Musculoskeletal Nociceptive Pain in Participants With Neuromuscular Disorders

Summary

The primary aim is to characterize the prevalence, severity and quality of musculoskeletal nociceptive pain in adult patients with neuromuscular disorders (NMD). The secondary objectives are to evaluate whether severity and distribution of muscle pain is associated with muscle function, and to assess whether muscle pain is associated with alterations of muscle elasticity and muscle stiffness. Results of patients with neuromuscular disorders will be compared to age- and gender-matched healthy volunteers. Approx. 70 patients with neuromuscular disorders and 20 healthy volunteers will be enrolled, including patients with the following neuromuscular disorders: histologically confirmed inclusion body myositis (IBM), genetically confirmed late-onset Pompe disease (LOPD), genetically confirmed spinal muscular atrophy type 3 (SMA3), genetically confirmed facio-scapulo-humeral muscle dystrophy (FSHD), genetically confirmed myotonic dystrophy type 1 or type 2 (DM1, DM2). The duration of patient recruitment will be around 12 months.

Conditions

• Pompe Disease (Late-onset)
• Myotonic Dystrophy Type 1 (DM1)
• Myotonic Dystrophy Type 2
• Spinal Muscular Atrophy Type 3
• Inclusion Body Myositis, Sporadic
• Facioscapulohumeral Muscular Dystrophy 1
• Healthy

Interventions

DIAGNOSTIC_TEST

Beck depression inventory fast screen

Beck depression inventory fast screen questionnaire to detect severe depression for eligibility.

DIAGNOSTIC_TEST

German Pain Inventory

German Pain Inventory questionnaire for evaluation of pain. Module A, abbreviated questions of module S (sociodemographic questions S1, S2, S3, S4, S5 and S8) and module L (quality of life) and V (therapies) will be used.

DIAGNOSTIC_TEST

Brief Pain Inventory

Validated questionnaire for pain.

DIAGNOSTIC_TEST

Fatigue Severity and Disability Scale (FSS)

Validated questionnaire for perceived fatigue

DIAGNOSTIC_TEST

Quick Motor Function Test

An evaluator observes the performance of a patient and scores the items separately on a 5-point ordinal scale (ranging from 0 to 4). A total score is obtained by adding the scores of all items and ranges between 0 and 64 points.

DIAGNOSTIC_TEST

Handheld Dynamometry (HHD)

To ensure a high level of objective measurement, muscle strength will also be assessed by handheld dynamometry. The following muscle groups will be tested: Arm abduction, elbow flexion, elbow extension, hip flexion, hip extension, knee extension, knee flexion, foot extension, foot flexion.

DIAGNOSTIC_TEST

Six-minute walk test (6MWT)

It is a sub-maximal exercise test used to assess aerobic capacity and endurance. The distance covered over a time of 6 minutes is used as the outcome by which to compare changes in performance capacity.

DIAGNOSTIC_TEST

Pressure pain threshold

For diagnosis of myofascial pain, pressure algometers are designed and conventionally used to measure deep pressure pain thresholds or tenderness resistance (Park, Kim et al. 2011), and the reliability of pressure pain thresholds according to raters or measurement frequencies has been proven to be relatively high (Chung, Um et al. 1992). The threshold is then determined as the arithmetic mean of the 3 series (in kPa). The measurement will be stopped immediately as the patient feels sensations of "burning", "stinging", "drilling" or "aching. Pressure algometry measurements will be performed on the trapezius, deltoid and supraspinatus muscles, the rectus femoris muscles, and the tibialis anterior muscles.

DIAGNOSTIC_TEST

Myotonometer Assessment

Muscle stiffness, muscle tone, relaxation periods and viscoelasticity will be assessed by a myotonometer of selected muscles (non-invasive measurement). The method of measurement consists of recording damped natural oscillation of soft biological tissue in the form of an acceleration signal and the subsequent simultaneous computation of the parameters of State of Tension, Biomechanical and Viscoelastic properties. Measurements will be performed on the trapezius, deltoid and supraspinatus muscles, the rectus femoris muscles, and the tibialis anterior muscles.

DIAGNOSTIC_TEST

Vital signs

Vital signs (blood pressure, heart rate, respiratory rate) will be measured before and after the six-Minute-Walk-Test (6MWT).

DIAGNOSTIC_TEST

Borg Scale

the Borg scale will be assessed, which is a self-reported questionnaire designed to subjectively assess dyspnea and exertion during activity (Borg 1982). The Borg scale rates dyspnea on a scale of 0 to 10 incorporating nonlinear spacing of verbal descriptors of the level of intensity of dyspnea. A higher Borg score indicates more severe dyspnea. The Borg scale will be administered before starting the 6MWT (≤ 5 minutes) and after completing the 6MWT (≤ 5 minutes).

Sponsors & Collaborators

• LMU Klinikum

  lead OTHER

Principal Investigators

• Stephan Wenninger, PD Dr. med. · Neurologist

Eligibility

Min Age

18 Years

Sex

ALL

Healthy Volunteers

Yes

Timeline & Regulatory

Start

2021-04-15

Primary Completion

2022-08-15

Completion

2022-08-15

Countries

• Germany

Study Locations