Phrenic nerve conduction study to diagnose unilateral diaphragmatic paralysis.

BACKGROUND: Unilateral diaphragmatic paralysis (UDP) has major clinical and etiological implications and, therefore, is important to diagnose. Lung function tests and invasive transdiaphragmatic pressure (Pdi) measurements are widely used to this end but, contrary to phrenic nerve conduction study (NCS), they require volitional maneuvers and/or may be poorly tolerated by patients. The purpose of this study was to compare the diagnostic accuracy of Pdi and phrenic NCS for UDP. METHODS: We retrospectively reviewed 28 patients with suspected UDP. The diagnosis established during a multidisciplinary meeting was the reference standard. RESULTS: Phrenic NCS correlated well with Pdi (r = 0.82, P < .005), and the two tests showed good agreement (κ = 0.82, P < .005). Phrenic NCS and Pdi measurements both had 95% sensitivity, 87.5% specificity, 95% positive predictive, and 87.5% negative predictive values. CONCLUSIONS: Both tests were highly sensitive and specific. Phrenic NCS measurement is a simple, reproducible, noninvasive method whose results correlate well with Pdi and provide insight into the UDP mechanism. In the most difficult cases, combining lung function tests, respiratory muscle assessments, and phrenic NCS can help to establish the diagnosis.

Foot Function Index: A Promising Questionnaire for Individuals With Charcot-Marie-Tooth Disease Type 1A.

OBJECTIVE: To evaluate in a preliminary methodologic study, the Foot Function Index (FFI), a 3-subscale (pain, disability, and activity restriction) foot disability assessment questionnaire, in patients with Charcot-Marie-Tooth disease type 1A (CMT1A). DESIGN: Monocentric exploratory cross-sectional study with 2 identical evaluations by the same physical medicine and rehabilitation physician at 14-day intervals (test-retest) according to international guidelines for validating health-related patient-reported outcomes, the Consensus-based Standards for the Selection of Health Measurement Instruments Criteria. SETTING: Physical medicine and rehabilitation and neurology departments in a French academic hospital. PARTICIPANTS: Patients with CMT1A confirmed by molecular biology (N=26). INTERVENTION: The FFI and a health-related quality-of-life questionnaire (Medical Outcomes Study Short Form 36 [SF-36] with mental and physical composite scores) combined with quantitative walk analysis by instrumental gait analysis and evaluation of isokinetic quadriceps and hamstrings peak torque by isokinetic dynamometer. MAIN OUTCOME MEASURES: FFI score and its dimensions. RESULTS: Acceptability was satisfactory, with less than 5% missing data and good distribution of results. Internal consistency was very satisfactory, with Cronbach α of 0.95. Reproducibility was very satisfactory, with Lin concordance coefficient 0.82. External consistency was satisfactory, with expected correlation coefficients: the FFI was significantly correlated with the SF-36 physical composite score and gait parameters (cadence) (r=-0.58 and r=-0.52; P<.005) but not with peak torque or SF-36 mental composite score. CONCLUSIONS: This study confirms the very good metrologic properties of the FFI in patients with CMT1A. The FFI could be a promising questionnaire to assess foot-related disability in a neurologic disease. Complementary studies are still needed to confirm these promising preliminary results.

Multidimensional evaluation is necessary to assess hand function in patients with Charcot-Marie-Tooth disease type 1A.

BACKGROUND: Charcot-Marie-Tooth (CMT) disease type 1A (CMT1A) is the most common hereditary neuropathy. Several studies have assessed the relation between axonal loss and grip strength; however, the functional impact on dexterity and health-related quality of life (HRQoL) is unknown. We hypothesized that the severity of axonal loss will be correlated with loss of function and HRQoL. OBJECTIVE: The purpose of this study was to evaluate the relation between severity of electroneuromyography impairment and its impact on function and HRQoL in adults with CMT1A. METHODS: Grip and lateral pinch strength were evaluated with specific dynamometers: the Jamar and the Pinch Gauge. Dexterity was explored with the Sollerman, Jebsen, and Nine-hole Peg tests. The CMT impact on well-being was assessed by the validated Medical Outcomes Study Short Form 36 (SF-36), Beck Depression Inventory, and Fatigue Severity Scale, and disease severity by the CMT neuropathy score and Inflammatory Neuropathy Cause and Treatment sensory sum score. Finally, axonal loss and demyelination process was assessed by electroneuromyography. RESULTS: We included 33 participants with CMT1A (23 females, mean [SD] age 47.0 [4.7] years). We found lack of correlation between severe electroneuromyography impairment (frequency of abnormal results >80%), significant distal amyotrophy (70%) and quality of life (mean [SD] scores for physical and mental SF-36 36.4 [10.0] and 48.4 [11.5]), autonomy for activities of daily living, and hand function that remains relatively preserved. We found a correlation between lateral pinch and dexterity according to the Sollerman test (r=0.52, p<0.05) but a lack of correlation among the other parameters. CONCLUSIONS: Electrophysiological follow-up seems to be of little relevance to follow HRQoL in individuals with CMT1A and manual function related to functional objectives for everyday physical medicine and rehabilitation practice. The manual function is complex and requires an overall, quantitative, qualitative and multidisciplinary assessment. Each tool (Pinch Gauge, Jamar, Sollerman, Jebsen, Nine-hole Peg) measures a specific element of manual function and is necessary when performing a grip function analysis.

Core Outcome Measurement Instruments for Clinical Trials of Total Knee Arthroplasty: A Systematic Review.

(1) Background: We have updated knowledge of the psychometric qualities of patient-reported outcome measures and, for the first time, systematically reviewed and compared the psychometric qualities of physical tests for patients with knee osteoarthritis who are undergoing total knee arthroplasty. This work was conducted to facilitate the choice of the most appropriate instruments to use in studies and clinical practice. (2) Methods: A search of medical databases up to December 2019 identified the studies and thus the instruments used. The quality of the measurement properties was assessed by the Bot et al. criteria. (3) Results: We identified 20 studies involving 25 instruments. Half of the instruments were questionnaires (n = 13). Among the condition-specific instruments, the Oxford knee score, Knee injury and Osteoarthritis Outcomes Score, and the Western Ontario and McMaster Universities Osteoarthritis index had the highest overall scores. Concerning generic tools, the Medical Outcomes Study Short-Form 36 (SF-36) or SF-12 obtained the highest overall score. For patient-specific tools, the Hospital Anxiety and Depression Scale ranked the highest. Some physical tests seemed robust in psychometric properties: 6-min Walk Test, five times Sit-To-Stand test, Timed Up and Go test strength testing of knee flexor/extensor by isometric or isokinetic dynamometer and Pressure Pain Threshold. (4) Conclusion: To make stronger recommendations, key areas such as reproducibility, responsiveness to clinical change, and minimal important change still need more rigorous evaluations. Some promising physical tests (e.g., actimetry) lack validation and require rigorous studies to be used as a core set of outcomes in future studies.

A TUG Value Longer Than 11 s Predicts Fall Risk at 6-Month in Individuals with COPD.

Risk of a fall is increased in individuals with chronic obstructive pulmonary disease (COPD), and is usually evaluated using the Berg Balance Scale (BBS), but this is difficult to perform in everyday clinical practice. We aimed to prospectively predict short-term fall recurrence in COPD patients using a predetermined cut-off value of the Timed Up and Go test (TUG). In stable COPD patients, we collected self-reported records of the number of falls in the previous year, and measured TUG and BBS scores for each individual. Records of fall recurrence were obtained prospectively at 6-months after the initial evaluation. Among the 50 patients recruited, 23 (46%) had at least one fall during the past year. The optimal diagnosis value for the TUG to detect a fall was 10.9 s with a sensitivity of 100% and a specificity of 97%. A cut-off of 11 s predicted fall recurrence with high sensitivity and specificity (93% and 74%, respectively). The TUG as well as the BBS score detected fallers, and a cut-off value of 11 s predicted fall recurrence. TUG could be easily incorporated into the scheduled functional evaluations of COPD patients, could predict the risk of a fall and when appropriate, could guide specific balance training exercises to prevent fall.

Walking Speed Is Correlated With the Isokinetic Muscular Strength of the Knee in Patients With Charcot-Marie-Tooth Type 1A.

Charcot-Marie-Tooth disease type 1A is the most common hereditary neuropathy. Affected individuals have a distal motor deficit, initially affecting the lower limbs and impairing walking performance. Isokinetic dynamometry can be used to objectively assess muscle strength of patients with neuromuscular disorders. No studies have evaluated the effect of muscle strength deficits of knee extensors and flexors on walking parameters for patients with Charcot-Marie-Tooth disease type 1A. The purpose of this study was to determine correlations between the isokinetic muscular strength of knee flexors and knee extensors and walk parameters for patients with Charcot-Marie-Tooth disease type 1A. isokinetic muscular strength of the knee was assessed on an isokinetic dynamometer (Cybex) and walking by instrumented walkway analysis (GaitRite). We included 33 patients (23 females, mean ± SD age 46.7 ± 13.3 yrs, mean ± SD body mass index 25.7 ± 4.6 kg/m). We found a correlation between walking speed and isokinetic muscular strength of knee extensors for the entire population and between walking speed and isokinetic muscular strength of knee extensors and knee flexors for patients younger than 50 yrs. Isokinetic dynamometry can provide objective measures of knee muscle strength, which is correlated with walking speed but not cadence or step/stride length of patients with Charcot-Marie-Tooth disease.

Measurement properties of the Star Excursion Balance Test in patients with ACL deficiency.

OBJECTIVES: To assess intrarater reliability, agreement and construct validity of the Star Excursion Balance Test (SEBT) in individuals with anterior cruciate ligament (ACL) deficiency and the ability of the SEBT to distinguish between healthy and ACL-deficient individuals. METHODS: Patients with ACL deficiency and healthy matched controls performed the SEBT and the One Leg Hop for Distance test (OLHD). Three consecutive trials were analyzed. Also evaluated were isokinetic thigh muscle strength and self-reported function. RESULTS: We included 33 patients and 33 matched controls. Intrarater reliability of the SEBT was good to excellent (ICC: 0.88-0.96). Agreement was estimated by the minimal detectable change (5.93-8.36 cm). Posteromedial and posterolateral direction and composite score of the SEBT were correlated with OLHD performance (r: 0.34-0.53, p < 0.05) and thigh muscle strength (r: 0.35-0.45, p < 0.05). Posteromedial and posterolateral direction and composite scores of the SEBT were significantly lower bilaterally for patients with ACL deficiency than controls (p < 0.05). CONCLUSION: The SEBT can be used in patients with ACL deficiency with good to excellent intrarater reliability. Construct validity of the SEBT versus the OLHD test and isokinetic thigh muscle strength can be confirmed. Furthermore, posteromedial and posterolateral performance of the SEBT can detect bilateral neuromuscular control deficits.