Diagnostic accuracy of gray scale muscle ultrasound screening for pediatric neuromuscular disease.

OBJECTIVE: Gray scale ultrasound (US) has been demonstrated to be a sensitive and specific tool in the diagnosis of pediatric neuromuscular disease (NMD). With recent advances in genetic testing, the diagnostic work up for NMD has evolved. The purpose of this study was to compare the current diagnostic value of gray scale US to previously defined sensitivities and specificities to determine when this test can add value to a patient's diagnostic workup. METHODS: Standardized quantitative gray scale US imaging was performed on 148 pediatric patients presenting for electrodiagnostic testing to evaluate for NMD. Patients were categorized as having an NMD, a non-NMD, or as "uncertain." The US results were defined as normal, borderline or abnormal based on echointensity values. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of the test were calculated. RESULTS: Forty-five percent of the patients had an NMD, 54% a non-NMD, and in 1% the diagnosis remained uncertain. US was abnormal in 73% of myopathies, 63% of neuromuscular junction disorders, 60% of generalized neuropathies and 58% of focal neuropathies. After excluding patients in whom muscle US was not expected to be abnormal (eg, sensory neuropathy), sensitivity was 83%, specificity 79%, PPV 75%, NPV 86%, and accuracy 81%. CONCLUSIONS: Quantitative gray scale muscle US still has good diagnostic value as a screening tool in pediatric NMD. As with any diagnostic test, muscle US is best used in conjunction with history and physical examination to increase specificity and diagnostic yield.

Reliability of a novel ultrasound system for gray-scale analysis of muscle.

INTRODUCTION: Ultrasound is increasingly used as an adjunct in the diagnosis of neuromuscular disease by measuring muscle thickness and echointensity (EI). Reproducibility is limited because of variations in scanning technique and proprietary algorithms that alter EI values. METHODS: We developed a standardized scanning protocol and a portable machine without any postimaging processing. Ten subjects underwent scanning of 6 muscles by 3 sonographers on 2 separate days. One of the sonographers repeated the protocol with 4 different machine/transducer combinations. Gray-scale values were measured from each image with the use of a region of interest (ROI) box. RESULTS: Combined intraclass correlation coefficients were 0.92 (intra-rater), 0.88 (inter-rater), and 0.96 (inter-system). The biceps had the highest variability (coefficient of variance [COV] 12.7%), and the medial gastrocnemius had the lowest variability (COV 7.4%). CONCLUSIONS: We demonstrate excellent reliability of a reproducible ultrasound system for gray-scale analysis of muscle that has potential applicability as a screening tool for neuromuscular disease. Muscle Nerve 56: 408-412, 2017.

Neuromonitoring, Cerebrospinal Fluid Drainage, and Selective Use of Iliofemoral Conduits to Minimize Risk of Spinal Cord Injury During Complex Endovascular Aortic Repair.

PURPOSE: To review outcomes of continuous motor/somatosensory-evoked potential (MEP/SSEP) monitoring, cerebrospinal fluid drainage, and selective use of iliofemoral conduits in patients undergoing endovascular repair of descending thoracic aneurysm (DTA) and thoracoabdominal aortic aneurysms (TAAAs). METHODS: The clinical data of 49 patients (mean age 75±8 years; 38 men) who underwent endovascular repair of DTA and TAAAs (2011-2014) were reviewed. All patients had cerebrospinal fluid drainage, permissive hypertension (mean arterial pressure ≥80 mm Hg), and MEP/SSEP monitoring. There were 44 (90%) patients with TAAAs and 5 (10%) with DTA. Types I and II TAAAs were repaired in staged procedures. Iliofemoral conduits were used for small iliac arteries and to minimize time of lower extremity ischemia in patients with difficult anatomy. In patients with changes in MEP/SSEPs, a standardized protocol was employed to optimize spinal cord perfusion and restore lower extremity blood flow. Endpoints were mortality, spinal cord injury (SCI), and lower extremity ischemic complications. RESULTS: Sixteen (33%) patients had staged TAAA repair. A total of 163 visceral arteries were targeted by fenestrations and branches (mean 3.7±1.0 vessels/patient). Temporary iliofemoral conduits were used in 16 limbs/14 patients. A stable MEP/SSEP was achieved in all patients. Thirty-one (63%) patients had a ≥75% decrease in MEP/SSEP amplitude in 50 limbs starting on average 75±28 minutes after obtaining vascular access. MEP/SSEP amplitude improved with maneuvers in 12 (39%) patients and returned to baseline with restoration of lower extremity flow in all except 1 patient who developed immediate SCI. Thirty-day mortality was 4%. Three (6%) patients had SCI, 2 permanent and 1 temporary at 14 days. There were no lower extremity ischemic complications. CONCLUSION: Neuromonitoring predicted immediate SCI and allowed use of a protocol to optimize spinal cord and lower extremity perfusion during complex endovascular aortic repair. Larger clinical experience is needed to evaluate the efficacy of neuromonitoring to prevent SCI.