A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

Speech.A function controlled by bulbar motor neurons is arguably one of the most complex motor acts performed by humans. Speech is a product of coordinated movements of the respiratory atory, atory, and articulatory motor subsystems. The muscles of respiration provide power for speech generation.

The laryngeal structures are the source of phonation or voice. The atory source is shaped into various speech sounds by the actions of the articulatory subsystem composed of the tongue, jaw, and lower and upper lip. The atory subsystem comprised of the muscles of the velum and pharynx is used to prevent air from escaping through the nose and to distinguish oral from nasal speech sounds.

A LS is a progressive neurologic disorder affecting the motor neurons within the brain and spinal cord. Once the brain stem motor neurons are involved, the devastating consequences of this disease in Sue Currently in clinical neurology, we have no objective, reliable measure of bulbar motor neuron deterioration. It leads to speech and swallowing difficulties.

It's therefore essential for us to have an assessment that we can follow not only for diagnostic purposes, but to follow the patients throughout our clinic. In this video, we'll show you a series of procedures that we use in our laboratories to assess bulbar function in patients with a LS.We're currently using this protocol to investigate the relationship between bulbar system deterioration and loss of oral communication, which is an important clinical goal. The outcome of this research will provide essential knowledge needed to advance important research and clinical goals, including improving the diagnosis and management of A LS and determining the effectiveness of new experimental drugs.

Demonstrating these procedures will be June Wong, a graduate student, and Lori Horst, a research coordinator at the Speech Production Laboratory at the University of Nebraska Lincoln. To evaluate the respiratory subsystem, record oral pressure, airflow, and speech acoustics using the Atory aerodynamics system First record vital capacity, the maximum volume of air that is exhaled following maximum inhalation, select the PAS vital capacity protocol for the recording. Next, connect a disposable face mask to the Pneumo Tacho.

Now instruct the participant to inhale as maximally as possible and exhale maximally into the mask with the PAS software, derive the maximum expiratory volume. Next, collect sub glottal pressure the air pressure available in the lungs for production of pressure consonants, select the PAS voicing efficiency protocol. Pass the pressure sensing tube through the face mask.

Occlude the nasal passages with a nose clip. To eliminate potential nasal airflow escape, hold the mask against the participant's face. Adjust the tube so that it is located at the midline of the tongue, approximately two centimeters into the mouth.

Instruct the participant to inhale approximately twice their normal amount and say paw seven times on one exhalation while maintaining consistent pitch and loudness paw paw paw. The rate is maintained at 1.5 syllables per second. Measure peak oral pressure for five repetitions of paw.

Finally, record speech breathing During connected speech, select the PAS running speech protocol. Collect the airflow signal using a disposable mask fit around the face, emphasizing use of a normal, comfortable speaking rate and loudness. Instruct the participant to read a standard 60 word paragraph, developed specifically for accurate automatic pause boundary detection.

Export the airflow traces into a custom made speech pause analysis software program in matlab. In this program, identify examples of the pauses and onsets and offsets of speech set thresholds. For these events manually, the SPA software will extract percent pause time automatically among other measures.

In order to evaluate the laryngeal subsystem via voice recordings, use high quality acoustic recording equipment. Position the microphone approximately 15 centimeters away from the mouth. Now position a microphone on the PAS unit, the same distance away from the mouth.

To collect SPL data, place a nasal clip to eliminate the potential effect of the velo pharyngeal inadequacy on the quality of ation. For maximum phonation, instruct the participant to inhale the maximum amount of air possible and then fate. Awe at a normal pitch and loudness for as long as possible.

Practice at least once and emphasize the importance of putting forth maximum effort prior to recording. Using the acoustic waveform, measure the maximum donation duration in seconds. Load the digitized acoustic waveform into the multidimensional voice profile software for analyses and extract measures of mean F zero noise to harmonic ratio and percent jitter and shimmer.

Among other measures, evaluate the atory subsystem using a exometer. Be sure to calibrate the device prior to each recording. Place on the participant's head with the baffle plate, resting above the upper lip and positioned parallel to the ground.

Ask the participant to repeat one nasal and one non nasal sentence three times at a habitual speaking rate and loudness. Five, a poppy, a poppy. Identify a sentence.

Calculate descriptive statistics for each sentence. Using nater software, calibrate a high resolution optical motion capture system to register facial movements in 3D, attach reflective markers to the participant's head and face at specific anatomical landmarks. Position the microphone for speech acoustic recordings about 15 centimeters away from the mouth.

Ask the participant to read sentences and phrases at their habitual speaking rate and loudness. Bye, Bobby. A poppy Check movements of the facial markers for tracking errors and head corrected based on the subtraction of both the translational and rotational components of head movement.

Load the data into a custom made analysis software smash to derive peak movement speed as the primary indicator of our articulatory function for the jaw and lips. To simultaneously acquire movement and acoustic data of tongue tracking, use an electromagnetic tracking device wave. Attach a six D sensor to the bridge of the nose to record head movement Glue one small five D sensor to the tongue at midline, approximately two centimeters posterior to the tongue tip to obtain tongue movements that are independent from the underlying jaw.

Fit the participant with a pre-made five millimeter bite block. Place the bite block between the molars on the right side of the mouth and to prevent swallowing of the bite block, secure the bite block with string. Now ask the participant to read sentences and phrases.

Record the tongue movements relative to head position post-acquisition. Transfer the data into smash to calculate 3D speed, and also to determine an index of disease related change of each articulator with the sentence intelligibility. Test measure, speech intelligibility and speaking rate.

Ask the participant to read the list at habitual speaking rate and loudness. A trained judge who is unfamiliar to the participant transcribes the sentences orthographically. The judge also marks sentence onsets and offsets.

Finally, the sentence intelligibility software generates speech intelligibility and speaking rate results. The instrumental assessment of each of these subsystems results in a comprehensive profile of bulbar speech performance. For an individual, this profile forms the basis for understanding speech impairment relative to the normal performance.

Typically, healthy talkers are 100%intelligible and read at 190 to 220 words per minute. In this case, a 72-year-old female diagnosed with definite A LS is 90%intelligible and has a very slow speaking rate of 94 words per minute. Compared to age and sex matched healthy controls, her respiratory subsystem appears to be functioning relatively normally.

Percent pause time is the only measure that is indicative of an early change in breathing. For speech, the atory subsystem indicates lower than normal vocal pitch, higher cycle to cycle variability as measured by jitter and increased harmonic to noise ratio. Atory performance is characterized by a notable increase in nasal in the oral sentence, presumably due to weakness in the vlo pharyngeal musculature.

The reduction in the contrast between oral and nasal sounds is notably large. The oral articulators show slight reduction in the peak speed of the jaw and lower lip movements, and a very large drop in tongue speed. In an assessment of change over time for the subsystem and clinical measures for two hypothetical individuals with a LS, the change over time is depicted by standardized slopes calculated for each measure across a series of recording times.

Subject one has a profile characteristic of bulbar a LS showing changes across bulbar measures with the articulatory subsystem showing the largest slope of decline over time. Subject two has a profile characteristic of spinal a LS showing relative stability across the subsystem and clinical bulbar measures. This protocol will provide new knowledge about how a LS affects bulbar functions, including speech.

The data will help to develop more cost efficient and clinically feasible approaches to quantify bulbar involvement. This objective bulbar assessment may be used in the future to assess a broad range of speech motor impairments, including those related to stroke, traumatic brain injury, multiple sclerosis, and Parkinson's disease.