Noninvasive Examination of the Work of Breathing in Patients With Amyotrophic Lateral Sclerosis (ALS).

Study Overview
=================
Brief Summary
-----------------
The literature to date indicates that noninvasive positive pressure ventilation (NIPPV) provides effective noninvasive ventilator support, prolongs survival, and improves quality of life (QOL) in Amyotrophic Lateral Sclerosis (ALS) patients. It is generally recommended to patients when their pulmonary function testing demonstrates a drop to 50% forced vital capacity (FVC). One result of using NIPPV may be a reduction in the work of the breathing which would lead to decreased caloric needs. However, the work of breathing and the effects of noninvasive ventilation on caloric use have not been studied in patients with ALS. This is extremely important since there may be a reduction in the caloric needs when ALS patients are placed on NIPPV and if the caloric intake is not adjusted, overfeeding can occur. Overfeeding with too many calories can lead to an increase in carbon dioxide which would actually worsen the respiratory failure. The overall aim of this project is to evaluate how many calories are used by ALS patients while at rest, when placed on NIPPV, and when breathing against a resistance. This will be accomplished using a metabolic cart during these activities. At present, the metabolic cart is routinely used in ALS patients at the time of feeding tube placement to calculate caloric needs. Using the cart to calculate the caloric expenditure on and off the ventilator will aid in calculating the work of breathing and the effects of NIPPV on work of breathing.

Official Title
-----------------
Noninvasive Examination of the Work of Breathing in Patients With Amyotrophic Lateral Sclerosis (ALS).

Conditions
-----------------
Amyotrophic Lateral Sclerosis, Neurodegenerative Disease, Motor Neuron Disease

Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Patients with probable or definite ALS by El Escorial Criteria between ages 20-85 will be included for evaluation. They must have progression of disease over the preceding 6 months and duration of disease less then 5 years. Exclusion Criteria: Patients who do not meet clinical criteria of the diagnosis including those with pure upper motor neuron syndromes (PLS) or pure lower motor syndromes (SMA, Multifocal conduction block, motor neuropathies).

Ages Eligible for Study
-----------------
Minimum Age: 20 Years
Maximum Age: 85 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| ALS<br>Subjects having either definite or probable ALS by El Escorial Criteria. | |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Calories Used with and without NIPPV | Indirect calorimetry will be performed using a metabolic cart to determine caloric use over a 20 minute period without NIPPV. Following this, the test will be repeated over a 20 minute period with NIPPV. | 20 minutes | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Amyotrophic Lateral Sclerosis, Cerebrospinal Fluid, Neurodegenerative Disease, Motor Neuron Disease, Autonomic Nervous System, Neurodegenerative Diseases, Movement Disorders