Faculty Bibliography

Purpose of Review: This article focuses on a review of the non-surgical treatment options for patients with phantom limb pain (PLP). Recent Findings: Based on a review of the published literature over the past 5 years, the most promising evidenced-based therapies involve sensory feedback to the user through either visual or tactile stimulation. Summary: Of these, the most effective therapies include mirror therapy, phantom motor imagery, and phantom motor execution and, therefore, should be considered when treating individuals with PLP.

The transpalpebral "eyelid" approach is an innovative alternative to the traditional incisions for exposure of the anterior cranial fossa for neurosurgery. Yet, there is a paucity of data on such a surgical technique in the plastic surgery literature for accessing the anterior cranial fossa. A retrospective review was performed of patients who underwent supraorbital frontal craniotomy using an anterior skull base approach with transpalpebral exposure over eight years by a single plastic surgeon (D.A.S.). Surgical techniques, medical co-morbidities, intra-operative complications, and long-term complications were assessed. Twenty patients (mean age 52±12 years, 55% male, 45% female) underwent supraorbital frontal craniotomy using an anterior skull base approach with upper transpalpebral exposure. Operative indications included: 75% had anterior communicating aneurysms with a mean aneurysm size of 5.36±1.91 mm, 10% had meningiomas, 10% had dural fistulas, and 5% had an orbital hemangioma. Notably, 60% had a smoking history. No intra-operative complications were encountered, and no cases required conversion to a traditional open approach. Mean length of hospital stay was 3.2±1.5 days. Post-operative imaging revealed no residual or recurrent pathology. Mean follow up time was 62.2±30.6 months. No long-term neurological or ophthalmologic complications or infections occurred. No forehead paresthesias, and no brow ptosis or brow paralysis were noted. The transpalpebral technique is an excellent, minimally invasive alternative to approach lesions of the anterior cranial fossa. Successful application may require appropriate management of the frontal sinus and supraorbital nerve. As described, this approach does not limit neurosurgical access or results, and led to no neurosurgical complications.

The WHO argues that a pharmacy-first approach should no longer be the reflexive treatment for mental health diagnoses. Heart rate variability biofeedback (HRVB) demonstrably treats various conditions"”especially effective at regulating emotion, particularly managing and alleviating anger, stress, anxiety, and depression, common co-morbid diagnoses for rehabilitation medicine patients. HRVB trains users to study their biofeedback data in real time, alter bodily functions previously believed to be automatic, and garner health benefits. Despite convenience, relatively low cost, and empowering patients to manage their own symptoms, the current lack of reimbursability, and the lack of Phase III RCTs limit HRVB application. Ideally, the confidence of practitioners, patients, and insurers would follow the known efficacy of HRVB for the treatment of mental health conditions.

PURPOSE/OBJECTIVE:A dysfunction of beta oscillatory activity is the neurophysiological hallmark of Parkinson disease (PD). How cortical activity reacts to external perturbations may provide insight into pathophysiological mechanisms. This study aims at identifying modifications in EEG rhythms after transcranial magnetic stimulation (TMS) in PD. We hypothesize that single-pulse TMS can modulate brain intrinsic oscillatory properties (e.g., beta excess). METHODS:EEG data were coregistered during single-pulse TMS (100 stimuli over the primary motor cortex [M1, hotspot for Abductor Pollicis Brevis], random intertrial interval from 8 to 13 seconds). We used a time-frequency analysis based on wavelet method to characterize modification of oscillatory rhythms (delta [1-4 Hz], theta [4-7 Hz], alpha [8-12 Hz], and beta [13-30 Hz] in 15 participants with PD compared with 10 healthy controls. RESULTS:An increase in beta power over the sensorimotor areas was recorded at rest in the PD group (P < 0.05). Brain oscillations in PD transiently reset after TMS: beta power over M1 becomes comparable to that recorded in aged-matched healthy subjects in the 2 seconds following TMS. CONCLUSIONS:Transcranial magnetic stimulation over the dominant motor cortex transiently normalizes cortical oscillations. More user-friendly noninvasive brain stimulation needs to be trialed, based on this proof of concept, to provide practical, portable techniques to treat motor symptoms in PD.

The discriminability of motion direction is asymmetric, with some motion directions that are better discriminated than others. For example, discrimination of directions near the cardinal axes (upward/downward/leftward/rightward) tends to be better than oblique directions. Here, we tested discriminability for multiple motion directions at multiple polar angle locations. We found three systematic asymmetries. First, we found a large cardinal advantage in a cartesian reference frame - better discriminability for motion near cardinal reference directions than oblique directions. Second, we found a moderate cardinal advantage in a polar reference frame - better discriminability for motion near radial (inward/outward) and tangential (clockwise/counterclockwise) reference directions than other directions. Third, we found a small advantage for discriminating motion near radial compared to tangential reference directions. The three advantages combine in an approximately linear manner, and together predict variation in motion discrimination as a function of both motion direction and location around the visual field. For example, best performance is found for radial motion on the horizontal and vertical meridians, as these directions encompass all three advantages, whereas poorest performance is found for oblique motion stimuli located on the horizontal and vertical meridians, as these directions encompass all three disadvantages. Our results constrain models of motion perception and suggest that reference frames at multiple stages of the visual processing hierarchy limit performance.

Central metabolism has a profound impact on the clinical phenotypes and penetrance of neurological diseases such as Alzheimer"™s (AD) and Parkinson"™s (PD) diseases, Amyotrophic Lateral Sclerosis (ALS) and Autism Spectrum Disorder (ASD). In contrast to the multifactorial origin of these neurological diseases, neurodevelopmental impairment and neurodegeneration in Familial Dysautonomia (FD) results from a single point mutation in the ELP1 gene. FD patients represent a well-defined population who can help us better understand the cellular networks underlying neurodegeneration, and how disease traits are affected by metabolic dysfunction, which in turn may contribute to dysregulation of the gut"“brain axis of FD. Here, ¹H NMR spectroscopy was employed to characterize the serum and fecal metabolomes of FD patients, and to assess similarities and differences in the polar metabolite profiles between FD patients and healthy relative controls. Findings from this work revealed noteworthy metabolic alterations reflected in energy (ATP) production, mitochondrial function, amino acid and nucleotide catabolism, neurosignaling molecules, and gut-microbial metabolism. These results provide further evidence for a close interconnection between metabolism, neurodegeneration, and gut microbiome dysbiosis in FD, and create an opportunity to explore whether metabolic interventions targeting the gut"“brain"“metabolism axis of FD could be used to redress or slow down the progressive neurodegeneration observed in FD patients.

OBJECTIVE:and to assess the ability of the IC-CoDE to produce definable and stable cognitive phenotypes in a large, multi-center temporal lobe epilepsy (TLE) patient sample. METHOD/METHODS:were derived across samples using the IC-CoDE and compared to distributions of phenotypes reported in existing studies. RESULTS:Impairment rates were highest on tests of language, followed by memory, executive functioning, attention/processing speed, and visuospatial ability. Application of the IC-CoDE using varying operational definitions of impairment (≤ 1.0 and ≤ 1.5 SD) produced cognitive phenotypes with the following distribution: cognitively intact (30%-50%), single-domain (26%-29%), bi-domain (14%-19%), and generalized (10%-22%) impairment. Application of the ≤ 1.5 cutoff produced a distribution of phenotypes that was consistent across cohorts and approximated the distribution produced using data-driven approaches in prior studies. CONCLUSIONS:The IC-CoDE is the first iteration of a classification system for harmonizing cognitive diagnostics in epilepsy research that can be applied across neuropsychological tests and TLE cohorts. This proof-of-principle study in TLE offers a promising path for enhancing research collaborations globally and accelerating scientific discoveries in epilepsy. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a recently described CNS inflammatory disorder that may manifest with optic neuritis, myelitis, seizures, and/or acute disseminated encephalomyelitis. While MOG-specific antibodies in patients with MOGAD are IgG1, a T-cell-dependent antibody isotype, immunologic mechanisms of this disease are not fully understood. Thymic hyperplasia can be associated with certain autoimmune diseases. In this report we describe a case of MOGAD associated with thymic hyperplasia in a young adult.

Simulation is an engaging modality of medical education that leverages adult learning theory. Since its inception, educators have used simulation to train clinicians in bedside procedures and neurologic emergencies, as well as in communication, teamwork, and leadership skills. Many applications of simulation in neurology are yet to be fully adopted or explored. However, challenges to traditional educational paradigms, such as the shift to competency-based assessments and the need for remote or hybrid platforms, have created an impetus for neurologists to embrace simulation. In this article, we explore how simulation might be adapted to meet these current challenges in neurologic education by reviewing the existing literature in simulation from the field of neurology and beyond. We discuss how simulation can engage neurology trainees who seek interactive, contextualized, on-demand education. We consider how educators can incorporate simulation for competency-based evaluations and procedural training. We foresee a growing role of simulation initiatives that assess bias and promote equity. We also provide tangible solutions that make simulation an educational tool that is within reach for any educator in both high-resource and low-resource settings.

OBJECTIVE:This study was undertaken to evaluate the cross-cultural application of the International Classification of Cognitive Disorders in Epilepsy (IC-CoDE) to a cohort of Spanish-speaking patients with temporal lobe epilepsy (TLE) living in the United States. METHODS:Eighty-four Spanish-speaking patients with TLE completed neuropsychological measures of memory, language, executive function, visuospatial functioning, and attention/processing speed as part of the Neuropsychological Screening Battery for Hispanics. The contribution of demographic and clinical variables to cognitive performance was evaluated. A sensitivity analysis was conducted by examining the base rates of impairment across several impairment thresholds. The IC-CoDE taxonomy was then applied, and the base rate of cognitive phenotypes for each cutoff was calculated. The distribution of phenotypes was compared to the published IC-CoDE taxonomy data, which utilized a large, multicenter cohort of English-speaking patients with TLE. RESULTS:Across the different impairment cutoffs, memory was the most impaired cognitive domain, with impairments in list learning ranging from 50% to 78%. Application of the IC-CoDE taxonomy utilizing a -1.5-SD cutoff revealed an intact cognitive profile in 47.6% of patients, single-domain impairment in 23.8% of patients, bidomain impairment in 14.3% of patients, and generalized impairment in 14.3% of the sample. This distribution was comparable to the phenotype distribution observed in the IC-CoDE validation sample. SIGNIFICANCE/CONCLUSIONS:We demonstrate a similar pattern and distribution of cognitive phenotypes in a Spanish-speaking epilepsy cohort compared to an English-speaking sample. This suggests stability in the underlying phenotypes associated with TLE and applicability of the IC-CoDE for guiding cognitive diagnostics in epilepsy research that can be applied to culturally and linguistically diverse samples.