The CMTHI represents a valid and reliable outcome to assess patient-reported disease burden in CMT. Ann Neurol 2018;84:225-233.
Objective To evaluate in-vivo reflectance confocal microscopy (RCM) of Meissner’s corpuscles (MC) in diabetic distal symmetric polyneuropathy (DSP). Methods Forty-three adults with diabetes and 21 control subjects underwent RCM of MC density at the fingertip of digit V, thenar eminence (TE), and arch of the foot, ankle skin biopsy for epidermal nerve fiber density (ENFD), electrophysiological studies, monofilament threshold testing, and timed vibration at the toe. Subjects with diabetes were subdivided into groups with and without clinical DSP using the American Academy of Neurology (AAN) case definition and neuropathy outcomes were compared across groups. Results Both diabetic groups (with and without AAN clinical DSP criteria) had objective evidence of peripheral sensory involvement using conventional sensory measures, although those with clinical DSP criteria had greater abnormalities. MC densities were lower in the entire diabetic group at the TE and digit V relative to controls. MC densities at all imaging sites were associated with corresponding conventional sensory measures. MC densities were reduced in subjects without AAN clinical DSP criteria at the TE and digit V compared to controls whereas conventional upper limb sensory measures did not differ between these groups. Conclusions In-vivo RCM of MC density at digit V is a non-invasive, painless, objective marker in diabetes that offers a window into early large fiber sensory nerve terminal loss. Further studies are needed to determine whether RCM of MCs can identify quantitative changes in DSP associated with disease progression or treatment.
Objective Experimental therapies under development for Friedreich’s Ataxia (FRDA) require validated biomarkers. In‐vivo reflectance confocal microscopy (RCM) of skin is a noninvasive way to quantify Meissner’s corpuscle (MC) density and has emerged as a sensitive measure of sensory polyneuropathies. We conducted a prospective, cross‐sectional study evaluating RCM of MCs and conventional peripheral nerve measures as candidate peripheral nerve markers in FRDA. Methods Sixteen individuals with FRDA and 16 age‐ and gender‐matched controls underwent RCM of MC density and morphology, skin biopsies for epidermal nerve fiber density (ENFD), nerve conduction studies (NCS), and quantitative sensory testing (QST) including touch, vibration, and cooling thresholds. Results MC densities were measurable in all participants with FRDA, and were lower at digit V (hand), thenar eminence, and arch (foot) compared to controls. By contrast, sensory NCS showed floor effects and were obtainable in only 13% of FRDA participants. QST thresholds for touch, vibration, and cooling were higher at the hand and foot in FRDA than controls. Reductions in ENFDs were present in more severely affected individuals with FRDA (Friedreich’s Ataxia Rating Scale (FARS) >60) compared to matched controls, although skin biopsies were not well tolerated in children. MC densities, ENFDs, and touch and vibration thresholds were associated with clinical disease severity (FARS and modified FARS) and duration since symptom onset. Interpretation MC density, ENFD, and QST thresholds provide structural and physiologic markers of sensory involvement in FRDA. Longitudinal evaluation is needed to determine whether these measures can identify changes associated with disease progression or treatment.
Objective We aimed to improve the education of medical students involved in a longitudinal perinatal health improvement project in Gowa, Malawi. Design We conducted qualitative interviews with students who participated in the project, reviewed their quantitative reports, and assessed the application of methodologies consonant with the learning objectives of a novel community health improvement course within their experience. Setting The Gowa Health Promotions Project, designed to improve perinatal care for women and their families within the Gowa Health Clinic, used community participatory research strategies. Method Medical students partnered with clinic workers and the local residents, evaluated, and revised an existing perinatal educational program. Qualitative and quantitative health and program data were collected, and program revisions were implemented. The value of the student experiences as a public health educational tool was evaluated by the authors. Results Project sustainability was enhanced by a fellowship and planning for sequential students. The community health course structure and goals enhanced learning in the project. Engagement of investigators as early as possible in an international public health enhancement project improves student learning and ongoing commitment. Conclusion Service learning objectives aimed at providing valuable medical learning to student learners immersed in other cultures are consistent with evidence-based learning objectives in the field of public health. Proactively structuring this experience to explicate these goals can enhance student learning. This dual strategy may improve the sustainability of international health programs by educating medical students while leading them into careers where these skills will be leveraged.
Neurologists around the country and the world are rapidly transitioning from traditional in-person visits to remote neurologic care because of the corona virus disease 2019 pandemic. Given calls and mandates for social distancing, most clinics have shuttered or are only conducting urgent and emergent visits. As a result, many neurologists are turning to teleneurology with real-time remote video-based visits with patients, to provide ongoing care. Although telemedicine utilization and comfort has grown for many acute and ambulatory neurologic conditions in the past decade, remote visits and workflows remain foreign to many patients and neurologists. Here, we provide a practical framework for clinicians to orient themselves to the remote neurologic assessment, offering suggestions for clinician and patient preparation prior to the visit; recommendations to manage common challenges with remote neurologic care; modifications to the neurologic exam for remote performance, including subspecialty-specific considerations for a variety of neurologic conditions; and a discussion of the key limitations of remote visits. These recommendations are intended to serve as a guide for immediate implementation as neurologists transition to remote care. These will be relevant not only for practice today, but also for the likely sustained expansion of teleneurology following the pandemic.
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