Podcast-Based Medical Education and Content Alignment with National Education Bodies.

Introduction: Despite the rapid expansion of medical education podcasts in the past decade, there are few efforts to characterize the landscape of available content for specific medical specialties. We trialed a method of rigorous characterization for the field of neurology. Materials and Methods: Using a censoring date of July 25, 2022, we queried the top three podcast platforms for neurology education podcasts: Apple Podcasts, Spotify, and Google Podcasts. We characterized podcasts based on total number of episodes, episode release frequency, target audience, and affiliation type. We characterized individual episodes by length and primary content area as defined by the Accreditation Council for Graduate Medical Education (ACGME) and American Board of Psychiatry and Neurology (ABPN). We compared content availability with content recommendations from these education bodies. Results: We identified 30 podcasts sharing 1772 episodes and totaling 46,287 min. The ACGME subspecialties most frequently covered were vascular neurology (5082 total min, 11%), neuroimmunology (4,406, 10%), and neuromuscular diseases (3,771, 8%). Subjects that were underrepresented included palliative neurology (89 min, 0.2%), neuropathology (95 min, 0.2%), and bioethics (171 min, 0.4%). The coverage of ABPN examination topics varied substantially from the content distribution for the examination. Discussion: The current landscape of neurology education podcasts features heterogeneous coverage of topics and varies considerably from recommended distribution of content by national education bodies. As podcasts have tremendous potential in supplementing neurology education, characterizing available content may help various stakeholders in the neurology education pipeline optimize the use of this e-learning modality. Supplementary Information: The online version contains supplementary material available at 10.1007/s40670-023-01971-0.

Diagnosis and treatment of hereditary transthyretin amyloidosis with polyneuropathy in the United States: Recommendations from a panel of experts.

Hereditary transthyretin (ATTRv; v for variant) amyloidosis is a rare, multisystem, progressive, and fatal disease in which polyneuropathy is a cardinal manifestation. Due to a lack of United States (US)-specific guidance on ATTRv amyloidosis with polyneuropathy, a panel of US-based expert clinicians convened to address identification, monitoring, and treatment of this disease. ATTRv amyloidosis with polyneuropathy should be suspected in unexplained progressive neuropathy, especially if associated with systemic symptoms or family history. The diagnosis is confirmed through genetic testing, biopsy, or cardiac technetium-based scintigraphy. Treatment should be initiated as soon as possible after diagnosis, with gene-silencing therapeutics recommended as a first-line option. Consensus is lacking on what represents "disease progression" during treatment; however, the aggressive natural history of this disease should be considered when evaluating the effectiveness of any therapy.

Hereditary gelsolin amyloidosis: a rare cause of cranial, peripheral and autonomic neuropathies linked to D187N and Y447H substitutions.

INTRODUCTION: Hereditary gelsolin (AGel) amyloidosis is a systemic disease that is characterised by neurologic, ophthalmologic, dermatologic, and other organ involvements. We describe the clinical features with a focus on neurological manifestations in a cohort of patients with AGel amyloidosis referred to the Amyloidosis Centre in the United States. METHODS: Fifteen patients with AGel amyloidosis were included in the study between 2005 and 2022 with the permission of the Institutional Review Board. Data were collected from the prospectively maintained clinical database, electronic medical records and telephone interviews. RESULTS: Neurologic manifestations were featured in 15 patients: cranial neuropathy in 93%, peripheral and autonomic neuropathy in 57% and bilateral carpal tunnel syndrome in 73% of cases. A novel p.Y474H gelsolin variant featured a unique clinical phenotype that differed from the one associated with the most common variant of AGel amyloidosis. DISCUSSION: We report high rates of cranial and peripheral neuropathy, carpal tunnel syndrome and autonomic dysfunction in patients with systemic AGel amyloidosis. The awareness of these features will enable earlier diagnosis and timely screening for end-organ dysfunction. The characterisation of pathophysiology will assist the development of therapeutic options in AGel amyloidosis.

Clinical Reasoning: A 23-Year-Old Man With Progressive Asymmetric Weakness and Numbness.

We report a case of a 23-year-old man who presented with progressive asymmetric weakness and numbness in his distal extremities over 4 months, with initial symptoms starting days after a coronavirus 2019 (COVID-19) vaccine booster. Initial neurologic examination was notable for distal weakness of both upper and lower extremities that was more pronounced on the left, complete areflexia, and decreased distal sensation to pinprick and vibration without loss of proprioception. Nerve conduction studies demonstrated a generalized, non-length-dependent, sensorimotor, demyelinating polyneuropathy, with conduction block seen in multiple compound muscle action potentials. Sensory nerve action potentials were normal in absolute terms but had asymmetric amplitudes.Based on the patient's nerve conduction studies, he was diagnosed with a specific immune-mediated neuromuscular disorder. He was started on intravenous immunoglobulin, but within days of the first infusions experienced a rare and potentially life-threatening complication. He received appropriate treatment and was started on alternative immunotherapy, after which his symptoms improved.Our case exemplifies the features of a specific subtype of a more common immune-mediated neuromuscular diagnosis with unique elements of history, examination, and nerve conduction studies that required interpretation in the clinical context. We also discuss a rare side effect of a commonly used immunotherapy and its risk factors and comment on the likelihood that this diagnosis may be related to a preceding COVID-19 vaccine booster.

Disparate healthcare access and telehealth-based hybrid consultations during the COVID-19 pandemic.

BACKGROUND: The coronavirus disease-2019 pandemic led to rapid expansion of telehealth services. This was speculated to improve healthcare access among underserved populations, including individuals unable to take time off work or arrange transportation. OBJECTIVE: We completed a quality improvement project to evaluate the feasibility of hybrid consultations that combined televisits and abbreviated in-person visits for neuromuscular referrals. METHODS: Using a censoring date of August 5, 2021, we reviewed all outpatient neuromuscular consultations from August 5, 2020 to February 5, 2021. For both hybrid and traditional in-person consultations, we reviewed no-show rates, completion rates of ordered diagnostic workup, and billing codes. For hybrid consultations only, we also reviewed intervals between initial televisit and subsequent examination and rates of video-enhanced versus audio-only televisits. RESULTS: During the study period, we completed 153 hybrid and 59 in-person new-patient consultations (no-show rates 9% and 27% respectively.) For hybrid consultations, 77% and 73% of laboratory and imaging studies were completed respectively, compared to 89% and 91% for in-person consultations. For hybrid visits, average RVUs (a marker for reimbursement) per consultation depended on whether audio-only televisits were billed as telephone calls or E/M visits per insurance payer rules, while video-enhanced televisits were uniformly billed as E/M visits. This resulted in average RVUs between 2.09 and 2.26, compared to 2.30 for in-person consultations. CONCLUSIONS: Telehealth-based hybrid neuromuscular consultations are feasible with minor caveats. However, the future of telehealth may be restricted by decreasing reimbursement rates particularly for audio-only televisits, limiting its potential to improve healthcare access.

Multimodal deep learning for Alzheimer's disease dementia assessment.

Worldwide, there are nearly 10 million new cases of dementia annually, of which Alzheimer's disease (AD) is the most common. New measures are needed to improve the diagnosis of individuals with cognitive impairment due to various etiologies. Here, we report a deep learning framework that accomplishes multiple diagnostic steps in successive fashion to identify persons with normal cognition (NC), mild cognitive impairment (MCI), AD, and non-AD dementias (nADD). We demonstrate a range of models capable of accepting flexible combinations of routinely collected clinical information, including demographics, medical history, neuropsychological testing, neuroimaging, and functional assessments. We then show that these frameworks compare favorably with the diagnostic accuracy of practicing neurologists and neuroradiologists. Lastly, we apply interpretability methods in computer vision to show that disease-specific patterns detected by our models track distinct patterns of degenerative changes throughout the brain and correspond closely with the presence of neuropathological lesions on autopsy. Our work demonstrates methodologies for validating computational predictions with established standards of medical diagnosis.

Neurological manifestations of hereditary transthyretin amyloidosis: a focus on diagnostic delays.

BACKGROUND: The recent availability of disease-modifying therapies for hereditary transthyretin amyloid (ATTRv) amyloidosis warrants urgency for earlier diagnosis and timely identification of active disease state among genetic carriers. METHODS: We reviewed clinical neurological data of all patients with ATTRv amyloidosis with initial visits at our amyloidosis centre between January 2016 and December 2018. We abstracted the signs and symptoms of neurological manifestations, as well as rates and patterns of diagnostic testing. RESULTS: Of 92 patients with 19 different transthyretin (TTR) mutations, 66 and 36% had symptoms attributed to large-fibre and small-fibre neuropathy, respectively, compared to 75 and 66% with corresponding examination findings. Thirty-six patients with V122I ATTR mutation had asymptomatic polyneuropathy identified on neurological examination, eight without concurrent cardiac disease. Seventy-three percent of patients had symptoms of carpal tunnel syndrome (CTS), while 26% had dysautonomia. The average delays between the onset of symptoms of large fibre neuropathy (LFN) or CTS to ATTRv amyloidosis diagnosis were 2.9 and 6.7 years, respectively. DISCUSSION: Our study found higher rates of polyneuropathy by examination than patient-reported symptoms, especially among those with V122I TTR amyloidosis, signalling asymptomatic polyneuropathy. Our findings suggest the need for routine neurological examinations and other testing for genetic carriers to achieve earlier identification of active disease state.

Optimizing the operation of an electrodiagnostic laboratory during the COVID-19 pandemic: A 6-month single-center experience.

INTRODUCTION/AIMS: The initial surge of the coronavirus disease-2019 (COVID-19) pandemic in early 2020 led to widespread cancellation of elective medical procedures in the United States, including nonurgent outpatient and inpatient electrodiagnostic (EDx) studies. As certain regions later showed a downtrend in daily new cases, EDx laboratories have reopened under the guidance of the American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM). In our reopening experience guided by the AANEM, we measured relevant outcomes to determine further workflow adaptations. We aimed to detail our experience and share the lessons learned. METHODS: We reviewed the clinical volumes, billing data, diagnosis distributions, and rates of COVID-19 exposure and transmission among patients and staff in our EDx laboratory during the first 6 months of reopening, starting on June 1, 2020. For context, we detailed the recent AANEM guidelines we adopted at our laboratory, supplemented by other consensus statements. RESULTS: We completed 816 outpatient studies from June 1 to December 1, 2020, reaching 97% of the total volume and 97% of total billing compared with the same time period in 2019. The average relative value units per study were similar. There were no major shifts in diagnosis distributions. We completed 10 of 12 requested inpatient studies during this period. There were no known COVID-19 transmissions between patients and staff. DISCUSSION: Our experience suggests that it is possible to safely operate an EDx laboratory under the guidance of the AANEM and other experts, with clinical volume and billing rates comparable to pre-pandemic baselines.