Neurology Commons^™

Thalamic Deep Brain Stimulation Improves Movement In A Cerebellar Model Of Lesion-Based Status Dystonicus, Megan X Nguyen, Amanda M Brown, Tao Lin, Roy V Sillitoe, Jason S Gill

Faculty, Staff and Students Publications

Dystonia is the third most common movement disorder and an incapacitating co-morbidity in a variety of neurologic conditions. Dystonia can be caused by genetic, degenerative, idiopathic, and acquired etiologies, which are hypothesized to converge on a "dystonia network" consisting of the basal ganglia, thalamus, cerebellum, and cerebral cortex. In acquired dystonia, focal lesions to subcortical network regions lead to dystonia that can be difficult to manage with canonical treatments, including deep brain stimulation (DBS). While studies in animal models have begun to parse the contribution of individual nodes in the dystonia network, how acquired injury to the cerebellar outflow tracts …

Use Of Robust Norming To Create A Sensitive Cognitive Summary Score In De Novo Parkinson's Disease: An Illustrative Example, Daniel Weintraub, Michael C Brumm, Ryan Kurth, Michele K York

Faculty, Staff and Students Publications

Background: Cognitive impairment is common at all stages of Parkinson's disease (PD), but there is no consensus on which neuropsychological tests to use or how to interpret cognitive battery results. A cognitive summary score (CSS) combines the richness of a neuropsychological battery with the simplicity of a single score.

Objective: The objective of this study was to determine whether a CSS created using robust norming can detect early cognitive deficits in de novo, untreated PD.

Methods: Baseline cognitive data from PD participants and healthy control participants (HCs) in the Parkinson's Progression Markers Initiative were used to (1) create a robust …

Open Versus Minimally Invasive Spine Surgery Remains A Global Question - A Commentary On "Open Versus Minimally Invasive Spine Surgery In The Treatment Of Single-Level Degenerative Lumbar Spondylolisthesis: An Ao Spine Global Cross-Sectional Study", Alexander E Ropper

Faculty, Staff and Students Publications

No abstract provided.

Visible Light Polarization-Sensitive Optical Coherence Tomography With Balanced Detection, Bahar Baradaran, Adam J Black, Sarah R Heilbronner, Taner Akkin

Faculty, Staff and Students Publications

Significance: We introduce a visible-light polarization-sensitive optical coherence tomography (PS-OCT) system that operates in the spectral domain with balanced detection (BD) capability. While the BD improves the signal-to-noise ratio (SNR), the use of shorter wavelengths improves spatial resolution and birefringence sensitivity.

Aim: We aim to implement a new optical design, characterize its performance, and investigate the imaging potential for biological tissues.

Approach: The design utilizes a unique interferometer and a custom spectrometer that captures four highly aligned spectra with a single area/multi-line camera. Each pair of spectral lines is highly aligned, and their subtraction yields balanced detected spectra of the …

Multi-Omics Delineate Growth Factor Network Underlying Exercise Effects In An Alzheimer’S Mouse Model, Xin Li, Chaozhong Liu, Wenbo Li, Guantong Qi, Yanwan Dai, Chaohao Gu, Yuxiang Sun, Wenjun Zhou, Veronica C Ciliberto, Jing Liang, Udhaya Kumar S, Dongyin Guan, Zhaoyong Hu, Hui Zheng, Zhandong Liu, Hu Chen, Ying-Wooi Wan, Zheng Sun

Duncan NRI Faculty and Staff Publications

Introduction: Physical exercise is a primary defense against age-related cognitive decline and Alzheimer's disease (AD).

Methods: We conducted single-nucleus transcriptomic and chromatin accessibility analyses (snRNA-seq and snATAC-seq) on the hippocampus of mice carrying mutations in the amyloid precursor protein gene (APPNL-G-F) following prolonged voluntary wheel-running exercise.

Results: Exercise mitigates amyloid-induced changes in transcriptome and chromatin accessibility through cell type-specific regulatory networks converging on growth factor signaling, particularly the epidermal growth factor receptor (EGFR) signaling. The beneficial effects of exercise on neurocognition can be blocked by pharmacological inhibition of EGFR and its downstream PI3K signaling. Exercise leads to elevated levels of …

Dementia In A Resource-Constrained Sub-Saharan African Setting: A Comprehensive Retrospective Analysis Of Prevalence, Risk Factors, And Management At The Only Neuropsychiatric Facility In Northeastern Nigeria, Ibrahim Abdu Wakawa, Umar Baba Musami, Suleiman Hamidu Kwairanga, Placidus Nwankuba Ogualili, Mohammed Yusuf Mahmood, Muhammad Abba Fugu, Mohammed Mala Gimba, Muktar Mohammed Allamin, Zaharadeen Umar Abbas, Chinedu Momoh

Brain and Mind Institute

INTRODUCTION: Dementia prevalence is increasing in sub-Saharan Africa, poten-tially due to population growth and aging. Resource-constrained settings such asNortheastern Nigeria face challenges in dementia management.

METHODS: We assessed dementia burden and management at the Federal Neuropsy-chiatric Hospital Maiduguri, the only neuropsychiatric facility in Northeastern Nigeria.This retrospective analysis included patient records from 1999 to 2023 for individuals60 year of age and older with a dementia diagnosis.

RESULTS: Of the 1216 cases reported, Alzheimer’s disease (60.5%) was the mostcommon subtype, followed by vascular dementia (24.5%). Hypertension (41.6%) wasthe most frequent comorbidity. Memory loss was present in all cases, whereasbehavioral …

Pre-Hospital And Post-Hospital Quality Of Care In Traumatic Spinal Column And Cord Injuries In Iran, Arman Zeinaddini-Meymand, Vali Baigi, Mohammad Mehdi Mousavi-Nasab, Sina Shool, Mohsen Sadeghi-Naini, Zahra Azadmanjir, Seyed Behnam Jazayeri, Samuel Berchi Kankam, Mohammad Dashtkoohi, Aidin Shakeri, Esmail Fakharian, Leila Kouchakinejad-Eramsadati, Habibollah Pirnejad, Homayoun Sadeghi-Bazargani, Laleh Bagheri, Yasaman Pourandish, Malihe Amiri, Ahmad Pour-Rashidi, James Harrop, Vafa Rahimi-Movaghar

Department of Neurosurgery Faculty Papers

STUDY DESIGN: A retrospective study.

OBJECTIVES: The quality of care (QoC) for spinal column/cord injury patients is a major health care concern. This study aimed to implement the QoC assessment tool (QoCAT) in the National Spinal Cord/Column Injury Registry of Iran (NSCIR-IR) to define the current state of pre- and post-hospital QoC of individuals with Traumatic Spinal Column and Spinal Cord Injuries (TSC/SCIs).

METHODS: The QoCAT, previously developed by our team to measure the QoC in patients with TSC/SCIs, was implemented in the NSCIR-IR. The pre-hospital QoC was evaluated through a retrospective analysis of NSCIR-IR registry data. Telephone interviews and …

Ethical Considerations Of Percutaneous Endoscopic Gastrostomy Tube Placement In A Patient With Creutzfeldt-Jakob Disease And Advanced Dementia, Toni Habib, Niamh Siobhán Mulrooney, Yisroel Grabie, Mohammad Maruf, Sophia Kasapira

HCA Healthcare Journal of Medicine

Background

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative condition that is not easily diagnosed and has no curative treatments available, essentially requiring palliative measures, including tube feeding. Nonetheless, data shows that the endoscope used for percutaneous endoscopic gastrostomy (PEG) tube insertion can be a vector for CJD transmission.

Case Presentation

We discuss the case of an elderly patient diagnosed with CJD, requiring tube feeding. Multiple specialists were consulted, and they were unable to reach an agreement on the best approach. Eventually, the hospital ethics committee intervened, and the family agreed to comfort measures only. This case report sheds light …

Fluorescence Lifetime Imaging Ophthalmoscopy (Flio): A Novel Retinal Metabolic Biomarker For Multiple Sclerosis, Daniel Markowitz, Kylee Shivok, Elizabeth Affel, Ms, Jose S. Pulido, Md, Ms, Mba, Mph, Thomas P. Leist, Md, Phd, Gyorgy Hajnoczky, Md, Phd, Mahdi Alizadeh, Phd, Robert Sergott, Md

Farber Institute for Neuroscience Staff Papers and Presentations

Introduction

- Fluorescence lifetime imaging ophthalmoscopy (FLIO, Heidelberg Engineering, Figures 1 and 2), a novel in vivo retinal imaging biomarker, generates fluorescence decay lifetimes in 2 spectral channels corresponding to the following metabolic processes:

- Short Spectral Channel (SSC): 498 – 560 nm

• Corresponds to macular pigment, flavin adenine dinucleotide (FAD) and oxidative phosphorylation.

- Long Spectral Channel (LSC): 560 – 720nm

• Corresponds to predominantly lipofuscin and lysosomal function

- Based upon animal experimental models and clinical data, mitochondrial dysfunction has a role in the pathophysiology of both inflammatory and degenerative components of Multiple Sclerosis

Spetzler-Martin Grade I And Ii Cerebral Arteriovenous Malformations: A Propensity-Score Matched Analysis Of Resection And Stereotactic Radiosurgery In Adult Patients, Salem M Tos, Mahmoud Osama, Georgios Mantziaris, Bardia Hajikarimloo, Nimer Adeeb, Sandeep Kandregula, Adam A Dmytriw, Hamza Adel Salim, Basel Musmar, Kareem El Naamani, Christopher Ogilvy, Douglas Kondziolka, Ahmed Abdelsalam, Deepak Kumbhare, Sanjeev Gummadi, Cagdas Ataoglu, Ufuk Erginoglu, Muhammed Amir Essibayi, Abdullah Keles, Sandeep Muram, Daniel Sconzo, Howard Riina, Arwin Rezai, Johannes Pöppe, Rajeev D Sen, Omar Alwakaa, Christoph J Griessenauer, Pascal Jabbour, Stavropoula I Tjoumakaris, Jan-Karl Burkhardt, Robert M Starke, Mustafa Baskaya, Laligam N Sekhar, Michael R Levitt, David J Altschul, Neil Haranhalli, Malia Mcavoy, Assala Aslan, Abdallah Abushehab, Christian Swaid, Adib Abla, Christopher Stapleton, Matthew Koch, Visish M Srinivasan, Peng R Chen, Spiros Blackburn, Mark J Dannenbaum, Omar Choudhri, Bryan Pukenas, Darren Orbach, Edward Smith, Markus Möhlenbruch, Ali Alaraj, Ali Aziz-Sultan, Aman B Patel, Hugo H Cuellar, Michael Lawton, Jacques Morcos, Bharat Guthikonda, Jason Sheehan

Faculty, Staff and Student Publications

Cerebral arteriovenous malformations (AVMs) are congenital vascular anomalies that can lead to severe complications, including hemorrhage and neurological deficits. This study compares the outcomes of microsurgical resection and stereotactic radiosurgery (SRS) for SM grade I and II AVMs. Out of a large multicenter registry, we identified 180 matched patients with SM grade I and II AVMs treated with either microsurgical resection or SRS between 2010 and 2023. The primary outcomes were AVM obliteration rates and complications; secondary outcomes included neurological status and functional outcomes measured by the modified Rankin Scale (mRS). Propensity score matching (PSM) was utilized to ensure comparability …

Inhibition Of Il-6 Trans-Signaling Promotes Post-Stroke Functional Recovery In A Sex And Dose-Dependent Manner, Cassandra Hall, Dustin T Nguyen, Kate Mendoza, Chunfeng Tan, Anjali Chauhan

Faculty, Staff and Student Publications

Introduction: Elevated circulating IL-6 levels are associated with poorer outcomes after stroke, and increased serum IL-6 levels are linked to a higher risk of stroke. IL-6 binds to soluble IL-6 receptors (sIL-6R) and subsequently to ubiquitously expressed gp130, initiating proinflammatory trans-signaling. This study tested the hypothesis that inhibiting IL-6 trans-signaling by administering soluble (s) gp130 improves long-term functional outcomes in young mice after stroke.

Methods: Recombinant mouse gp130Fc chimera (sgp130) was administered one hour after middle cerebral artery occlusion (MCAO) followed by twice-weekly administration for 2 weeks in mice (8-15 weeks old). Behavioral assessments were done on days 7 and …

At-Home Stroke Neurorehabilitation: Early Findings With The Neuroexo Bci System, Juan José González-España, Lianne Sánchez-Rodríguez, Maxine Annel Pacheco-Ramírez, Jeff Feng, Kathryn Nedley, Shuo-Hsiu Chang, Gerard E Francisco, Jose L Contreras-Vidal

Faculty, Staff and Student Publications

Background: Democratized access to safe and effective robotic neurorehabilitation for stroke survivors requires innovative, affordable solutions that can be used not only in clinics but also at home. This requires the high usability of the devices involved to minimize costs associated with support from physical therapists or technicians.

Methods: This paper describes the early findings of the NeuroExo brain-machine interface (BMI) with an upper-limb robotic exoskeleton for stroke neurorehabilitation. This early feasibility study consisted of a six-week protocol, with an initial training and BMI calibration phase at the clinic followed by 60 sessions of neuromotor therapy at the homes of …

Neural Signal Analysis In Chronic Stroke: Advancing Intracortical Brain-Computer Interface Design, Nabila Shawki, Alessandro Naopli, Carlos E. Vargas-Irwin, Christopher K. Thompson, John P. Donoghue, Mijail D. Serruya

Department of Medicine Faculty Papers

INTRODUCTION: Intracortical Brain-computer interfaces (iBCIs) are a promising technology to restore function after stroke. It remains unclear whether iBCIs will be able to use the signals available in the neocortex overlying stroke affecting the underlying white matter and basal ganglia.

METHODS: Here, we decoded both local field potentials (LFPs) and spikes recorded from intracortical electrode arrays in a person with chronic cerebral subcortical stroke performing various tasks with his paretic hand, with and without a powered orthosis. Analysis of these neural signals provides an opportunity to explore the electrophysiological activities of a stroke affected brain and inform the design of …

Glycemic Variability And Migraine: New Insights From Glucose Monitoring, Christina A. Nelson, Kyle Reavely, Matthew Jennings, Brandon Burger, Jackson Nielsen, Amanda Page, Alexander Kim, David Sant, Kyle Bills

Annual Research Symposium

No abstract provided.

Single-Cell Analysis Identifies Ifi27l2a As A Gene Regulator Of Microglial Inflammation In The Context Of Aging And Stroke In Mice, Gab Seok Kim, Elisabeth Harmon, Manuel C Gutierrez, Sodam Kim, Lauren Vance, Haven Burrous, Jessica M Stephenson, Anjali Chauhan, Anik Banerjee, Zachary Wise, Andrea Doan, John Ahn, Ting Wu, Jesus Bautista-Garrido, Juneyoung Lee, Chunfeng Tan, Joo Eun Jung, Louise D Mccullough, Joshua D Wythe, Sean P Marrelli

Faculty, Staff and Student Publications

Inflammation is a significant driver of ischemic stroke pathology in the brain. To identify potential regulators of inflammation, we performed single-cell RNA sequencing (scRNA-seq) of young and aged mouse brains following stroke and found that interferon alpha-inducible protein 27 like 2 A (Ifi27l2a) was significantly up-regulated, particularly in microglia of aged brain. Ifi27l2a is induced by interferons for viral host defense and has been linked with pro-inflammatory cellular mechanisms. However, its potential role in neurodegeneration is unknown. Using a combination of cell culture, experimental stroke models in mice, and human autopsy brain samples, we demonstrated that induction of Ifi27l2a occurs …

Treatment Of Small Intracranial Aneurysms Using The Smallss Scoring System: A Novel System For Decision Making, Mira Salih, Michael Young, Thomas B. Fodor, Alexander Andreev, Samuel D. Pettersson, Joanna M. Roy, Basel Musmar, Max Shutran, Phillip Taussky, Christopher S. Ogilvy

Department of Neurosurgery Faculty Papers

BACKGROUND: Treatment of unruptured intracranial aneurysms (UIA) less than 7 mm is controversial. We created a scoring system to guide decision making and validated the system utilizing over 700 cases treated with microsurgery or endovascular procedures.

METHODS: The scoring system SMALLSS included Size, (4-7 mm: 1 point, < 3.9 mm: 0 point), Multiple aneurysms (yes: 1, no: 0), Anatomic location (posterior: 1, anterior: 0), Lineage- family history of aneurysm (yes:1, no: 0), Lifetime risk (age < 65: 1, age > 65: 0), Smoking history (yes: 1, no: 0 ), Shape (irregular:1, smooth:0 ). Validation of this system was performed through retrospective review of prospectively maintained data for UIA patients treated between 2014 and 2021. We then performed an external validation of a cohort of 200 small aneurysms < 7 mm treated at a high volume cerebrovascular center.

RESULTS: A total of 1152 cases …

Mexiletine Dosing And Tolerability In Refractory Chronic Migraine: Insights From Post-Lidocaine Infusion Treatment, Lucia Liao, Hsiangkuo Yuan, Dylan Selbst, Emily Casaletto, Jiangang Liao, Clinton Lauritsen

Department of Neurology Faculty Papers

Background: Mexiletine is used as a preventive medication for refractory chronic migraine (rCM) and to prolong migraine improvement following intravenous lidocaine infusion. However, published evidence supporting its use in migraine remains limited. This retrospective study describes the dosing, treatment duration, and adverse effects of mexiletine in a group of patients with rCM following lidocaine infusion. Methods: Electronic medical records were reviewed at the Jefferson Headache Center. Baseline pain intensity and monthly headache days were collected at baseline and up to 6 office visits within 1-year post-discharge. The duration of mexiletine treatment was evaluated for up to 40 months post-discharge. Results: …

Use Of Synthetic Water-Soluble Kcc2 To Modulate Chloride Homeostasis In Cultured Dopamine-Neuron-Like Cells, Mason Terry, Andrew Payne, Dario Mizrachi

Annual Research Symposium

No abstract provided.

A Targeted Gene Expression Biomarker Predicts Clinic Low-Risk Meningioma Recurrence, Minh P Nguyen, Ramin A Morshed, Mark W Youngblood, Haley K Perlow, Calixto-Hope G Lucas, Akash J Patel, Joshua D Palmer, Craig M Horbinski, Stephen T Magill, William C Chen, David R Raleigh

Duncan NRI Faculty and Staff Publications

Background: Despite reassuring clinical and histological features, low-grade meningiomas can recur after surgery. Targeted gene expression profiling improves risk stratification of meningiomas, but the utility of this approach for clinical low-risk meningiomas is incompletely understood.

Methods: This was a multicenter retrospective cohort study of meningiomas from patients who were treated at 4 institutions from 1992 to 2023. Adult patients with newly diagnosed or recurrent World Health Organization (WHO) grade 1 meningiomas that were treated with gross total resection (GTR) or subtotal resection (STR), or newly diagnosed WHO grade 2 meningiomas that were treated with GTR, were included. A 34-gene expression …

Neuronal Network Activation Induced By Forniceal Deep Brain Stimulation In Mice, Bin Tang, Zhenyu Wu, Qi Wang, Jianrong Tang

Faculty, Staff and Students Publications

Background: The fimbria-fornix is a nerve fiber bundle that connects various structures of the limbic system in the brain and plays a key role in cognition. It has become a major target of deep brain stimulation (DBS) to treat memory impairment in both dementia patients and animal models of neurological diseases. Previously, we have reported the beneficial memory effects of chronic forniceal DBS in mouse models of intellectual disability disorders. In Rett syndrome and CDKL5 deficiency disorder models, DBS strengthens hippocampal synaptic plasticity, reduces dentate inhibitory transmission or increases adult hippocampal neurogenesis that aids memory. However, the underlying neuronal circuitry …

Motor Pool Selectivity Of Neuromuscular Degeneration In Type I Spinal Muscular Atrophy Is Conserved Between Human And Mouse, Justin C Lee, Wendy K Chung, David J Pisapia, Christopher E Henderson

Faculty, Staff and Students Publications

Spinal muscular atrophy (SMA) is caused by low levels of the survival motor neuron (SMN) protein. Even though SMN is ubiquitously expressed, the disease selectively affects motor neurons, leading to progressive muscle weakness. Even among motor neurons, certain motor units appear more clinically resistant to SMA. To quantitatively survey selective resistance, we studied extensive neuromuscular autopsies of Type I SMA patients and age-matched controls. We found highly divergent degrees of degeneration of neighboring motor units, even within individual cranial nerves or a single anatomical area such as the neck. Examination of a Type I SMA patient maintained on life support …

Tdp43 Negtively Regulates Tbk1-Medicated Ifn1 Production Through Irf7 Pathway In Neurodegenerative Diseases, Wenjuan Zhang, Zhen Yi, Daihe Yang, Yifan Hao, Feng Zhou, Guohua Song, Cao Huang, Yun Zhou, Bo Huang

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Mutations in the genes encoding TAR DNA-binding protein 43 (TDP43) or TANK- binding kinase 1 (TBK1) have been strongly associated with neurological disorders, including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. TDP43 is a key component of pathological protein aggregates found in more than 90% of ALS cases, while TBK1 plays a critical role in innate immune signaling and autophagy. Despite these associations, the precise molecular mechanisms linking TDP43 or TBK1 dysfunction to neurodegeneration remain poorly understood. The present study examined the impact of TDP43 on TBK1-mediated type I interferon (IFN1) production in HEK-293T cells. The findings demonstrated that co-expression …

Targeting Tiam1 Enhances Hippocampal-Dependent Learning And Memory In The Adult Brain And Promotes Nmda Receptor-Mediated Synaptic Plasticity And Function, Francisco A Blanco, Md Ali Bin Saifullah, Jinxuan X Cheng, Carlota Abella, Federico Scala, Karen Firozi, Sanyong Niu, Jin Park, Jeannie Chin, Kimberley F Tolias

Faculty, Staff and Students Publications

Excitatory synapses and the actin-rich dendritic spines on which they reside are indispensable for information processing and storage in the brain. In the adult hippocampus, excitatory synapses must balance plasticity and stability to support learning and memory. However, the mechanisms governing this balance remain poorly understood. Tiam1 is an actin cytoskeleton regulator prominently expressed in the dentate gyrus (DG) throughout life. Previously, we showed that Tiam1 promotes dentate granule cell synapse and spine stabilization during development, but its role in the adult hippocampus remains unclear. Here, we deleted Tiam1 from adult forebrain excitatory neurons (Tiam1^fKO) and assessed …

Murder Mystery Solved! Unmasking Multi-Generational Familial Death As A Result Of Brugada Syndrome, Ryan Tam, Sandres Aodish, Jeremiah Tom, Mohammad Jawish

Advances in Clinical Medical Research and Healthcare Delivery

Brugada syndrome is an inherited condition that has an increased risk of developing ventricular arrhythmias and sudden cardiac death. This condition can be diagnosed by using the Shanghai Scoring System. Here, we present a patient with an incidental finding of Type 1 Brugada pattern on electrocardiogram amidst atypical neurological findings on presentation to the emergency department and a history of cardiac-related familial deaths of uncertain etiology.

Unilateral Hearing Loss Caused By Bilateral Otosclerosis In The Context Of Prior Viral Infection - Case Report, Ryan Tam, Michael Mullinax, Timothy Ehmann, Bryan Scott

Advances in Clinical Medical Research and Healthcare Delivery

This is a case report of a patient who presented with left sided hearing loss. The patient was found to have mixed conductive and sensorineural hearing loss of the left ear and restrictive hearing of the right ear. Subsequent imaging demonstrated evidence of bilateral otosclerosis. Otosclerosis is characterized by progressive pathological bone remodeling in the otic capsule, resulting in hearing loss. Given the disease burden of otosclerosis and the corresponding effects on quality of life, providers must have knowledge of the disease to incorporate it into working differential diagnoses for patients presenting with hearing loss. With increasing recognition of otosclerosis …

Anatomy And Variations Of The Carina And Its Cartilaginous Makeup: A Cadaveric Study, Chung Yoh Kim, Kristen Rizzuto, Daniel Tahan, Devendra Shekhawat, Rarinthorn Samrid, Yoko Tabira, Stephen J. Bordes, Joe Iwanaga, R. Shane Tubbs

School of Medicine Faculty Publications

Purpose: The carina, located at the bifurcation of the trachea, has been regarded as a part of the trachea. Although clinically useful as an anatomical landmark, studies of its detailed morphology are lacking in the literature. Methods: The distal trachea and left and right main bronchi were harvested from 32 cadavers and the carina studied using microsurgical dissection, endoscopy, micro-CT, and histology. Results: The right bronchial cartilages were most commonly involved in forming the carina (72.41%), and the left bronchial cartilages were the second most commonly involved (37.93%). The carinal cartilages were slightly deviated to the left of midline in …

Early Surgical Decompression For The Treatment Of Hourglass-Like Constriction Of The Posterior Interosseus Nerve, Nathan Redlich, Matan Ozery, Adam Haydel, Charles Cush, Rasheed Ahmad

School of Medicine Faculty Publications

Posterior interosseous nerve (PIN) syndrome refers to a rare compressive neuropathy of the PIN resulting in motor dysfunction of the forearm extensor compartment. Often, surgical exploration will reveal extrinsic structures that mechanically compress the nerve. Less frequently, PIN syndrome has been reported with a visible hourglass-like constriction and no identifiable causative adjacent structures. In our report, we describe an adolescent patient with PIN syndrome and associated hourglass-like constriction of the PIN. For diagnosis, we used physical examination, preoperative imaging, nerve studies, and intraoperative findings. To monitor recovery, we performed serial neurological examinations and documented patient-reported functional outcomes. The patient underwent …

From Bench To Bedside: Murine Models Of Inherited And Sporadic Brain Arteriovenous Malformations, Ashely R Ricciardelli, Gael Genet, Nafiisha Genet, Samuel T Mcclugage, Peter T Kan, Karen K Hirschi, Jason E Fish, Joshua D Wythe

Faculty, Staff and Students Publications

Brain arteriovenous malformations are abnormal vascular structures in which an artery shunts high pressure blood directly to a vein without an intervening capillary bed. These lesions become highly remodeled over time and are prone to rupture. Historically, brain arteriovenous malformations have been challenging to treat, using primarily surgical approaches. Over the past few decades, the genetic causes of these malformations have been uncovered. These can be divided into (1) familial forms, such as loss of function mutations in TGF-β (BMP9/10) components in hereditary hemorrhagic telangiectasia, or (2) sporadic forms, resulting from somatic gain of function mutations in genes involved in …

Understanding Pediatric Bipolar Disorder Through The Investigation Of Clinical, Neuroanatomic, Neurophysiological And Neurocognitive Dimensions: A Pilot Study, Alessio Simonetti, Evelina Bernardi, Sherin Kurian, Antonio Restaino, Claudia Calderoni, Emanuela De Chiara, Francesca Bardi, Gabriele Sani, Jair C Soares, Kirti Saxena

Faculty, Staff and Student Publications

Background: Pathophysiological models of pediatric bipolar disorder (PBD) are lacking. Multimodal approaches may provide a comprehensive description of the complex relationship between the brain and behavior. Aim: To assess behavioral, neuropsychological, neurophysiological, and neuroanatomical alterations in youth with PBD.

Methods: Subjects with PBD (n = 23) and healthy controls (HCs, n = 23) underwent (a) clinical assessments encompassing the severity of psychiatric symptoms, (b) neuropsychological evaluation, (c) analyses of event-related potentials (related to the passive viewing of fearful, neutral, and happy faces during electroencephalography recording, and (d) cortical thickness and deep gray matter volume measurement using magnetic …

Investigating The In Vivo Effects Of Anti-Prion Protein Nanobodies On Prion Disease With Aav Vector, Jingjing Zhang, Mengfei Wang, Dan Wang, Xiangyi Zhang, Yue Ma, Els Pardon, Jan Steyaert, Romany Abskharon, Fei Wang, Jiyan Ma

Faculty, Staff and Student Publications

Prion diseases are fatal neurodegenerative disorders affecting humans and animals, and the central pathogenic event is the conversion of normal prion protein (PrPC) into the pathogenic PrPSc isoform. Previous studies have identified nanobodies that specifically recognize PrPC and inhibit the PrPC to PrPSc conversion in vitro. In this study, we investigated the potential for in vivo expression of anti-PrPC nanobodies and evaluated their impact on prion disease. The coding sequences of three nanobodies were packaged into recombinant adeno-associated virus (rAAV) and were administered via intracerebroventricular (ICV) injection in newborn mice. We found that the expression of these nanobodies remained robust …