Bulbar Function in Neuromuscular Patients (ORDEG)

Prospective Evaluation of Bulbar Function in Neuromuscular Patients

The main objective is prospectively study the upper airways function in an adult population of neuromuscular patients. The main measurement will be the evolution of tongue strength recorded each year during annual clinical evaluation on a 5 years period.

Study Overview

• Status: Unknown
• Conditions: Neuromuscular Diseases
• Intervention / Treatment: Procedure: tongue strength measurement; Procedure: respiratory function measurement; Procedure: swallowing tests; Other: Magnetic resonance imaging

Detailed Description

The main objective is to study the function of the superior airways (tongue and pharynx) in an non invasive way in neuromuscular patients followed in the home ventilation unit of the Raymond POINCARE hospital (myopathy, neuropathy, disease of the neuromuscular junction or anterior horn) or of Necker Hospital and to detect prematurely the bulbar dysfunction.

The secondary objectives of this prospective monocentric observational physiopathological study are:

1. to study the correlation between the possible swallowing dysfunction and the anomalies of the respiratory parameters
2. to establish correlations between infraclinical or clinical and presence of morphological tongue anomalies detected by magnetic resonance imaging like in acid maltase deficiency.

The maximal tongue strength protrusion will be measured according to a method already used in the laboratory to explore sleep apnea syndromes.

Swallowing will be estimated by a non invasive method already used to highlight swallowing disorders in neurological and muscular pathologies This method will be associated with a method of recording of thoracic and abdominal movements.

Data wil be compared to a control database obtained for a previous study

• Study Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

• Study Contact: Name: David Orlikowski, MD PhD; Phone Number: 33147107777; Email: david.orlikowski@rpc.aphp.fr
• Study Contact Backup: Name: Frederic Barbot, MD; Phone Number: 33147104474; Email: frederic.barbot@rpc.aphp.fr

Study Locations

• France
  • Garches, France, 92380
    • Recruiting
    • Home Ventilation Unit, Raymond Poincare Hospital
    • Sub-Investigator: Hélène Prigent, MD, PhD
    • Sub-Investigator: Frederic Lofaso, MD, PhD
    • Contact: David Orlikowski, MD, PhD; Phone Number: 33 1 47 10 77 77; Email: david.orlikowski@rpc.aphp.fr
    • Contact: Marjorie Figere, CRA; Phone Number: 33147104615; Email: marjorie.figere@rpc.aphp.fr
    • Principal Investigator: David Orlikowski, MD,PhD
  • Paris, France, 75015
    • Not yet recruiting
    • Non invasive ventilation Unit - Necker Hospital
    • Contact: David Orlikowski, PHD; Phone Number: 0033147107777; Email: david.orlikowski@rpc.aphp.fr
    • Sub-Investigator: Brigitte Fauroux, PHD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 70 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

patients affected by neuromuscular pathology

Description

Inclusion Criteria:

• neuromuscular disorder
• age greater than or equal to 6
• Hospitalization in Home Ventilation Unit - Written informed consent

Exclusion Criteria:

• patient's refusal to participate
• major swallowing disfunction
• Acute respiratory failure
• no affiliation to social security

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
neuromuscular patient; Patients affected by neuromuscular pathology will be explored by tongue strength measurement, respiratory function measurement, swallowing tests and Magnetic resonance imaging	Procedure: tongue strength measurement; tongue strength measurement; Procedure: respiratory function measurement; respiratory function measurement; Procedure: swallowing tests; swallowing tests; Other: Magnetic resonance imaging; Magnetic resonance imaging

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change of tongue strength measurement	Measurement using mechanical force transducer	5 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
swallowing tests	Non invasive measurement of swallowing function using surface electromyography, accelerometry and inductance plethysmography	5 years
Respiratory parameters	Vital capacity and respiratory muscle strength measurement using spirometry and manometry	5 years
Vital capacity	Spirometry	5 years
Respiratory muscle strength	manometry (maximal inspiratory pressure and expiratory pressure)	5 years

Collaborators and Investigators

• Sponsor: Centre d'Investigation Clinique et Technologique 805
• Investigators: Principal Investigator: Frederic Lofaso, MD, PhD, Centre d'Investigation Clinique et Technologique 805; Principal Investigator: David Orlikowski, MD, PhD, Centre d'Investigation Clinique et Technologique 805; Principal Investigator: Helene Prigent, MD,PhD, Centre d'Investigation Clinique et Technologique 805

Study record dates

Study Major Dates

• Study Start: April 1, 2014
• Primary Completion (Anticipated): January 1, 2019
• Study Completion (Anticipated): December 1, 2020

Study Registration Dates

• First Submitted: December 20, 2013
• First Submitted That Met QC Criteria: March 24, 2014
• First Posted (Estimate): March 27, 2014

Study Record Updates

• Last Update Posted (Actual): January 25, 2018
• Last Update Submitted That Met QC Criteria: January 24, 2018
• Last Verified: January 1, 2018

More Information

Terms related to this study

• Keywords: neuromuscular; swallowing; Tongue strength
• Additional Relevant MeSH Terms: Nervous System Diseases; Neuromuscular Diseases
• Other Study ID Numbers: 2013-A00601-44