Faculty Bibliography

BACKGROUND:Visual tests in Alzheimer disease (AD) have been examined over the last several decades to identify a sensitive and noninvasive marker of the disease. Rapid automatized naming (RAN) tasks have shown promise for detecting prodromal AD or mild cognitive impairment (MCI). The purpose of this investigation was to determine the capacity for new rapid image and number naming tests and other measures of visual pathway structure and function to distinguish individuals with MCI due to AD from those with normal aging and cognition. The relation of these tests to vision-specific quality of life scores was also examined in this pilot study. METHODS:Participants with MCI due to AD and controls from well-characterized NYU research and clinical cohorts performed high and low-contrast letter acuity (LCLA) testing, as well as RAN using the Mobile Universal Lexicon Evaluation System (MULES) and Staggered Uneven Number test, and vision-specific quality of life scales, including the 25-Item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25) and 10-Item Neuro-Ophthalmic Supplement. Individuals also underwent optical coherence tomography scans to assess peripapillary retinal nerve fiber layer and ganglion cell/inner plexiform layer thicknesses. Hippocampal atrophy on brain MRI was also determined from the participants' Alzheimer disease research center or clinical data. RESULTS:Participants with MCI (n = 14) had worse binocular LCLA at 1.25% contrast compared with controls (P = 0.009) and longer (worse) MULES test times (P = 0.006) with more errors in naming images (P = 0.009) compared with controls (n = 16). These were the only significantly different visual tests between groups. MULES test times (area under the receiver operating characteristic curve [AUC] = 0.79), MULES errors (AUC = 0.78), and binocular 1.25% LCLA (AUC = 0.78) showed good diagnostic accuracy for distinguishing MCI from controls. A combination of the MULES score and 1.25% LCLA demonstrated the greatest capacity to distinguish (AUC = 0.87). These visual measures were better predictors of MCI vs control status than the presence of hippocampal atrophy on brain MRI in this cohort. A greater number of MULES test errors (rs = -0.50, P = 0.005) and worse 1.25% LCLA scores (rs = 0.39, P = 0.03) were associated with lower (worse) NEI-VFQ-25 scores. CONCLUSIONS:Rapid image naming (MULES) and LCLA are able to distinguish MCI due to AD from normal aging and reflect vision-specific quality of life. Larger studies will determine how these easily administered tests may identify patients at risk for AD and serve as measures in disease-modifying therapy clinical trials.

OBJECTIVE:Temporal lobe encephaloceles (TLENs) are a significant cause of medically refractory epilepsy, but there is little consensus regarding their workup and treatment. This study characterizes these lesions and their role in seizures and aims to standardize preoperative evaluation and surgical management. METHODS:Patients with TLEN who had undergone resective epilepsy surgery from December 2015 to August 2020 at a single institution were included in the study. Medical records were reviewed for each patient to collect relevant seizure workup information including demographics, radiological findings, surgical data, and neuropsychological evaluation. RESULTS:For patients who presented to the authors' program with suspected medically intractable temporal lobe epilepsy (219 patients), TLEN was considered to be the epileptogenic focus in 5.5%. Ten patients with TLEN had undergone resection and were included in this study. Concordance between ictal scalp electroencephalography (EEG) lateralization and TLEN was found in 9/10 patients (90%), and 4/10 patients (40%) had signs suggestive of idiopathic intracranial hypertension (IIH). Surgical outcome was reported in patients with at least 12 months of follow-up (9/10). Patients with scalp EEG findings concordant with the TLEN side had a good outcome (Engel class I: 7 patients, class II: 1 patient). One patient with discordant EEG findings had a bad outcome (Engel class III). No significant neuropsychological deficits were observed after the surgery. CONCLUSIONS:TLENs are epileptogenic lesions that should be screened for in patients with medically refractory epilepsy who have signs of IIH and no other lesions on MRI. Restricted resection is safe and effective in patients with scalp EEG findings concordant with TLEN.

Importance/UNASSIGNED:In the Platelet-Oriented Inhibition in New TIA and Minor Ischemic Stroke (POINT) trial, acute treatment with clopidogrel-aspirin was associated with significantly reduced risk of recurrent stroke. There may be specific patient groups who are more likely to benefit from this treatment. Objective/UNASSIGNED:To investigate whether the association of clopidogrel-aspirin with stroke recurrence in patients with minor stroke or high-risk transient ischemic attack (TIA) is modified by the presence of infarct on imaging attributed to the index event (index imaging) among patients enrolled in the POINT Trial. Design, Setting, and Participants/UNASSIGNED:In the POINT randomized clinical trial, patients with high-risk TIA and minor ischemic stroke were enrolled at 269 sites in 10 countries in North America, Europe, Australia, and New Zealand from May 28, 2010, to December 19, 2017. In this post hoc analysis, patients were divided into 2 groups according to whether they had an acute infarct on index imaging. All POINT trial participants with information available on the presence or absence of acute infarct on index imaging were eligible for this study. Univariable Cox regression models evaluated associations between the presence of an infarct on index imaging and subsequent ischemic stroke and evaluated whether the presence of infarct on index imaging modified the association of clopidogrel-aspirin with subsequent ischemic stroke risk. Data were analyzed from July 2020 to May 2021. Exposures/UNASSIGNED:Presence or absence of acute infarct on index imaging. Main Outcomes and Measures/UNASSIGNED:The primary outcome is whether the presence of infarct on index imaging modified the association of clopidogrel-aspirin with subsequent ischemic stroke risk. Results/UNASSIGNED:Of the 4881 patients enrolled in POINT, 4876 (99.9%) met the inclusion criteria (mean [SD] age, 65 [13] years; 2685 men [55.0%]). A total of 1793 patients (36.8%) had an acute infarct on index imaging. Infarct on index imaging was associated with ischemic stroke during follow-up (hazard ratio [HR], 3.68; 95% CI, 2.73-4.95; P < .001). Clopidogrel-aspirin vs aspirin alone was associated with decreased ischemic stroke risk in patients with an infarct on index imaging (HR, 0.56; 95% CI, 0.41-0.77; P < .001) compared with those without an infarct on index imaging (HR, 1.11; 95% CI, 0.74-1.65; P = .62), with a significant interaction association (P for interaction = .008). Conclusions and Relevance/UNASSIGNED:In this study, the presence of an acute infarct on index imaging was associated with increased risk of recurrent stroke and a more pronounced benefit from clopidogrel-aspirin. Future work should focus on validating these findings before targeting specific patient populations for acute clopidogrel-aspirin treatment.

ABSTRACT/UNASSIGNED:A 78-year-old man was evaluated for altered mentation in the setting of significant uremia. On examination, he was found to be encephalopathic with generalized myoclonus and spontaneous opsoclonus. He had no known risk factors for the development of opsoclonus and upon undergoing hemodialysis, experienced near resolution of his eye movement abnormalities, thus highlighting a possible link between the uremic state and opsoclonus.

To derive meaning from sound, the brain must integrate information across many timescales. What computations underlie multiscale integration in human auditory cortex? Evidence suggests that auditory cortex analyses sound using both generic acoustic representations (for example, spectrotemporal modulation tuning) and category-specific computations, but the timescales over which these putatively distinct computations integrate remain unclear. To answer this question, we developed a general method to estimate sensory integration windows-the time window when stimuli alter the neural response-and applied our method to intracranial recordings from neurosurgical patients. We show that human auditory cortex integrates hierarchically across diverse timescales spanning from ~50 to 400 ms. Moreover, we find that neural populations with short and long integration windows exhibit distinct functional properties: short-integration electrodes (less than ~200 ms) show prominent spectrotemporal modulation selectivity, while long-integration electrodes (greater than ~200 ms) show prominent category selectivity. These findings reveal how multiscale integration organizes auditory computation in the human brain.

Background: Sturge-Weber syndrome (SWS) involves the skin, brain, and eyes. The dermatologic manifestation is the facial port wine birthmark (PWB), while there are several potential neurologic and ophthalmologic manifestations, notably epilepsy and glaucoma, respectively. This consensus aims to develop evidence-based expert-defined recommendations for management of the neurologic and ophthalmic features and provides a framework for dermatologists to determine workup for patients with PWB who seek laser treatment. Study Design/Materials and Method: Thirteen national experts in neurology, radiology, and ophthalmology were assembled as part of a larger consensus statement for the management of SWS. Key topics and questions regarding risk stratification, referral indications, and treatment were formulated. A systematic PubMed search was performed. Evidence-based recommendations were developed.
Result(s): High-risk PWB distributions involve the hemifacial, forehead, or median locations, including the upper eyelids, especially when concerned for glaucoma. Any child with a high-risk facial PWB should be referred to a pediatric neurologist and pediatric ophthalmologist for a baseline evaluation, with periodic follow-up. Routine screening for brain involvement is not recommended for newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms but can be performed in cases of extreme parental anxiety, an abnormal EEG, or when presymptomatic treatment is contemplated such as with extensive bilateral PWB. In children with stable neurocognitive symptoms, routine follow-up neuroimaging is not advised. In adults with a high-risk PWB and no prior imaging, neuroimaging should be obtained, but follow-up neuroimaging is not recommended in adults with established SWS and stable neurocognitive symptoms. The treatment of glaucoma varies depending on the patient's age and clinical presentation.
Conclusion(s): Recommendations were developed by experts in neurology, neuroradiology, and ophthalmology. These guidelines can guide evidence-based discussions between patients and providers and increase dermatologists' awareness regarding when and what workup is necessary in patients seeking laser treatment for PWB

Purpose/UNASSIGNED:To report 3 otherwise healthy patients with Herpes zoster reactivation shortly after administration of a mRNA vaccine against the novel COVID-19 virus. Observations/UNASSIGNED:Patient 1 is a 54 year old who presented with Herpes zoster meningitis complicated by enhancing nodular leptomeningeal lesions of the spinal cord. The subsequent two patients had Herpes zoster ophthalmicus of the cornea (Case 2) and eyelid (Case 3). All three presented within 2 weeks of receiving the Pfizer/BioNTech COVID-19 vaccine. Conclusions/UNASSIGNED:Herpes zoster may be a side effect of m RNA vaccination against the Sars-CoV2 vaccine and requires further investigation.

The International League Against Epilepsy (ILAE) seizure classification scheme has been periodically updated to improve its reliability and applicability to clinicians and researchers alike. Here, members of the Epilepsy Study Consortium propose a pragmatic seizure classification, based on the ILAE scheme, designed for use in clinical trials with a focus on outcome measures that have high reliability, broad interpretability across stakeholders, and clinical relevance in the context of the development of novel antiseizure medications. Controversies around the current ILAE classification scheme are discussed in the context of clinical trials, and pragmatic simplifications to the existing scheme are proposed, for intended use by investigators, industry sponsors, and regulatory agencies.