Huntington Disease
Conditions
Keywords
Huntington Disease, Respiratory dysfunction, Peak cough flow, Handgrip strength, Screening tool
Brief summary
Hereditary neurodegenerative diagnosis of Huntington's disease (HD) is associated with a progressive deterioration of the respiratory system function . This fact contributes strongly to the increased risk of aspiration pneumonia as a primary cause of death in people with HD. But regularly objective monitoring of the airway system condition is in common clinical practice almost impossible for high time requirements and the need for specialized expensive devices . This drives the need for a simpler and more cost-effective screening tool. In recent years published studies, working with all ages and genders, show correlation between hand grip strength (HGS) and respiratory parameters. As a second simple screening tool, we chose a short questionnaire called the Index of pulmonary dysfunction.
Detailed description
Goal: To identify a simple method for screening respiratory muscle and cough weakness in Huntington's Disease (HD) patients, suitable for clinical practice. Hypothesis 1: Maximal inspiratory pressure, maximal expiratory pressure, and voluntary peak cough flow will be significantly correlated with maximal hand grip strength in HD patients. Hypothesis 2: Maximal inspiratory pressure, maximal expiratory pressure, and voluntary peak cough flow will be significantly correlated with the Index of pulmonary dysfunction in HD patients.
Interventions
OTHERAssessment of respiratory paramenters (Maximal inspiratory pressure, maximal expiratory pressure, and voluntary peak cough flow))
The voluntary peak cough flow is measured using a pneumotachograph (BTL cardiopoint Spiro, BTL industries), which meets the recommendations of the American Thoracic Society and the European Respiratory Society for range and accuracy in forced expiratory maneuvers. Maximal expiratory and inspiratory pressures assessments were performed using a flanged rubber mouthpiece connected to a pressure manometer (Micro RPM, Vyaire Medical). Assessments are conducted according to the statements on respiratory muscle testing of the American Thoracic Society and the European Respiratory Society. Hand grip strength Grip strength is measured using DHD-1 digital hand dynamometer (SAEHAN®,Seahan Corporation), assessment follows recommendation of The American Society of Hand Therapists.
Sponsors
General University Hospital, Prague
Study design
Observational model
CASE_ONLY
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* Genetic confirmed diagnosis of Huntington's disease
Exclusion criteria
* diagnosis of other concomitant neurological diseases; * a history of cardiovascular or lung disease; * respiratory symptoms such as cough, phlegm, wheezing, or dyspnea at the time of assessment
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Maximal Hand grip strength | Up to 35 weeks (once on a single screening visit) | Unit of Measure: kg Assessment followed recommendation of The American Society of Hand Therapists. HSG is measured using DHD-1 digital hand dynamometer (SAEHAN®,Seahan Corporation). |
| Voluntary peak cough flow | Up to 35 weeks (once on a single screening visit) | Unit of measure: l/min Assessments will be performed in accordance with American Thoracic Society/European Thoracic Society guidelines. |
| Maximal expiratory pressure, Maximal inspiratory pressure | Up to 35 weeks (once on a single screening visit) | Unite of measure: cmH2O Assessments will be performed in accordance with American Thoracic Society/European Thoracic Society guidelines. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Index of Pulmonary dysfunction | Up to 35 weeks (once on a single screening visit) | Questionnaire of four questions. Scale range: 0-11 points A higher score indicates a worse pulmonary condition. |
Countries
Czechia
Outcome results
None listed