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| Name | Class |
|---|---|
| Arizona State University | OTHER |
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The purpose of this study is to find out if changes in speech can signal changes in the ability to think or remember. ALS patients with and without cognitive dysfunction will be followed for one year. Every three months, patients will undergo a series of cognitive and basic clinical outcomes tests. In addition, participants will take home a study-provided tablet on which they will complete weekly speech recording activities.
Cognitive dysfunction is increasingly recognized as a core feature of amyotrophic lateral sclerosis (ALS). With appropriate testing, up to 50% of ALS patients will show evidence of frontotemporal dysfunction. Approximately 15% of patients meet formal criteria for frontotemporal dementia (FTD). Certain genetic forms of ALS (e.g., mutations in C9orf72) have even higher incidences of FTD. The presence of cognitive abnormalities is an adverse risk factor for survival, and its presence influences the ability of patients to cooperate in clinical trials. However, screening for frontotemporal abnormalities is frequently not performed in ALS clinics, and tools for diagnosing cognitive dysfunction are either time consuming or insensitive. Additionally, the frequently co-existing dysarthria complicates the assessment and may mask more subtle cognitive deficits. Once identified, ways of following progressive decline are also lacking. In an ongoing study, it has been shown that a sophisticated suite of speech and language analytics, developed by two of the investigators, can identify abnormalities in cognitively normal ALS patients without speech symptoms, and predict important functional changes outside of the speech domain. In this study, investigators will evaluate both speech and language in 50 patients with ALS both with and without symptoms of cognitive decline. This evaluation will be paired with two cognitive screening tools frequently used in ALS clinics, the ALS Cognitive Behavioral Screen (ALS-CBS) and the Montreal Cognitive Assessment (MoCA). The investigators will evaluate the extent to which speech and language deficits precede abnormalities as measured by the above tools and determine whether cognitive change can be accurately followed over 12 months using speech and language measures. It is hypothesized that speech and language measures will accurately and sensitively predict cognitive changes. If so, such measures may be very useful in future studies of potential therapeutic agents for ALS-FTD and other dementias.
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| Measure | Description | Time Frame |
|---|---|---|
| Change in ability on Speech and Language Battery | Speech recordings made at home via a tablet using the " SpeechAssess" app. | Weekly recordings for one year |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Montreal Cognitive Assessment | Cognitive screening tool | Administered every three months for a year |
| Change in ALS Cognitive Behavioral Screen | Cognitive screening tool |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with ALS, diagnosed as definite, probable, or possible ALS according to the modified El Escorial Criteria.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Barrow Neurological Institute | Phoenix | Arizona | 85013 | United States |
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| ID | Term |
|---|---|
| D000690 | Amyotrophic Lateral Sclerosis |
| D060825 | Cognitive Dysfunction |
| D013060 | Speech |
| ID | Term |
|---|---|
| D013118 | Spinal Cord Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D016472 | Motor Neuron Disease |
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Blood serum and blood plasma to be collected at three timepoints and retained in a biorepository.
| Administered every three months for a year |
| Change in Vital Capacity | Measure of breathing function | Administered every three months for a year |
| Change in Negative Inspiratory Force | Measure of nasal inhale capabilities | Administered every three months for a year |
| Change in "ALS Functional Rating Scale- Revised" | Questionnaire regarding daily functioning. Scale is measured from 0 to 48 points, with 48 being normal function and 0 indicating no functional abilities. | Administered every three months for a year |
| D019636 | Neurodegenerative Diseases |
| D057177 | TDP-43 Proteinopathies |
| D009468 | Neuromuscular Diseases |
| D057165 | Proteostasis Deficiencies |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D003072 | Cognition Disorders |
| D019965 | Neurocognitive Disorders |
| D001523 | Mental Disorders |
| D014705 | Verbal Behavior |
| D003142 | Communication |
| D001519 | Behavior |