Klanderman, Molly

AbstractBackgroundThe prognosis in patients with advanced cardiac amyloidosis (CA) remains poor.ObjectivesWe sought to describe survival post heart transplantation (HT) in amyloid compared with non‐amyloid recipients, highlight waitlist times within the new allocation system across three Organ Procurement and Transplantation Network (OPTN) regions, and describe multiorgan transplantation (MOT) in hereditary amyloidosis.MethodsThis is a retrospective review of end‐stage CA patients who underwent HT at Mayo Clinic from January 2007 to December 2020. Wait time was compared in the new versus old OPTN allocation era starting December 18, 2018 by Wilcoxon rank sum test. All‐cause mortality for those with and without CA was compared using Kaplan–Meier estimates with log rank analysis, censoring December 16, 2022.ResultsFifty‐five patients with CA underwent HT between 2007 and 2020, 8 light chain amyloidosis (AL) (14.5%), 28 hereditary transthyretin (ATTRv) (50.9%), 17 wildtype transthyretin (ATTRwt) (30.9%), and 2 hereditary apolipoprotein A1 (AApoA1) amyloidosis patients (3.6%). No significant difference in overall survival post‐transplant was seen in amyloid compared with non‐amyloid (p = 0.816). Median time to HT was shorter in the new system, 45 days (IQR 24, 78) versus 174 days (IQR 76.5, 483.5), p = 0.006. There was a decline in MOT in hereditary amyloidosis over time with the concomitant rise in disease‐targeted therapies.ConclusionsHT survival in CA patients was similar to non‐amyloid patients. The new allocation system benefits this cohort with shorter wait times. There is less MOT in hereditary amyloidosis with increased utilization of disease‐targeted therapy.

Abstract Background Movement clinical neurophysiology studies can distinguish myoclonus, tremor, and other jerky movements; however, there has been limited demonstration of their real‐world clinical impact. Objective The aim was to investigate movement study utility in clarifying movement classification and guiding patient management. Method A retrospective study of myoclonus‐related movement studies was performed. Results Of 262 patients referred for consideration of myoclonus, 105 (40%) had myoclonus, 156 (59%) had no myoclonus (the commonest alternative classifications were functional jerks and tremor), and 1 was uncertain. An additional 29 studies identified myoclonus without prior clinical suspicion. A total of 119 of 134 (89%) myoclonus patients had a specific neurophysiologic subtype identified, most commonly cortical (64, 54%). Diagnostic differential narrowed in 60% of patients, and a new diagnosis was made in 42 (14%) patients. Medication changes were made in 151 patients (52%), with improvement in 35 of 51 (67%) with follow‐up. Conclusions Movement studies effectively determined movement classification and identified unsuspected myoclonus, leading to changes in diagnosis and management. © 2024 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

ImportancePatients are frequently copositive for multiple allergens simultaneously, either due to chemical similarity or simultaneous sensitization. A better understanding of copositivity groups would help guide contact avoidance.ObjectiveTo use patient data to systematically determine copositivity groups in the Mayo Clinic Standard Series.Design, Setting, and ParticipantsIn this retrospective cross-sectional analysis, the Mayo Clinic patch test database was queried for pairwise copositivity rates in the 80 allergen Mayo Clinic Standard Series between 2012 and 2021. Data were collected from 3 tertiary care sites of the Mayo Clinic Contact Dermatitis Group and a total of 5943 patients were included, comprising all patients undergoing patch testing to the Mayo Clinic Standard Series allergens.Main Outcomes and MeasuresCopositivity rates between every 2 allergens in the 80-allergen Mayo Clinic Standard Series were estimated. After background correction, copositivity rates were analyzed using unsupervised hierarchical clustering to systematically identify copositivity groups in an unbiased manner.ResultsOverall, 394 921 total patches were applied to 5943 patients (4164 [70.1%] women, 1776 [29.9%] men, with a mean [SD] age of 52.3 [18.8] years ), comprising 9545 positive reactions. After background correction based on overall positivity rates, hierarchical clustering revealed distinct copositivity groups. Many were supported by prior literature, including formaldehyde releasers, cobalt-nickel-potassium dichromate, acrylates, 3-dimethylaminopropylamine-amidoamine-oleamidopropyl dimethylamine, alkyl glucosides, budesonide-hydrocortisone-17-butyrate, certain fragrances, compositae-sesquiterpene lactone mix, mercapto mix-mercaptobenzothiazole, carba mix-thiuram mix, and disperse orange-p-phenylenediamine. However, novel associations were also found, including glutaraldehyde-sorbitan sesquioleate, benzalkonium chloride-neomycin-bacitracin, bronopol-methylchloroisothiazolinone-methylisothiazolinone, and benzoic acid-iodopropynyl butylcarbamate.Conclusions and RelevanceThis retrospective cross-sectional analysis found that copositivity rates varied between allergens; allergens with extremely high positivity rates demonstrated nonspecific copositivity to multiple other allergens. Background correction based on positivity rates followed by hierarchical clustering confirmed prior known copositivity groups, contaminants and/or excipients leading to copositivity, and novel associations to guide contact avoidance.

Objectives: The purpose of this study is to evaluate the effect of low-intensity blood flow restriction (LI- BFR) training utilized in a pre-rehabilitative setting for patients undergoing anterior cruciate ligament reconstruction (ACLR) on postoperative outcomes by minimizing postoperative atrophy and impairment of the quadriceps. We hypothesize that postoperative strength and hypertrophy of the quadriceps will be greater in a population that undergoes preoperative LI-BFR training than that of a population that undergoes low-intensity sham control (LI-SHAM) training. Methods: Twenty-eight subjects undergoing primary ACLR underwent 2 weeks of either a BFR intervention or a SHAM intervention at a rate of 2 sessions per week before surgery. Quadriceps strength and cross-sectional area were measured as well as other exploratory outcomes were measured before and after the intervention before surgery and at 5 different time points after surgery, through 6 months postoperative. Quadriceps strength was assessed through handheld dynamometry and a leg press test and quadriceps cross-sectional area was assessed using musculoskeletal ultrasound imaging. Results: Adjusting for sex, time, and strength/hypertrophy at baseline, there were no significant differences found between treatment groups for the primary endpoints. Compared to 24-48 hours pre- op, this suggests that the post-op average hypertrophy remains significantly lower through 24 weeks post-op, while leg strength is similar at 24 weeks post-op as measured by handheld dynamometer and even significantly larger at 24 weeks as measured by the leg press test. Conclusions: A 2-week preoperative program of LI-BFR training may not be effective to improve postoperative quadriceps strength and hypertrophy after ACLR.

ABSTRACTBackgroundTreating functional movement disorder (FMD) with motor retraining is effective but resource intensive.ObjectivesIdentify patient, disease, and program variables associated with favorable treatment outcomes.MethodsRetrospective review of the 1 week intensive outpatient FMD program at Mayo Clinic in Minnesota from February 2019 to August 2021. Outcomes included patient‐reported measures (Canadian Occupational Performance Measure‐Performance and Satisfaction subscales [COPM‐P and COPM‐S, range 0–10] and Global Rating of Change [GROC, −7 to +7]) and a retrospective investigator‐rated scale (0–3, worse/not improved to significantly improved/resolved). Linear regression models identified variables predicting favorable outcomes.ResultsParticipants (n = 201, 74% female, mean age = 46) had median FMD duration of 24 months. The commonest FMD subtypes were gait disorder (65%), tremor (41%) and weakness (17%); 53% had ≥2 subtypes. Most patients (88%) completed a therapeutic screening process before program entry. Patient‐reported outcomes at the end of the week improved substantially (COPM‐P average change 3.8 ± 1.9; GROC post‐program average 5.5 ± 1.7). Available investigator‐rated outcomes from short‐term follow‐up were also positive (102/122 [84%] moderately to significantly improved/resolved). Factors predicting greater improvement in COPM‐P were completing therapeutic screening, higher number of non‐motor symptoms, shorter FMD duration, earlier program entry, lower baseline COPM scores, and (among screened patients) higher GROC between therapeutic screening and program start.ConclusionPatients with diverse FMD subtypes improved substantially over a 1 week period. Utilization of therapeutic screening and greater improvement between therapeutic screening and program start were novel predictors of favorable outcomes. Non‐motor symptoms did not preclude positive responses, although patients with predominant non‐motor burden were excluded.

Objectives Somatostatin receptor (SSTR) PET imaging is utilized with increasing frequency in the clinical management of neuroendocrine tumors. Incidental PET-avid CNS lesions are commonly noted and presumed to be meningiomas. However, SSTR PET lacks specificity for meningioma identification. This study aimed to clarify the role of SSTR-based imaging in the classification of incidental CNS lesions based on current clinical practice. Methods Patients who underwent both Ga-68-DOTATATE PET and brain MRI and had an incidental CNS lesion identified with a radiographic prediction of meningioma via one (discordant prediction) or both (concordant prediction) imaging modalities were retrospectively analyzed. Imaging indication, semiquantitative measures, and clinical history were recorded. Results Among 48 patients with a CNS lesion identified on both imaging modalities, most scans were performed for a history of neuroendocrine tumor (64.6%). Cases with concordant lesion-type prediction of meningioma between imaging modalities (N = 24) displayed a significantly higher SUV max (median 7.9 vs. 4.0; P = 0.008) and Krenning score (median 3.0 vs. 2.0; P = 0.005) on Ga-68-DOTATATE PET compared with cases with a discordant prediction of meningioma (N = 24). In cases with lower SUV max values, Ga-68-DOTATATE was more likely to discordantly predict meningioma without agreement by the corresponding MRI. Prior cranial radiation or use of somatostatin mimetics did not affect quantitative radiographic measures, and MRI-based tumor size was similar across groups. Conclusion Lesions with increased avidity may be more confidently predicted as meningioma in Ga-68-DOTATATE PET scans, whereas there is more discrepancy in prediction among low SUV cases.

Background: Atypical Atrial Flutter (AAFL) prevalence is increasing due to the escalating Atrial Fibrillation (AF) ablations and cardiac surgeries. We wanted to explore the outcome of the AAFL ablation, considering the recent changes in mapping and ablation. Methods: This study was approved by the Institutional Review Board (IRB) of Mayo Clinic hospital. We retrospectively studied 419 patients who had undergone AAFL ablation at Mayo Clinic from January 2017 to June 2022. Thirteen patients declined research authorization, and 19 patients were lost to follow-up during the 90-day blanking period, resulting in a sample size of 387. The median follow-up time for patients was 25.7 months (95% CI 23.7, 32.3). Results: Recurrent symptoms with documentation of atrial arrhythmia Occurred in 226/387 (58.4%) patients, of which 151/226 (66.8%) occurred within the first year. The median time to recurrence was 8.5 months (max 57.8 months). Eleven patients died during the study period, 9 of whom experienced recurrence prior to death. Overall, the median recurrence-free survival (RFS) time was 16.6 months (95% CI 13.2, 20.0) with a 1-year RFS rate of 57.2% (95% CI 52.2, 62.7%). Acute termination occurred 324/387 (83.7%) during the ablation. The 1-year RFS rate was 58.9% (95% CI 53.5%, 64.9%) for patients with acute termination and 49.0% (95% CI 37.9%, 63.4%) for those without acute termination. The rate was not significantly different based on acute termination status (p = 0.11). Conclusions: The one-year RFS rate of 57.2% following AAFL ablation, even though 83.7% achieved acute termination during the procedure, signifies the extent of the underlying substrate abnormalities.

Introduction: Coccidioidal meningitis (CM) is the most lethal form of disseminated coccidioidomycosis. Current guidelines recommend fluconazole as initial therapy but there has been a paucity of data regarding failure of fluconazole and optimal fluconazole dosage in the treatment of CM. We conducted this study to understand risk factors for fluconazole failure. Methods: We conducted a single-center retrospective chart review of patients diagnosed with coccidioidal meningitis between 1 January 1988 and 15 May 2021. Relevant demographic and clinical variables were collected, along with outcomes including treatment failure and death at any point. Univariate tests were conducted using the chi-squared goodness of fit test and analysis of variance. Results: Among 71 patients who began treatment for CM with fluconazole, 22 (31%) developed worsening meningitis at a median time of 206 days. Longer time from symptom onset to diagnosis of CM was a risk factor for fluconazole failure. Although the absolute failure rate of fluconazole starting dose of 400 mg daily was higher than that of 800 mg daily, the differences did not achieve statistical significance (p = 0.39). Conclusion: Fluconazole failure is not uncommon in the treatment of CM. A dose of 800 mg daily was not superior to a dose of 400 mg daily. All patients on fluconazole for CM require close monitoring.