Neuro-Ophthalmic Manifestations of Adult Polyglucosan Body Disease.

Background: Adult polyglucosan body disease (APBD) is caused by a deficiency in glycogen branching enzyme that leads to polyglucosan accumulation in multiple organs. It has a progressive clinical course with prominent neurologic manifestations. We aim to describe the neuro-ophthalmic manifestations of APBD.

MICK (Mobile Integrated Cognitive Kit) App for Concussion Assessment in a Youth Ice Hockey League.

Background: Visual symptoms are common after concussion. Rapid automatized naming (RAN) tasks are simple performance measures that demonstrate worse time scores in the setting of acute or more remote injury.

Methods: We evaluated the capacity for the Mobile Universal Lexicon Evaluation System (MULES) and Staggered Uneven Number (SUN) testing to be feasibly administered during preseason testing in a cohort of youth ice hockey athletes using a novel computerized app, the Mobile Integrated Cognitive Kit (MICK).

AI in Neuro-Ophthalmology: Current Practice and Future Opportunities.

Background: Neuro-ophthalmology frequently requires a complex and multi-faceted clinical assessment supported by sophisticated imaging techniques in order to assess disease status. The current approach to diagnosis requires substantial expertise and time. The emergence of AI has brought forth innovative solutions to streamline and enhance this diagnostic process, which is especially valuable given the shortage of neuro-ophthalmologists.

Testing the Validity and Reliability of a Standardized Virtual Examination for Concussion.

Background And Objectives: We determined inter-modality (in-person vs telemedicine examination) and inter-rater agreement for telemedicine assessments (2 different examiners) using the Telemedicine Buffalo Concussion Physical Examination (Tele-BCPE), a standardized concussion examination designed for remote use.

Methods: Patients referred for an initial evaluation for concussion were invited to participate. Participants had a brief initial assessment by the treating neurologist.

Navigating the U.S. regulatory landscape for neurologic digital health technologies.

Digital health technologies (DHTs) can transform neurological assessments, improving quality and continuity of care. In the United States, the Food & Drug Administration (FDA) oversees the safety and efficacy of these technologies, employing a detailed regulatory process that classifies devices based on risk and requires rigorous review and post-market surveillance. Following FDA approval, DHTs enter the Current Procedural Terminology, Relative Value Scale Update Committee, and Centers for Medicare & Medicaid Services coding and valuation processes leading to coverage and payment decisions.

Myelin Oligodendrocyte Glycoprotein Antibody Disease Optic Neuritis: A Structure-Function Paradox?

Background: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a demyelinating disorder that most commonly presents with optic neuritis (ON) and affects children more often than adults. We report 8 pediatric patients with MOG-associated ON and characterize focal optical coherence tomography (OCT) abnormalities over time that help distinguish this condition from the trajectories of other demyelinating disorders. These OCT findings are examined in the context of longitudinal visual function testing.

Relapsing White Matter Disease and Subclinical Optic Neuropathy: From the National Multiple Sclerosis Society Case Conference Proceedings.

A 16-year-old adolescent boy presented with recurrent episodes of weakness and numbness. Brain MRI demonstrated subcortical, juxtacortical, and periventricular white matter T2 hyperintensities with gadolinium enhancement. CSF was positive for oligoclonal bands that were not present in serum.

Precision Concussion Management: Approaches to Quantifying Head Injury Severity and Recovery.

Mitigating the substantial public health impact of concussion is a particularly difficult challenge. This is partly because concussion is a highly prevalent condition, and diagnosis is predominantly symptom-based. Much of contemporary concussion management relies on symptom interpretation and accurate reporting by patients.

Opsoclonus and ocular flutter: evaluation and management.

Purpose Of Review: Opsoclonus and ocular flutter are saccadic intrusions characterized by spontaneous, back-to-back, fast eye movements (saccades) that oscillate about the midline of central visual fixation without intervening inter-saccadic intervals. When this type of movement occurs exclusively in the horizontal plane, it is called ocular flutter. When it occurs in multiple planes (i.

Curriculum Innovations: A Comprehensive Teleneurology Curriculum for Neurology Trainees.

Introduction And Problem Statement: As the role of teleneurology expands, it is important to prepare trainees to perform virtual encounters proficiently.

Objectives: We created a comprehensive multimodality teleneurology curriculum for residents to teach key aspects of telehealth encounters including the virtual examination and skill development across several environments.

Methods And Curriculum Description: We developed and implemented a teleneurology curriculum focused on teaching the virtual neurologic examination, measuring teleneurology competency, and providing opportunities for trainees to perform telehealth encounters in multiple settings.

Neuro-Ophthalmologic Variability in Presentation of Genetically Confirmed Wolfram Syndrome: A Case Series and Review.

Wolfram syndrome is a neurodegenerative disorder caused by pathogenic variants in the genes or . Clinically, the classic phenotype is composed of optic atrophy, diabetes mellitus type 1, diabetes insipidus, and deafness. Wolfram syndrome, however, is phenotypically heterogenous with variable clinical manifestations and age of onset.

Occipital Nocardia Abscess Presenting With Positive Visual Phenomenon and Quadrantanopsia.

A 74-year-old man with chronic obstructive pulmonary disease, glaucoma, and Stage IIIB squamous cell lung cancer experienced several minutes of flashing lights in his right visual hemifield, followed by onset of a right visual field defect. On examination, the patient had a right homonymous hemianopsia that was most dense inferiorly by confrontation testing. Emergent CT scan of the head revealed a 2.

Vision as a piece of the head trauma puzzle.

Approximately half of the brain's circuits are involved in vision and control of eye movements. Therefore, visual dysfunction is a common symptom of concussion, the mildest form of traumatic brain injury (TBI). Photosensitivity, vergence dysfunction, saccadic abnormalities, and distortions in visual perception have been reported as vision-related symptoms following concussion.

Trends in concussion mechanism of injury during the COVID-19 pandemic.

Objective: The primary objective was to determine the effect of the COVID-19 pandemic on volume, demographics, and mechanisms of injury (MOI) for patients seen at an urban multidisciplinary concussion center. During the first phase of the pandemic in the United States, stay-at-home orders led to decreased group activities and required cancellation of outpatient appointments or initiation of telemedicine visits.

Methods: This study was a retrospective chart review of 3500 patient electronic medical records (EMR).

MICK (Mobile Integrated Cognitive Kit) app: Feasibility of an accessible tablet-based rapid picture and number naming task for concussion assessment in a division 1 college football cohort.

Although visual symptoms are common following concussion, quantitative measures of visual function are missing from concussion evaluation protocols on the athletic sideline. For the past half century, rapid automatized naming (RAN) tasks have demonstrated promise as quantitative neuro-visual assessment tools in the setting of head trauma and other disorders but have been previously limited in accessibility and scalability. The Mobile Interactive Cognitive Kit (MICK) App is a digital RAN test that can be downloaded on most mobile devices and can therefore provide a quantitative measure of visual function anywhere, including the athletic sideline.

Accuracy of clinical versus oculographic detection of pathological saccadic slowing.

Saccadic slowing as a component of supranuclear saccadic gaze palsy is an important diagnostic sign in multiple neurologic conditions, including degenerative, inflammatory, genetic, or ischemic lesions affecting brainstem structures responsible for saccadic generation. Little attention has been given to the accuracy with which clinicians correctly identify saccadic slowing. We compared clinician (n = 19) judgements of horizontal and vertical saccade speed on video recordings of saccades (from 9 patients with slow saccades, 3 healthy controls) to objective saccade peak velocity measurements from infrared oculographic recordings.

Training in Neurology: Objective Structured Clinical Examination Case to Teach and Model Feedback Skills in Neurology Residency.

We describe an educational intervention for neurology residents aimed at developing feedback skills. An objective structured clinical examination case was designed to simulate the provision of feedback to a medical student. After the simulated case session, residents received structured, individualized feedback on their performance and then participated in a group discussion about feedback methods.

The MICK (Mobile integrated cognitive kit) app: Digital rapid automatized naming for visual assessment across the spectrum of neurological disorders.

Objective: Rapid automatized naming (RAN) tasks have been utilized for decades to evaluate neurological conditions. Time scores for the Mobile Universal Lexicon Evaluation System (MULES, rapid picture naming) and Staggered Uneven Number (SUN, rapid number naming) are prolonged (worse) with concussion, mild cognitive impairment, multiple sclerosis and Parkinson's disease. The purpose of this investigation was to compare paper/pencil versions of MULES and SUN with a new digitized format, the MICK app.

Proceedings of the 2020 Epilepsy Foundation Pipeline Conference: Emerging Drugs and Devices.

From August 27-28, 2020 the Epilepsy Foundation hosted the Pipeline Conference, exploring emerging issues related to antiepileptic drug and device development. The conference featured epilepsy therapeutic companies and academic laboratories developing drugs for focal epilepsies, innovations for rare and ultra-rare diseases, and devices both in clinical trials and approved for use. In this paper, we outline the virtual presentations by the authors, including novel data from their development pipeline.