Citations

BACKGROUND MRI-guided targeted fascicular biopsy has been a valuable technique for diagnosing idiopathic neuropathies. It has an 80% yield and a minor permanent morbidity rate of 5%. OBSERVATIONS A 48-year-old woman with a history of localized amyloidosis to the orbital muscle 2 years earlier presented with new neurological symptoms. Extensive evaluations including skin, muscle, and distal cutaneous nerve biopsies were negative, and empirical treatment trials did not improve her condition. An MRI-guided targeted biopsy of the right ulnar nerve in the distal arm was planned. During surgery, a medial triceps branch arising from the ulnar nerve was identified and was biopsied along with a neighboring fascicle of the ulnar nerve to the flexor carpi ulnaris. The pathology of the ulnar nerve fascicle but not the contiguous triceps branch showed amyloid (AL [kappa] subtype on mass spectroscopy). The patient was started on treatment. LESSONS The positive biopsy of the ulnar nerve fascicle allowed targeted treatment of the neural amyloidosis. While the negative biopsy of the triceps branch could be attributed to sampling error, it could also be due to the fact that this branch found within the ulnar nerve is thought to originate from the radial nerve. https://thejns.org/doi/10.3171/CASE25484

Purpose: In 2022, the American Board of Physical Medicine and Rehabilitation (ABPMR) contracted with Professional Services Industries LLC to complete a Pediatric Rehabilitation Medicine (PRM) practice analysis. The goal was to develop an updated examination content outline that accurately depicts the knowledge and skills expected for a competent PRM physician following completion of training. The purpose of this paper is to describe the process of practice analysis and to present candidate perceptions of scope of practice and relevancy of content from the first two examinations in which the new outline was applied. Methods: The practice analysis process was undertaken in four distinct stages: subject matter expert meetings to determine the tasks and knowledge areas; the development, issuance and analysis of a national survey; development of exam specifications; and results verification. Results were analyzed by a psychometrician and team to determine mean relative importance of task and knowledge items. A new outline was then created and used for the administration of the 2023 and 2024 ABPMR PRM Certification Examinations. Results: All active board certified PRM physicians identified were sent surveys via e-mail. One hundred ninety six (61.1%) respondents completed the survey. The top five task statements in order of perceived importance were performing history and physical examinations, managing muscle and tone abnormalities, prescribing orthoses and equipment, prescribing therapy services, and making appropriate specialty care referrals. The top five knowledge statements in order of perceived importance were cerebral palsy, spinal cord injury, abnormal growth and development, neuromuscular disorders, and musculoskeletal conditions. The new PRM Examination content outline created included six domains and 22 subdomains of knowledge content areas. A higher percentage of initial certification candidates sitting for the 2023 and 2024 ABPMR PRM Certification Examinations selected “Strongly Agree” or “Agree” when responding to the examination content reflecting both scope of training and relevancy. Conclusion: A practice analysis process was undertaken resulting in a new content outline for the ABPMR PRM Certification Examination. Following the implementation of the new content outline, initial certification candidate survey responses showed improved favorability regarding content reflecting scope of training and relevancy.

Objectives To compare electrical stimulation and speech perception in vestibular schwannoma (VS) patients across treatment modalities and standard cochlear implant (CI) patients. Study Design Retrospective review. Setting Tertiary academic center. Patients Sixty-seven CI ears consisting of 23 standard CI controls and 44 VS patients with ipsilateral CI. VS patients were separated by treatment modality: 24 microsurgical resection, 15 radiation therapy, and 5 observation. Eleven VS patients with CI did not receive auditory sensation from electrical stimulation. Interventions VS microsurgical resection, radiation therapy, and observation. Main Outcome Measures Threshold (T-levels), comfort (C-levels), consonant-nucleus-consonant (CNC) word score, and AzBio sentence recognition scores. Results Patients who underwent microsurgical resection required significantly higher (p < 0.001) T-levels and C-levels compared with the standard CI group. Stimulation levels in the VS radiation therapy and observation groups were higher but not significantly different compared with standard CI controls. Overall rate of CI nonstimulation across all VS patients was 25%: 38% for microsurgery and 13% for radiation. Average CNC score for microsurgery group was 18% compared with 44% for radiation, 55% for observation, and 68.5% for standard CI controls. CNC word and AzBio scores were significantly lower (p < 0.001) for the microsurgery group compared with standard CI group. Conclusions Resection may negatively affect auditory nerve function, leading to higher stimulation levels, increased risk of nonstimulation, and poorer postoperative CI speech outcomes compared with observation or radiosurgery. When medically appropriate, nonsurgical VS management may be used to allow for optimization of CI programming and postoperative hearing outcomes.

Abstract Background and Aims Accurate differentiation of cardiac amyloidosis (CA) from phenotypic mimics remains challenging using current clinical and echocardiographic techniques. The accuracy of a novel artificial intelligence (AI) screening algorithm for echocardiography-based CA detection was assessed. Methods Utilizing a multisite, multiethnic dataset (n = 2612, 52% CA), a convolutional neural network was trained to differentiate CA from phenotypic controls using transthoracic apical four-chamber video clips. External validation was conducted globally across 18 sites including 597 CA cases and 2122 controls. Classification accuracy was assessed on the entire external validation dataset, and subgroup analyses were performed both on technetium pyrophosphate scintigraphy referrals, and individuals matched for age, sex, and wall thickness. Model accuracy was also compared with the transthyretin CA score and the increased wall thickness score within a subset of older heart failure with preserved ejection fraction patients with increased wall thickness. Results Cardiac amyloidosis patients and controls displayed similar age, sex, race, and comorbidities. After the removal of uncertain AI predictions (13%), model discrimination and classification were excellent for the entire external validation dataset [area under the receiver operating characteristic curve (AUROC) 0.93, sensitivity 85%, specificity 93%], irrespective of CA subtype (sensitivity: light-chain = 84%, wild-type transthyretin = 85%, and hereditary transthyretin = 86%). Performance was maintained in subgroup analysis in patients clinically referred for technetium pyrophosphate scintigraphy imaging (AUROC 0.86, sensitivity 77%, specificity 86%) and matched patients (AUROC 0.92, sensitivity 84%, specificity 91%). The AI model (AUROC 0.93) also outperformed transthyretin CA score (AUROC 0.73) and increased wall thickness (AUROC 0.80) scores. Conclusions This AI screening model—using only an apical four-chamber view—effectively differentiated CA from other causes of increased left ventricular wall thickness.

Abstract RATIONALE: Susceptibility to severe COVID-19 and long COVID may be influenced by pre-existing lung structure. ECG-gated, non-contrast cardiac CT scans, originally acquired for assessing coronary artery calcium, are available in multiple longitudinal cohort studies and have been previously validated against lung CT scans for densitometry; however, protocol variation across cohorts may hinder direct comparison. To address this, we developed a quality control (QC) rubric to grade cardiac CT scans for standardized quantitative lung measures across cohorts. METHODS: C4R assessed SARS-CoV-2 infection and outcomes in 14 NHLBI cohorts, of which 10 (six community-based and four longitudinal case-control studies) included CT scans (12,459 participants with lung and 13,752 with cardiac CTs). Logic rules were applied to categorize scans (Table): Grade “A-B” scans had 2-3.5mm slice thickness, at least 20 axial slices, no lung cropping in axial view and lung and airway volumes greater than the cohort-specific 1st percentile. “C-D” scans had one lung obscured by cropping around the heart, so quantitative measures were restricted to the lung in complete view. “F-H” scans were deemed unusable, suffering from low slice count (&lt;20), severe image cropping, low lung or airway volumes (&lt; 1st%ile). Visual inspection was performed on a sample from each category to ensure accuracy. Percent of lung voxels in low attenuation areas &lt; -950 HU (LAA950), high attenuation areas (-600 to -250 HU, HAA) and 15th percentile HU value (perc15) were measured. Within-participant variability was quantified using the intraclass correlation coefficient (ICC). RESULTS: We performed QC on cardiac CT scans from an initial sample of 6,275 participants from three C4R cohorts (ARIC, CARDIA, MASALA), imaged with GE or Siemens scanners. Of these, 5,125 (81.7%) were graded “A-B”, 496 (7.9%) were “C-D” and 654 (10.4%) were “F-H”. The percentage of “F-H” scans varied widely across cohorts [&lt;1% to 26%] due to image cropping. Among the 5,621 cardiac CT scans graded A-D, the mean±SD for LAA950, HAA and perc15 respectively were 1.1±2.1%, 7.2±4.7% and -887±33 HU. Repeated imaging was available for 107 participants (time interval 5.1±0.7y). For paired scans in grades A-B (n=101/107), the within-participant correlation for LAA950, HAA and perc15 were 0.91, 0.38 and 0.74 respectively. CONCLUSION: Initial application of QC to cardiac CT scans identified scans with high reproducibility, with the aim to provide valid quantitative lung metrics on over 20,000 highly characterized participants in C4R.

This cohort study examines the validity of an electronic health record data model for organ transplantation.

Abstract Rationale Increased risk of coronavirus disease (COVID-19) hospitalization and death has been reported among patients with clinical lung disease. Objectives To test the association of objective measures of prepandemic lung function and structure with COVID-19 outcomes in U.S. adults. Methods Prepandemic obstruction (FEV1/FVC &lt; 0.70) and restriction (FEV1/FVC ⩾ 0.7, FVC &lt; 80%) were defined based on the most recent spirometry exam conducted in 11 prospective U.S. general population–based cohorts. Severe obstruction was classified by FEV1 &lt; 50%. Percentage emphysema, percentage high-attenuation areas, and interstitial lung abnormalities were defined on computed tomography in a subset. Incident COVID-19 was ascertained via questionnaires, serosurvey, and medical records from 2020 to 2023 and classified as severe (hospitalized or fatal) or nonsevere. Cause-specific hazard models were adjusted for sociodemographics, anthropometry, smoking, comorbidities, and COVID-19 vaccination status. Measurements and Main Results Among 29,323 participants (mean age, 67 yr), there were 748 severe incident COVID-19 cases over median follow-up of 17.3 months from March 1, 2020. Greater hazards of severe COVID-19 were associated with severe obstruction (vs. normal; adjusted hazard ratio [aHR], 2.11; 95% confidence interval [CI], 1.02–1.27), restriction (vs. normal; aHR, 1.40; 95% CI, 1.12–1.76), and percentage emphysema (highest vs. lowest quartile; aHR, 1.64; 95% CI, 1.03–2.61), but not greater high-attenuation areas or interstitial lung abnormalities. COVID-19 vaccination provided greater absolute risk reduction in these groups. Results were similar in participants without smoking, obesity, or clinical cardiopulmonary disease. Conclusions Prepandemic severe spirometric obstruction, spirometric restriction, and greater percentage emphysema lung on computed tomography were associated with risk of severe COVID-19. These findings support enhanced COVID-19 risk mitigation for individuals with impaired lung health and warrant further mechanistic studies on interactions of lung function, structure, and vulnerability to acute respiratory illnesses.