Evaluation of the International Spinal Cord Injury Bowel Function Basic Data Set Version 2.0 in Children and Youth With Spinal Cord Injury.

OBJECTIVES: To establish the utility and feasibility of the International Spinal Cord Injury/Dysfunction (SCI/D) Bowel Function Basic Data Set Version 2.0 in pediatric SCI populations. METHODS: This was a noninterventional, repeated measure design conducted in Pennsylvania, Maryland, Illinois, Kentucky, and South Carolina. The International Spinal Cord Injury/Dysfunction (SCI/D) Bowel Function Basic Data Set Version 2.0 was administered repeatedly, twice at the point of care and once over the phone. Time to complete the data set was recorded. Inter- and intrarater reliability was examined by intraclass correlation coefficients (ICC) with 95% confidence intervals (CI), and agreement between the bowel function basic data set variables and medical records was calculated using percentages. Intrarater reliability involved the same person administering the data once at the point of care and once over the phone. RESULTS: Forty-one children/youth ages 1 to 20 years participated in this study. Average time to complete the data set was 5.17 minutes. Interrater reliability was good to excellent (ICC ≥ 0.75) for most variables. Five variables had moderate interrater reliability (ICC = 0.05-0.74) and three had poor interrater reliability (ICC < 0.05). With the exception of one variable that had poor intrarater reliability (constipating agent, ICC = 0.00) and one that approached moderate reliability (digital evacuation, ICC = 0.74), intrarater reliability was good to strong for every bowel variable (ICC = 0.88-1.00). Only 12 (32%) medical records had explicit documentation of one or more of the variables on the Basic Bowel Function Basic Data Set V2.0. CONCLUSION: The results support future research with a larger and more diverse sample of children with SCI to build upon the psychometric work described herein.

The Role of Age and Initial Deformation on Final Cranial Asymmetry in Infants with Plagiocephaly Treated with Helmet Therapy.

BACKGROUND/AIMS: To characterize the impact of cranial asymmetry and age at initiation of therapy on final cranial asymmetry in infants with deformational plagiocephaly treated with helmet orthotics. METHODS: We conducted a single-center retrospective review of 45 pediatric patients <12 months of age with deformational plagiocephaly who underwent STARband cranial orthotic helmet treatment. Cranial asymmetry was measured using a 3-dimensional laser surface scanner and defined as a cranial vault asymmetry index (CVAI) >3.5%. RESULTS: Twenty-one patients (47%) were <6 months of age at the start of helmet therapy. A greater initial CVAI or age at therapy initiation correlated with a greater final CVAI (univariate analysis, r = 0.53, p < 0.001; r = 0.42, p = 0.004). Subgroup analysis of 18 patients beginning therapy at 4-5 months of age and 20 patients beginning therapy at 6-8 months of age revealed that only a change in the initial CVAI was associated with a change in the final CVAI (r = 0.43, p = 0.007). CONCLUSION: In a comparison of patients who initiated helmet therapy at 4-5 and 6-8 months of age, only cranial asymmetry at the outset of therapy was correlated with final cranial asymmetry (r = 0.43, p = 0.007).

Reliability Assessment of Various Sonographic Techniques for Evaluating Carpal Tunnel Syndrome.

Objectives-The aim of this study was to determine the intra- and inter-rater reliability of sonographic measurements of the median nerve cross-sectional area in individuals with carpal tunnel syndrome and healthy control participants.Methods-The median nerve cross-sectional area was evaluated by sonography in 18 participants with carpal tunnel syndrome (18 upper extremities) and 9 control participants (18 upper extremities) at 2 visits 1 week apart. Two examiners, both blinded to the presence or absence of carpal tunnel syndrome, captured independent sonograms of the median nerve at the levels of the carpal tunnel inlet, pronator quadratus, and mid-forearm. The cross-sectional area was later measured by each examiner independently. Each also traced images that were captured by the other examiner.Results-Both the intra- and inter-rater reliability rates were highest for images taken at the carpal tunnel inlet (radiologist, r = 0.86; sonographer, r = 0.87; inter-rater, r = 0.95; all P < .0001), whereas they was lowest for the pronator quadratus (r = 0.49, 0.29, and 0.72, respectively; all P < .0001). At the mid-forearm, the intra-rater reliability was lower for both the radiologist and sonographer, whereas the inter-rater reliability was relatively high (r = 0.54, 0.55, and 0.81; all P < .0001). Tracing of captured images by different examiners showed high concordance for the median cross-sectional area at the carpal tunnel inlet (r = 0.96-0.98; P < .0001).Conclusions-The highest intra- and inter-rater reliability was found at the carpal tunnel inlet. The results also demonstrate that tracing of the median nerve cross-sectional area from captured images by different examiners does not contribute significantly to measurement variability.

Electrodiagnosis in neuromuscular disease.

Electromyography (EMG) is an important diagnostic tool for the assessment of individuals with various neuromuscular diseases. It should be an extension of a thorough history and physical examination. Some prototypical characteristics and findings of EMG and nerve conduction studies are discussed; however, a more thorough discussion can be found in the textbooks and resources sited in the article. With an increase in molecular genetic diagnostics, EMG continues to play an important role in the diagnosis and management of patients with neuromuscular diseases and also provides a cost-effective diagnostic workup before ordering a battery of costly genetic tests.

Lung insufflation capacity in neuromuscular disease.

OBJECTIVE: To compare maximal passive lung insufflation capacity (LIC) with lung inflation by air stacking (to maximum insufflation capacity [MIC]) and with vital capacity (VC); to explore relationships between these variables that correlate with glottic function and cough peak flows (CPF); to demonstrate the effect of routine inflation therapy on LIC and MIC; and to determine the relative importance of lung inflation therapy as a function of disease severity. DESIGN: Case series of 282 consecutive neuromuscular disease (NMD) clinic patients 7 yrs and older with VC <70% of the predicted normal value. All cooperative patients meeting these criteria were prescribed thrice-daily air stacking and/or maximal passive lung insufflation to pressures of 40-80 cm H2O, and they underwent measurements of VC, MIC, LIC, and unassisted and assisted CPF on every visit. RESULTS: Means +/- standard deviations for VC, MIC, and LIC were 1131 +/- 744, 1712 +/- 926, and 2069 +/- 867 ml, respectively, and, for unassisted and assisted CPF, they were 2.5 +/- 2.0 and 4.3 +/- 2.2 liters/sec, respectively, with all differences statistically significant (P < 0.001). MIC minus VC correlated inversely with LIC minus MIC (P = 0.01) and, therefore, with glottic function. Both MIC and LIC increased with practice (P < 0.001). Increases in LIC but not MIC over VC were greatest for patients with the lowest VC (P < 0.05). There were no complications of lung mobilization therapy. CONCLUSIONS: Passive lung insufflation can distend the lungs of patients with NMD significantly greater than air stacking, particularly when glottic and bulbar-innervated muscle dysfunction is severe. LIC, MIC, and VC measurements permit quantifiable assessment of glottic integrity and, therefore, bulbar-innervated muscle function for patients with NMD. The patients who benefit the most from insufflation therapy are those who have the lowest VC.