, which may increase risk of sudden cardiac arrest. Thus, prolonged QTc intervals and increased QTc dispersion may suggest an increased risk of sudden death in ALS, as in other neurodegenerative diseases.     

Voice onset time in aphasia,apraxia of speech and dysarthria: a review

Voice onset time (VOT) is an objective temporal acoustic parameter defined as the time between the release of the oral constriction for plosive production and the onset of vocal fold vibrations. Many researchers consider VOT to be the most reliable acoustic cue for the distinction between voiced and voiceless stops. Previous studies have explored the physiological and linguistic factors underlying VOT production in normal speakers across several languages. A major clinical goal of acoustic analysis in speech disorder is to establish a correlation between the acoustic abnormalities and the phonetic perturbations. VOT could thus be used as an acoustic parameter that indicates the phonetic contrast between voiced and voiceless stops. This paper includes a critical review of the measurement of VOT, factors of VOT variability and the effect of neurogenic communication disorders on VOT. We review the VOT data from subjects who exhibit aphasia, apraxia of speech and dysarthria. These studies reveal that VOT perturbations in aphasia have been interpreted as phonemic or phonetic errors, while VOT abnormalities in apraxia of speech and dysarthria grossly reflect loss of motor control.     

Proton magnetic resonance spectroscopy and transcranial magnetic stimulation for the detection of upper motor neuron degeneration in ALS patients

Transcranial magnetic stimulation (TMS) was compared to proton magnetic resonance spectroscopy (¹H-MRS) for the detection of upper motor neuron loss or dysfunction in 49 ALS patients classified according to the El Escorial criteria. Abnormal NAA/Cho ratios were detected in 53% of ALS patients. Abnormal TMS results (i.e. cortical inexcitability or prolonged CMCT's) were obtained in 63% of ALS patients. If one or both methods were considered for diagnosis of upper motor neuron degeneration/dysfunction, the percentage of abnormal findings was 77%, whilst in 39% of all patients both methods produced abnormal results. Compared to TMS, ¹H-MRS detected more patients with upper motor neuron involvement in the suspected El Escorial subgroup (42% versus 25%), whereas TMS detected more patients with upper motor neuron involvement in the possible (81% versus 50%), probable (71% versus 57%) and definite El Escorial subgroup (71% versus 64%). We conclude that the combined use of ¹H-MRS and TMS increases diagnostic accuracy for the detection of upper motor neuron involvement in ALS patients.     

The final month of life in patients with ALS

OBJECTIVE: To study the health care experiences and palliative care needs of patients with ALS in their final month of life. METHODS: Caregivers of decedent patients with ALS completed a single survey focused on the final month of life. They reported the patients' physical and emotional symptoms, preferences for end-of-life care, completion of advance directives, and preparation for death. The caregiver reported which life-sustaining treatments were administered, withheld, or withdrawn; whether the patient was enrolled in hospice; and their own satisfaction with the patient's medical care. RESULTS: Fifty caregivers completed the survey. Caregivers reported that the most common symptoms in the last month of life included difficulty communicating (62%), dyspnea (56%), insomnia (42%), and discomfort other than pain (48%). Pain was both frequent and severe. One-third of caregivers were dissatisfied with some aspect of symptom management. Caregivers reported an advance directive was completed by 88% of patients and the patients' goals of care were honored by 88% of health care practitioners. Two-thirds of patients were enrolled in hospice. Compared to nonhospice patients, hospice patients were significantly more likely to: 1) die in their preferred location; 2) die outside the hospital; and 3) receive morphine. Most caregivers reported that their loved one was at peace, and prepared for and was accepting of death. CONCLUSIONS: Caregivers report that many patients with ALS still experience distressing physical symptoms in the last month of life, despite enrollment in hospice. Most patients with ALS, however, anticipate and plan for their deaths and have their wishes respected.