Nervous System Diseases Commons^™

Accelerated Symptom Improvement In Parkinson’S Disease Via Remote Internet-Based Optimization Of Deep Brain Stimulation Therapy: A Randomized Controlled Multicenter Trial, Alireza Gharabaghi, Sergiu Groppa, Marta Navas-Garcia, Alfons Schnitzler, Laura Muñoz-Delgado, Vicky L. Marshall, Jessica Karl, Lin Zhang, Ramiro Alvarez, Mary S. Feldman, Michael J. Soileau, Lan Luo, S. Elizabeth Zauber, Benjamin L. Walter, Chengyuan Wu, Hong Lei, Damian M. Herz, Ming-Hua Chung, Yagna Pathak, Bram Blomme, Binith Cheeran, Corneliu Luca, Daniel Weiss

Department of Neurosurgery Faculty Papers

BACKGROUND: Deep brain stimulation (DBS) has emerged as an important therapeutic intervention for neurological and neuropsychiatric disorders. After initial programming, clinicians are tasked with fine-tuning DBS parameters through repeated in-person clinic visits. We aimed to evaluate whether DBS patients achieve clinical benefit more rapidly by incorporating remote internet-based adjustment (RIBA) of stimulation parameters into the continuum of care.

METHODS: We conducted a randomized controlled multicenter study (ClinicalTrails.gov NCT05269862) involving patients scheduled for de novo implantation with a DBS System to treat Parkinson's Disease. Eligibility criteria included the ability to incorporate RIBA as part of routine follow-up care. Ninety-six patients were …

Network Analysis Of Headache Diagnoses Using International Classification Of Headache Disorders, 3rd Edition, Pengfei Zhang, Thomas Berk

College of Nursing Faculty Papers & Presentations

BACKGROUND AND OBJECTIVE: The International Classification of Headache Disorders, Third Edition (ICHD-3), significantly influences clinicians' understanding of headache disorders. In this study, we aim to elucidate how the hierarchical structure of ICHD-3 shapes the understanding of interconnectivity among headache disorders.

METHODS: A network comprises elements known as "nodes," with the connections between them referred to as "edges." In our study, a node represents a headache diagnosis that meets at least one ICHD-3 diagnostic criterion of the ICHD-3. We developed two network models for ICHD-3: a non-hierarchical model, where edges are only formed by cross-references found within the text of diagnoses, …

Use Of Multi-Modal Non-Contrast Mri To Predict Functional Outcomes After Stroke: A Study Using Dp-Pcasl, Dti, Noddi, And Map Mri, Julia Diamandi, Christian Raimondo, Keenan Piper, Joanna Roy, Stephanie Serva, Mahdi Alizadeh, Adam Flanders, Stavropoula Tjoumakaris, Michael Gooch, Pascal Jabbour, Robert Rosenwasswer, Nikolaos Mouchtouris

Department of Neurosurgery Faculty Papers

PURPOSE: This study aims to assess whether water exchange rate (k_w), a surrogate for blood-brain barrier (BBB) permeability, is associated with functional outcomes in patients with acute ischemic stroke (AIS).

METHODS: We studied 22 AIS patients enrolled from 1/2022 to 4/2024 who underwent multi-modal non-contrast imaging on a 3.0-Tesla scanner, including DP-pCASL, DTI, NODDI and MAP imaging. For each parametric map, the intensity and standard deviation (SD) were calculated for the infarcted region. The diffusion maps included were b0, Fractional Anisotropy (FA), Mean Diffusivity (MD), Intra-cellular Volume Fraction (ICVF), Free Water Fraction (FWF), and Orientation Dispersion Index (ODI), …

A Randomized, Placebo-Controlled, Cross-Over Trial Of Ketamine In Rett Syndrome, Kathleen Campbell, Jeffrey L Neul, David N Lieberman, Elizabeth Berry-Kravis, Tim A Benke, Cary Fu, Alan Percy, Bernhard Suter, David Morris, Randall L Carpenter, Eric D Marsh, Jana Von Hehn

Faculty, Staff and Students Publications

BACKGROUND: Preclinical studies and anecdotal case reports support the potential therapeutic benefit of low-dose oral ketamine as a treatment of clinical symptoms in Rett syndrome (RTT); however, no controlled studies have been conducted in RTT to evaluate safety, tolerability and efficacy.

DESIGN: This was a sequentially initiated, dose-escalating cohort, placebo-controlled, double blind, randomized sequence, cross-over study of oral ketamine in 6-12-year-old girls with RTT to evaluate short-term safety and tolerability and explore efficacy.

METHODS: Participants were randomized to either five days treatment with oral ketamine or matched placebo, followed by a nine-day wash-out period and then crossed-over to the opposite …

Comparative Outcomes Of Endovascular Vs. Surgical Treatment In Craniocervical Junction Dural Arteriovenous Fistulas: A Systematic Review And Meta-Analysis, Basel Musmar, Joanna Roy, Atakan Orscelik, Sravanthi Koduri, Spyridon Karadimas, Saman Sizdahkhani, Elias Atallah, Stavropoula Tjoumakaris, Michael Gooch, Hekmat Zarzour, Ritam Ghosh, Richard Schmidt, Robert Rosenwasser, Pascal Jabbour

Department of Neurosurgery Faculty Papers

BACKGROUND: Craniocervical junction dural arteriovenous fistulas (CCJ-DAVFs) are rare and complex vascular malformations that are challenging to diagnose and treat. This study aims to compare surgical and endovascular treatments for CCJ-DAVFs through a systematic review and meta-analysis.

METHODS: A systematic review and meta-analysis was conducted according to the PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched from inception to July 2024.

RESULTS: Fifteen studies involving 266 patients were included. Of these, 143 (53.8 %) patients underwent surgical treatment alone and 123 (46.2 %) underwent endovascular treatment alone. In the surgical group, the complete obliteration rate at last …

Tdp43 Autoregulation Gives Rise To Dominant Negative Isoforms That Are Tightly Controlled By Transcriptional And Post-Translational Mechanisms, Megan M. Dykstra, Kaitlin Weskamp, Nicolás B. Gómez, Jacob Waksmacki, Elizabeth Tank, M. Rebecca Glineburg, Allison Snyder, Emile Pinarbasi, Michael Bekier, Xingli Li, Morgan R. Miller, Jen Bai, Shameena Shahzad, Neha Nedumaran, Clare Wieland, Corey Stewart, Sydney Willey, Nikolas Grotewold, Jonathon Mcbride, John J. Moran, Aditya V. Suryakumar, Michael Lucas, Peter M. Tessier, Michael Ward, Peter K. Todd, Sami J. Barmada

Biology, Chemistry, and Environmental Sciences Faculty Articles and Research

The nuclear RNA-binding protein TDP43 is integrally involved in the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Previous studies uncovered N-terminal TDP43 isoforms that are predominantly cytosolic in localization, prone to aggregation, and enriched in susceptible spinal motor neurons. In healthy cells, however, these shortened (s)TDP43 isoforms are difficult to detect in comparison to full-length (fl)TDP43, raising questions regarding their origin and selective regulation. Here, we show that sTDP43 is created as a by-product of TDP43 autoregulation and cleared by nonsense-mediated RNA decay (NMD). sTDP43-encoding transcripts that escape NMD are rapidly degraded post-translationally via the proteasome …

Molecular Logic For Cellular Specializations That Initiate The Auditory Parallel Processing Pathways, Junzhan Jing, Ming Hu, Tenzin Ngodup, Qianqian Ma, Shu-Ning Natalie Lau, M Cecilia Ljungberg, Matthew J Mcginley, Laurence O Trussell, Xiaolong Jiang

Faculty, Staff and Students Publications

The cochlear nuclear complex (CN), the starting point for all central auditory processing, encompasses a suite of neuronal cell types highly specialized for neural coding of acoustic signals. However, the molecular logic governing these specializations remains unknown. By combining single-nucleus RNA sequencing and Patch-seq analysis, we reveal a set of transcriptionally distinct cell populations encompassing all previously observed types and discover multiple hitherto unknown subtypes with anatomical and physiological identity. The resulting comprehensive cell-type taxonomy reconciles anatomical position, morphological, physiological, and molecular criteria, enabling the determination of the molecular basis of the specialized cellular phenotypes in the CN. In particular, …

Two-Year Follow-Up Of Gait And Postural Control Following Initiation Of Recombinant Human Tripeptidyl Intracerebroventricular Enzyme Replacement Therapy In Two Atypical Cln2 Patients, Rahul Soangra, Marybeth Grant-Beuttler, Harriet Chang, Raymond Y. Wang

Physical Therapy Faculty Articles and Research

Neuronal ceroid lipofuscinosis type 2 (CLN2) is a rapidly progressive neurodegenerative disorder leading to premature mortality. Ambulatory CLN2 patients typically receive standard of care treatment through biweekly intracerebroventricular (ICV) enzyme replacement therapy (ERT) involving recombinant human tripeptidyl peptidase 1, known as cerliponase alfa (Brineura^®, Biomarin Pharmaceuticals). This study longitudinally assessed the impact of ICV cerliponase alfa ERT on gait, and postural control across a two-year span in two siblings diagnosed with atypical CLN2 disease. Both participants, ID01 (18 years and 8 months old at enrollment) and ID02 (13 years and 3 months old at enrollment), exhibited symptomatic characteristics …

Rapid Neurological Decline Due To Creutzfeldt-Jakob Disease, Nicole Tesoro, David Blackwood, Robert Reif, Samuel Miller, Kalyan Paudel, Christopher Haas

Journal of Community Hospital Internal Medicine Perspectives

Creutzfeldt-Jakob Disease (CJD) is an exceedingly rare condition with an incidence of one case per million people worldwide. It is diagnosed with symptoms of rapid neurological decline, positive CSF biomarkers, and diagnostic findings on MRI and EEG. There is no known effective therapy for CJD and prompt diagnosis is required to prepare for the expected disease prognosis and goals of care discussions.

Multiparametric Mri Along With Machine Learning Predicts Prognosis And Treatment Response In Pediatric Low-Grade Glioma, Anahita Fathi Kazerooni, Adam Kraya, Komal Rathi, Meen Chul Kim, Arastoo Vossough, Nastaran Khalili, Ariana Familiar, Deep Gandhi, Neda Khalili, Varun Kesherwani, Debanjan Haldar, Hannah Anderson, Run Jin, Aria Mahtabfar, Sina Bagheri, Yiran Guo, Qi Li, Xiaoyan Huang, Yuankun Zhu, Alex Sickler, Matthew R Lueder, Saksham Phul, Mateusz Koptyra, Phillip Storm, Jeffrey Ware, Yuanquan Song, Christos Davatzikos, Jessica Foster, Sabine Mueller, Michael J Fisher, Adam Resnick, Ali Nabavizadeh

Department of Neurosurgery Faculty Papers

Pediatric low-grade gliomas (pLGGs) exhibit heterogeneous prognoses and variable responses to treatment, leading to tumor progression and adverse outcomes in cases where complete resection is unachievable. Early prediction of treatment responsiveness and suitability for immunotherapy has the potential to improve clinical management and outcomes. Here, we present a radiogenomic analysis of pLGGs, integrating MRI and RNA sequencing data. We identify three immunologically distinct clusters, with one group characterized by increased immune activity and poorer prognosis, indicating potential benefit from immunotherapies. We develop a radiomic signature that predicts these immune profiles with over 80% accuracy. Furthermore, our clinicoradiomic model predicts progression-free …

Nls-Binding Deficient Kapβ2 Reduces Neurotoxicity Via Selective Interaction With C9orf72-Als/Ftd Dipeptide Repeats, Kevin Kim, Amandeep Girdhar, Maria Elena Cicardi, V. Kankate, Miyuki Hayashi, Ruoyu Yang, Jenny Carey, Charlotte M Fare, James Shorter, Gino Cingolani, Davide Trotti, Lin Guo

Department of Biochemistry and Molecular Biology Faculty Papers

Arginine-rich dipeptide repeat proteins (R-DPRs) are highly toxic proteins found in patients with C9orf72-linked amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). R-DPRs can cause toxicity by disrupting the natural phase behavior of RNA-binding proteins (RBPs). Mitigating this abnormal phase behavior is, therefore, crucial to reduce R-DPR-induced toxicity. Here, we use FUS as a model RBP to investigate the mechanism of R-DPR-induced aberrant RBP phase transition. We find that this phase transition can be mitigated by Kapβ2. However, as a nuclear import receptor and phase modifier for PY-NLS-containing RBPs, the function of WT Kapβ2 could lead to undesired interaction with its …

Are You Sick Of Arsenic? Heavy Metal Poisoning In A Masonry Contractor Presenting With Atypical Neurologic Symptoms, Trent Percy, Lisa Flaherty

West Florida Division GME Research Day 2025

No abstract provided.

High-Grade Avm. Educational Vignette And Clinical Cases, Marcos Devanir Silva Da Costa, Marcelo Augusto Acosta Goiri, Daniela Coelho, Juan Alberto Paz Archila, Talita Helena Martins Sarti, Feres Chaddad-Neto

Archives of Neurosurgery

The arteriovenous malformation (AVM) is a vascular malformation characterized by fistulas between arteries and veins forming a nidus, without interposition of capillary vessels and of which early draining vein is identified at the digital angiography. Potential reports of its existence date from the second century AD. Spetzler and Martin (S-M) proposed a classification considering three features and Lawton then added new predictors. A maximum of 10 score can be achieved by applying the modified S-M grade system. This classification guides decision-making and the possible choice of the multimodal treatment (surgery, radiosurgery and embolization). We discuss historical, pathological, clinical and therapeutic …

Applications Of Motor Learning Strategies In Children With Neurodevelopmental Disorders: A Scoping Review, Kathlynne F. Eguia, Shamay S.M. Ng, Catherine M. Capio, Thomson W.L. Wong

Health Sciences Faculty Publications

Synthesized knowledge of motor learning strategies could be used to facilitate the motor development of children with neurodevelopmental disorders (NDDs). To map the current research, we followed the established framework and protocol for scoping reviews. Among the 25 papers, the most studied strategies include dual-task and observational learning. The studies predominantly involved children with cerebral palsy, but research is growing among children with intellectual disability, autism and developmental coordination disorder. Most of the studies integrated motor learning strategies in researcher-led interventions that are delivered over several weeks in school, home, or clinic settings. Further research using robust study designs and …

A Flea-Borne Mystery: Unraveling Murine Typhus In A Patient With Unexplained Encephalopathy, Jose Loayza Pintado, Jorge Aboytes Trevino, Ceasar Uribe

Gulf Coast Division GME Research Day 2025

No abstract provided.

Effective Communication In The Pronunciation Of Brain Death, Sumanth Reddy, Artur Amirkhanyan, Jeremiah Avarana, Praneetha Arge, Alicia Lay

North Texas Research Forum 2025

Background: Clinical brain death determination requires both technical proficiency and sensitive communication with families. However, residents often report inadequate training and confidence in performing and discussing these examinations. This study evaluated an educational intervention combining didactic training and simulation to improve residents’ competence in brain death protocols and family communication. Methods: A mixed-methods cohort study was conducted with resident physicians. Participants completed a pre-intervention questionnaire assessing baseline knowledge, experience, and confidence in brain death determination and communication. An educational session reviewed clinical criteria, examination protocols, and communication strategies. Residents then engaged in an in-person simulation with standardized actors portraying grieving …

Coexisting Type A And B Aortic Dissection With Extension To Carotid Arteries Resulting In Acute Ischemic Stroke, Dung Nguyen, Jilpa Shah, Amnah Andrabi

North Texas Research Forum 2025

Coexisting Type A and B aortic dissection with extension to carotid arteries resulting in acute ischemic stroke Abstract Background: Acute aortic dissection (AAD) is a rare cause of ischemic stroke. Additionally, it is even rarer to have a combined type A and type B aortic dissection. ADD is a high-mortality disease that can lead to immediate decompensation and can be masked by stroke-like symptoms, which can result in delayed or missed diagnosis. We report a patient with acute ischemic stroke (AIS) with subsequent combined type A and type B aortic dissection. Case Report: A 47-year-old man was admitted to the …

Atypical Presentation Of Moyamoya Disease Presenting With Severe Headache: A Case Report, Nilay Bhatt, Tirth Vyas

Gulf Coast Division GME Research Day 2025

No abstract provided.

Pembrolizumab Induced Myasthenia Gravis, Praise Ohabor, Alexis Lopez Cruz, Deekshita Valiveti, Brandi Addison, Zuhair Ali

Gulf Coast Division GME Research Day 2025

No abstract provided.

A Case Of Meningitis With Pyogenic Ventriculitis, Shterna Frand, Matthew Gutzmer, Melissa Jaworski, Calan Teel, Premal Patel

North Texas Research Forum 2025

Bacterial meningitis and subsequent pyogenic ventriculitis in patients with complex medical histories and prior cranial surgeries presents significant diagnostic and management challenges. While typical signs of infection such as fever and leukocytosis may be present, secondary complications including seizure, respiratory failure, and pyogenic ventriculitis can complicate the clinical course. This case illustrates the need for comprehensive evaluation and tailored intervention in immunocompromised patients presenting with atypical infection. The case involved a 63-year-old female with a prior craniotomy and multiple comorbidities, presenting to the Emergency Department with acute encephalopathy and suspected bacterial meningitis. Upon transfer to the ICU following acute onset …