Neurology. Clinical Practice

BACKGROUND AND OBJECTIVES: Visual auras in migraine have been extensively studied, but less is known about unusual experiences in other sensory domains, including whether they should be diagnostically considered as part of aura symptoms. This study aimed to examine the prevalence of multisensory aura experiences in migraine and their phenomenologic and clinical correlates. METHODS: Respondents were 729 participants with probable migraine, who completed an online study examining unusual sensory experiences...

OBJECTIVES: We report a case of Kelch-like protein-11 (KLHL11) encephalitis associated with squamous cell carcinoma (SCC) of unknown origin in the neck. METHODS: A 70-year-old man, diagnosed with prurigo nodularis 4 months prior, presented with subacute-onset progressive dysarthria, imbalance, and diplopia. Neurologic examination revealed horizontal and downbeating nystagmus with lateral gaze, dysarthria, dysmetria, and hearing difficulties. Skin examination showed localized pruritus with excoriated ulcerations on the neck and proximal upper extremities...

OBJECTIVES: Parakinesia brachialis oscitans (PBO) is the involuntary movement of an otherwise paretic upper limb triggered by yawning. We describe the first case of PBO in a patient with a first manifestation of tumefactive multiple sclerosis (MS). METHODS: A 35-year-old man presented to the emergency department with a first episode of generalized seizure. Neurologic examination revealed left-sided spastic hemiparesis, predominantly affecting his upper limb. Brain MRI showed a tumefactive right hemisphere lesion consistent with demyelination...

BACKGROUND AND OBJECTIVES: Accurate and reliable seizure data are essential for evaluating treatment strategies and tracking the quality of care in epilepsy clinics. This quality improvement project aimed to increase seizure documentation (i.e., documentation of seizure frequency from 80% to 100%, date of last seizure from 35% to 50%, and International League Against Epilepsy (ILAE) seizure classification from 35% to at least 50%) over 6 months. METHODS: We surveyed 7 epileptologists to determine their perceived seizure frequency, ILAE classification, and date of last seizure documentation habits...

BACKGROUND AND OBJECTIVES: No effective cure is available for neurogenetic diseases such as Huntington disease, spinocerebellar ataxias, and Friedreich ataxia, all of which cause progressive motor, cognitive, and psychiatric symptoms leading, in the long term, to severe communication (among other) impairments. In end-of-life situations, advanced directives (indications formulated by the patient about end-of-life choices) are one decision-making resource for relatives, caregivers, and health care professionals...

PURPOSE OF REVIEW: An estimated 6.5 million Americans live with Alzheimer dementia. Better understanding of advanced stages of Alzheimer disease (AD) and timely monitoring of its preventable complications would translate to improved survival and quality of life in this population. RECENT FINDINGS: In this perspective review, we proposed shifting the narrative to recognizing AD as a chronic life-limiting illness instead of a terminal disease. In support of this view, we discussed the following: (1) the biochemical, cellular (preclinical), and clinical phases of AD; (2) survival after AD; (3) AD therapeutics and potential implications for the population with AD in the advanced stages...

BACKGROUND AND OBJECTIVES: Recognition of stroke/TIA symptoms by emergency medical services (EMS) is instrumental in providing timely recanalization treatments. We assessed the recognition of stroke/TIA by EMS via the emergency medical call center (EMCC) dispatchers and out-of-hours health service (OOHS) dispatchers. METHODS: In a registry study, based on 2015-2020 data from the Copenhagen EMS, we calculated sensitivity, positive predictive value (PPV), specificity, and negative predictive value (NPV) of dispatcher suspicion of stroke or transient ischemic attack (TIA) and compared against discharge diagnosis...

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BACKGROUND AND OBJECTIVES: People with Parkinson disease (PWP) and their care partners have high palliative care needs resulting from disabling motor and nonmotor symptoms. There is growing support for palliative care (PC) approaches to Parkinson disease. However, little is known regarding the extent to which the palliative needs of PWP and care partners are currently being met. This study's primary objective is to describe PWP's and care partners' perceptions of the extent to which their PC needs are being met...

In collaboration with the American Academy of Pediatrics, Child Neurology Society, and Society for Critical Care Medicine, the American Academy of Neurology formulated an updated, evidence-informed consensus-based guideline for pediatric and adult brain death/death by neurologic criteria (BD/DNC) determination. In comparison with the prior guidelines, the revisions and additions in this guideline, which are summarized in this review, are intended to (1) ensure recommendations are conservative, yet practical, and emphasize circumstances in which BD/DNC determination should be delayed or deferred, so as to minimize the risk of a false-positive BD/DNC determination; and (2) provide guidance about aspects of BD/DNC determination that clinicians find challenging and/or controversial...

BACKGROUND AND OBJECTIVES: Evaluation of transient ischemic attack/nondisabling ischemic strokes (TIA/NDS) in the emergency department (ED) contributes to capacity issues and increasing health care expenditures, especially high-cost duplicative imaging. METHODS: As an institutional quality improvement project, we developed a novel pathway to evaluate patients with TIA/NDS in the ED using a core set of laboratory tests and CT-based neuroimaging. Patients identified as 'low risk' through a safety checklist were discharged and scheduled for prompt outpatient tests and stroke clinic follow-up...

BACKGROUND AND OBJECTIVES: Social and structural determinants of health (SDOH) have been associated with disability in neurologic diseases. However, the association between these factors and disability in Huntington disease (HD) has not been studied. This study aimed to evaluate the association of racial and sociodemographic factors with disease severity in patients with HD in North America. METHODS: We conducted a cross-sectional study of genetically confirmed participants with HD (36+ CAG repeats) in the North American region using the ENROLL-HD 2020 periodic dataset...

PURPOSE OF REVIEW: The objective of this study was to explore the clinical spectrum of movement disorders and associated neurologic findings in hypomagnesemia and challenges in diagnosis and treatment. RECENT FINDINGS: Sixty patients were identified in the literature for analysis. Movement disorders observed were postural tremor (23.3%, n = 14), resting tremor (8.3%, n = 5), intention tremor (10%, n = 6), ataxia involving the trunk (48.3%, n = 29) or limbs (25%, n = 15) and dysarthria (21...

BACKGROUND AND OBJECTIVES: Psychogenic nonepileptic seizures (PNES) are paroxysmal events that resemble epileptic seizures but have psychological underpinnings. Patients with PNES have high health care utilization. We hypothesize that appropriate care would lead to decreases in utilization. The aim of this study was to measure the impact of a multidisciplinary PNES clinic in reducing health care utilization (HCU) in youth with PNES referred to the clinic. METHODS: We reviewed the frequency of visits to the emergency department (ED), urgent care (UC), planned and unplanned hospitalizations, neurodiagnostic studies, and total charges associated with these encounters 12 months before and 12 months after initial referral to the clinic...

BACKGROUND AND OBJECTIVES: Global developmental delay/intellectual disability (GDD/ID) are among the most common neurologic conditions evaluated by child neurologists in the United States. No recent neurology-specific guidelines for GDD/ID diagnostic evaluation exist, which could lead to practice variability. We assessed current practices in GDD/ID diagnostic evaluation among US child neurologists, including drivers of exome sequencing (ES). METHODS: A 19-item online anonymous survey was distributed between April 2021 and September 2021 to 953 eligible child neurologists by email and/or online platforms through the American Academy of Neurology and Child Neurology Society...

OBJECTIVES: We describe an atypical delayed neurologic recovery from coma and unresponsive wakefulness syndrome (i.e., persistent vegetative state) in a patient with severe drug-induced toxic leukoencephalopathy (presumably due to synthetic cannabinoid intake). METHODS: The patient underwent standardized behavioral and multimodal neuroimaging assessments to monitor clinical evolution and brain function over a 5-month period after presumed intoxication. RESULTS: A progressive clinical recovery was observed, from an initial state of coma to emergence from a minimally conscious state after 2 months...

OBJECTIVES: The objective of this study was to complete a scoping review of current literature surrounding health inequities in epilepsy while providing recommendations for future research. METHODS: During July 2022, we searched MEDLINE and Ovid Embase to find published articles pertaining to epilepsy and health inequities. Initially, authors received training. Authors then screened, and data were extracted in a masked duplicate manner. Studies published within the time frame of 2011-2021 in all countries were deemed appropriate...

BACKGROUND AND OBJECTIVES: Impulse control disorders (ICD) are a group of behaviors in Parkinson disease (PD), (compulsive buying, gambling, binge eating, craving sweets, and hypersexuality) that occur in up to 20% of individuals with PD, sometimes with devastating results. We sought to determine the rate of ICD screening based on 2020 quality measures for PD care by the American Academy of Neurology. METHODS: We conducted a quality improvement project to document and improve physician ICD screening in a tertiary movement disorder program...

BACKGROUND AND OBJECTIVES: Dystonia in cerebral palsy (CP) is debilitating and common, but underdiagnosed, especially when coexistent with spasticity. With dedicated research-based assessment, dystonia is found in most people with spastic CP but is only clinically diagnosed in the minority. To begin addressing the high rates of dystonia underdiagnosis in this population, we determined the key feature experts use to assess upper extremity dystonia in people with spastic CP. METHODS: In this prospective cohort study, 3 pediatric movement disorder specialists assessed upper extremity dystonia in neurologic examination videos of people with spastic CP and isolated periventricular leukomalacia (PVL) on brain MRI (i...

BACKGROUND AND OBJECTIVES: Diffusion-restricted (DR) lesions of the splenium are encountered in a wide variety of pathologies, and their significance is often unclear. We sought to report the spectrum of clinical presentations, neuroimaging patterns, and the predictors of radiographic and clinical outcomes from DR splenial lesions. METHODS: This was a single-center, retrospective cohort study from January 1, 2009, to August 1, 2020. A consecutive sample of 3,490 individuals who underwent brain MRI with reported corpus callosum lesions during the study period were evaluated for DR lesions in the corpus callosum...