Neurology Commons^™

A Case Of Neurogenic Orthostatic Hypotension Secondary To Pure Autonomic Failure, Lauren Cooper, William Studt, Rayan Islam, Richard Weinberger

The Journal of Integrated Primary Care

Neurogenic orthostatic hypotension (nOH) is a debilitating manifestation of autonomic dysfunction and is most commonly associated with neurodegenerative disorders, though it can occur in isolation. Pure autonomic failure (PAF) is a rare and often underrecognized cause of nOH, particularly in older adults. We report the case of an 80-year-old man with hereditary hemochromatosis who presented with recurrent falls due to severe orthostatic hypotension. Orthostatic vitals demonstrated a marked blood pressure drop with minimal heart rate response consistent with a neurogenic etiology. He lacked motor, cognitive, or sensory features of neurodegenerative disease, and extensive evaluation excluded secondary causes, supporting a diagnosis …

Acute Treatment Response Among Patients With Migraine Subtypes: A Real-World Study In Thailand, Thanin Asawavichienjinda

Chulalongkorn Medical Journal

Background: Migraine with and without aura may have distinct etiologies. Aura can vary in complexity (simple to complex), possibly reflecting differences in cortical involvement and influencing acute treatment response.

Objective: To compare acute treatment responses between migraine patients with and without aura, and across aura complexity levels in Thailand.

Methods: A retrospective cohort study was conducted from January 3, 2007, to June 30, 2022, at the Chulalongkorn Comprehensive Headache Center, King Chulalongkorn Memorial Hospital, Thai Red Cross Society. Baseline data were retrieved from baseline case-record forms, while clinical data were obtained from paper headache diaries and follow-up case-record form. The …

Adjunctive Middle Meningeal Artery Embolization After Surgical Evacuation Of Chronic Subdural Hematoma: An Updated Meta-Analysis Of Randomized Controlled Trials, Tallal M. Hashmi, Hafsa Arshad Azam Raja, Zain Tariq, Faizan Ahmed, Niraj Arora, Majid T. Aized, Adeel Saleemi, Ameer E. Hassan, Adnan Qureshi, Osama O. Zaidat

School of Medicine Publications

Background: Middle meningeal artery embolization (MMAE) has emerged as a promising adjunctive treatment for chronic subdural hematoma (cSDH); however, the efficacy and safety of adjunctive MMAE compared with surgery alone remains uncertain.

Methods: We systematically searched PubMed, Embase, and the Cochrane Central Register of Controlled Trials from inception through June 2026. Randomized controlled trials comparing adjunctive MMAE plus surgical evacuation with surgical evacuation alone in patients with cSDH were included. The primary outcome was hematoma recurrence. Pooled odds ratios (ORs) with 95% CIs were calculated using a random-effects model.

Results: A total of eight randomized controlled trials were included. The …

Interface Geometry Governs Probe Insertion Mechanics And Electrode Deviation In Layered Brain-Mimicking Phantoms For Deep Brain Stimulation, Siyu Chen, Chengyuan Wu, Ethan Anders, Rungun Nathan, Olivia M. Bailey, Qifu Wang, Curtis L. Johnson, Qianhong Wu

Department of Neurosurgery Faculty Papers

Parkinson's disease (PD) is a progressive neurological disorder often treated with Deep Brain Stimulation (DBS), a surgical procedure that involves implanting electrodes to improve motor function. Accurate electrode placement is critical for treatment efficacy but can be influenced by tissue deformation, insertion forces, and the mechanical properties of brain tissue. In this study, we investigated the role of layer interface angle as a controlled biomechanical factor affecting insertion forces and electrode deviation. A two-layer agar gel phantom was constructed to produce a prescribed stiffness transition between a lower-stiffness and a higher-stiffness gel layer, and its small-amplitude dynamic shear-stiffness contrast was …

Intravascular Immune Cell Labeling As A Critical Methodological Tool For Characterizing Cd4+ T Cells In The Lung In Eae, Gholamreza Azizi, Mohamad Rostami

Department of Neurology Faculty Papers

INTRODUCTION: Growing evidence highlights the lung -brain axis as a key contributor to CNS autoimmunity. However, accurate characterization of lung immune cells remains technically challenging because standard transcardial perfusion does not eliminate the lung's extensive marginated intravascular leukocyte pool, allowing circulating cells to contaminate analyses of the lung parenchyma.

METHODS: Experimental autoimmune encephalomyelitis (EAE) was induced in mice by immunization with MOG35-55 peptide in complete Freund's adjuvant, followed by pertussis toxin administration. Lung mononuclear cells were isolated after intravascular (IV) CD45 labeling, transcardial perfusion, and enzymatic digestion. Immune cell populations and intracellular cytokine production were analyzed by multicolor flow cytometry …

Arriving At A Diagnosis: Effective Strategies Used By The Undiagnosed Diseases Network, Somin Hwang, Rachel M Brown, Dustin Baldridge, Erin E Baldwin, Alan H Beggs, Jonathan A Bernstein, Elizabeth Blue, Nicholas A Borja, Lorenzo D Botto, Lauren C Briere, Thomas Cassini, Cecilia Esteves, Elizabeth L Fieg, Gail P Jarvik, Shilpa Nadimpalli Kobren, Mia P Levanto, Julian A Martínez-Agosto, Shruti Marwaha, Stanley F Nelson, Jill A Rosenfeld, Tim Schedl, Tina K Truong, Jennifer A Wambach, Matthew T Wheeler, Shinya Yamamoto, Undiagnosed Diseases Network, Vandana Shashi, Alexa T Mccray, David R Adams, Kimberly Leblanc

Duncan NRI Faculty and Staff Publications

Purpose: Despite the increasing use of exome sequencing (ES) and genome sequencing (GS) in clinical settings, many individuals remain undiagnosed. This study examined the Undiagnosed Diseases Network (UDN)'s approach to establishing diagnoses for participants.

Methods: As a continuation of the first UDN cohort (9/16/2015-5/23/2017), this study reviewed diagnostic strategies and outcomes in the second UDN cohort (5/24/2017-6/30/2023).

Results: Over the study period, the proportion of UDN participants with prior ES/GS increased from 40.2% to 75.0%. The diagnostic rate for the UDN was 22.1% (379/1,713). Reanalysis/reinterpretation of sequencing data (prior ES: 21.4%, prior GS: 2.6%, UDN ES: 4.8%, UDN GS: 17.3%) …

Neuroimmune Phenotyping As The Next Frontier In Chronic Pain Medicine For Musculoskeletal Back Pain, Samer G. Salman, Rohan A. Phadke, Adithya Nair, Rahul Kumar, Arbaz Momin, Alireza Tavakkoli, James Rizkalla

Department of Neurosurgery Posters

Background: Chronic low back pain encompasses heterogeneous mechanical, neuropathic, nociplastic, and myofascial presentations that may reflect distinct neuroimmune mechanisms. We synthesized evidence linking these phenotypes to measurable biological features and clinically relevant treatment strategies.

Methods: We conducted a structured literature search of MEDLINE, Scopus, Web of Science, and the Cochrane Central Register of Controlled Trials for studies published from January 2005 through January 2025. Systematic reviews, randomized controlled trials, and mechanistic studies addressing neuroinflammation, glial activation, central sensitization, biomarkers, or imaging were organized according to four clinically relevant pain phenotypes and translational readiness.

Results: Neuroimmune findings differed across axial mechanical, …

Artificial Intelligence Models For Automated And Semiautomated Analysis And Interpretation Of Clinical Electroencephalography, Sándor Beniczky, Birgit Frauscher, Fábio Nascimento, Shobi Sivathamboo, Catalina Rojas, Michael Sperling, Samden Lhatoo, Philippe Ryvlin, Levin Kuhlmann

Department of Neurology Faculty Papers

Electroencephalography is the most commonly used diagnostic tool for epilepsy. However, interpreting electroencephalograms (EEGs) requires expertise that is not widely available. Advances in digital technology and wearables have enabled large-scale EEG recording, generating vast amounts of data that cannot be managed through traditional visual interpretation by experts. Artificial intelligence (AI) has the potential to augment human expertise and reduce workloads. The application of artificial neural networks in analysing clinical EEG recordings has led to major breakthroughs, bringing AI-based EEG interpretation closer to clinical implementation. In this Review, we summarise the most important research and development results in this field from …

Characteristics Of Individuals With Cerebral Palsy Across The Us., Bhooma R. Aravamuthan, Amy F. Bailes, Micah Baird, Kristie Bjornson, Ira Bowen, Angeline Bowman, Elizabeth R. Boyer, Rose N. Gelineau-Morel, Laurie Glader, Paul H. Gross, Stacey Hall, Edward A. Hurvitz, Michael Kruer, Thomas Larrew, Neena I. Marupudi, Patrick G. Mcphee, Stephen A. Nichols, Garey Noritz, Joyce Oleszek, Justin Ramsey, Jeffrey S. Raskin, Heather Riordan, Brandon G. Rocque, Manish N. Shah, Benjamin J. Shore, M Wade Shrader, David Spence, Charles B. Stevenson, Richard Stevenson, Sruthi P. Thomas, Joyce P. Trost, Stephen R. Wisniewski, Cerebral Palsy Research Network

Manuscripts, Articles, Book Chapters and Other Papers

This cross-sectional study examines how demographics and characteristics of individuals with cerebral palsy across the US associate with Gross Motor Function Classification System level and genetic etiology.

Predictors Of Complete Obliteration And Favourable Outcome After Single-Modality Treatment For Low-Grade Arteriovenous Malformations: A Multicentre Study, Angelica Fuentes, Salem Tos, Natasha Ironside, Georgios Mantziaris, Yuki Shinya, Basel Musmar, Hamza Adel Salim, Nimer Adeeb, Christopher Ogilvy, Douglas Kondziolka, Ali Alaraj, Min Park, Adam Dmytriw, Hussein Zeineddine, Finn Mccarthy, Hussam Abou-Al-Shaar, Ahmed Abdelsalam, Mustafa Baskaya, Cagdas Ataoglu, Anthony Sanchez-Forteza, Muhammed Amir Essibayi, Abdullah Keles, Sandeep Muram, Howard Riina, Arwin Rezai, Sahin Hanalioglu, Ufuk Erginoglu, Johannes Pöppe, Davide Simonato, Yan-Lin Li, Sandeep Kandregula, Dhairya Lakhani, Christoph Griessenauer, Rahim Abo Kasem, Alejandro Spiotta, Ajit Puri, Jasmeet Singh, Anna Luisa Kuhn, Jan Karl Burkhardt, Robert Starke, Laligam Sekhar, Michael Levitt, David Altschul, Neil Haranhalli, Malia Mcavoy, Paul Foreman, Osama Zaidat, Mohammad Almajali, Hakeem Shakir, Alfred Pokmeng See, Adib Abla, Aashay Patel, Andrew Nguyen, Matthew Koch, Visish Srinivasan, Peng Roc Chen, Spiros Blackburn, Ketan Bulsara, Peter Kan, Louis Kim, Omar Choudhri, Stavropoula Tjoumakaris, Pascal Jabbour, Amey Savardekar, Hugo Cuellar, Michael Lawton, Bharat Guthikonda, Jacques Morcos, Jason Sheehan

Department of Neurology Faculty Papers

INTRODUCTION: Patient and arteriovenous malformation (AVM) characteristics that portend success after treatment for low-grade brain AVMs remain unknown.

PATIENTS AND METHODS: We utilised the MISTA multicentre registry to identify patients with Spetzler-Martin (SM) grade I or II AVMs treated with stand-alone curative-intent intervention (resection, stereotactic radiosurgery [SRS] or endovascular embolisation). Bivariate and multivariable analyses were performed to identify patient and AVM characteristics predictive of complete obliteration or favourable outcome (complete obliteration without new permanent deficit or post-treatment haemorrhage).

RESULTS: A total of 522 patients were included (292 microsurgery, 152 SRS, 78 embolisation). Complete obliteration rates differed between microsurgery (95.9%), SRS …

Variation In Meningioma Recurrence Risk Estimates Across Observational Cohorts: The Influence Of Calendar Time, Who Classifications, Geographical Settings, And Healthcare Systems, Christian Mirian, Lasse Rehné Jensen, Adam Gorm Hoffmann, Tareq A Juratli, Anders Broechner, Sverre H Torp, Helen A Shih, Ramin A Morshed, Jacob S Young, Stephen T Magill, Luca Bertero, Walter Stummer, Dorothee Cäcilia Spille, Benjamin Brokinkel, Soichi Oya, Satoru Miyawaki, Nobuhito Saito, Martin Proescholdt, Yasuhiro Kuroi, Konstantinos Gousias, Matthias Simon, Jennifer Moliterno, Ricardo Prat-Acin, Stéphane Goutagny, Vikram C Prabhu, John T Tsiang, Johannes Wach, Erdem Güresir, Junkoh Yamamoto, Young Zoon Kim, Joo Ho Lee, Daniel W Kim, Matthew Koshy, Karthikeyan Perumal, Mustafa K Baskaya, Donald M Cannon, Dennis C Shrieve, Chang-Ok Suh, Jong Hee Chang, Maria Kamenova, Sven Straumann, Jehuda Soleman, Ilker Y Eyüpoglu, Tony Catalan, Austin Lui, Philip V Theodosopoulos, Michael W Mcdermott, Fang Wang, Pedro Góes, Manoel Antonio De Paiva Neto, Ricardo Komotar, Michael E Ivan, Aria Jamshidi, Evan Luther, Luis Souhami, Marie-Christine Guiot, Tamás Csonka, Toshiki Endo, Olivia Claire Barrett, Randy Jensen, Tejpal Gupta, Akash J Patel, Tiemo J Klisch, Jun Won Kim, Francesco Maiuri, Valeria Barresi, María Dolores Tabernero, Simon Skyrman, Ian Law, Bjarne Winther Kristensen, Tina Nørgaard Munch, Torstein Meling, Kåre Fugleholm, Paul Blanche, Tiit Mathiesen, Andrea Daniela Maier

Duncan NRI Faculty and Staff Publications

Purpose: Recurrence risk estimates underpin meningioma research, including molecular classification and clinical trial benchmarking, yet are often based on retrospective or historical data. The aim of this study was to assess the variation of recurrence risk estimates across calendar periods, WHO classification editions, geographical settings, and healthcare systems.thetermine METHODS: We analyzed 4,111 patients with primary WHO-1/-2 meningiomas from 31 centers in 15 countries (1990-2019). Recurrence was defined according to local radiological assessment. The 5- and 10-year recurrence risks were estimated using regression standardization with inverse probability of censoring weights, adjusting for key clinical, surgical, and histopathological variables.

Results: Recurrence risk …

Mechanism Of Injury And Fracture Status Influence Outcomes After Central Cord Injury, Karlo M. Pedro, Mohammed Ali Alvi, Kathleen Zang, Christopher Lozano, Jetan H. Badhiwala, Jefferson R. Wilson, James Harrop, James D. Guest, Bizhan Aarabi, Christopher I. Shaffrey, Michael G. Fehlings

Department of Neurosurgery Faculty Papers

Central cord syndrome (CCS) is the most common form of incomplete cervical spinal cord injury (SCI), yet its optimal management remains controversial. We examined whether stratifying CCS by injury stability improves treatment assessment. CCS was defined as an American Spinal Injury Association Impairment Scale (AIS) C/D cervical SCI with a ≥5-point lower-upper extremity motor score difference and classified as CCS-stable (CCS-S) or -unstable (CCS-U) based on the absence or presence of fracture/dislocation. Functional recovery at 1 year was assessed using the functional independence measure (FIM) motor score. Among 291 patients, 131 (45%) had CCS-S. These patients were older and more …

International Federation Of Clinical Neurophysiology Standardized Stereo-Electroencephalography Terminology, Birgit Frauscher, Guoping Ren, Chifaou Abdallah, Fabrice Bartolomei, Milan Brazdil, Francine Chassoux, Patrick Chauvel, Mark Cook, Beate Diehl, Guada Fernandez Baca Vaca, Stefano Francione, Vadym Gnatkovsky, Walter Gonzalez, Akio Ikeda, Jean Gotman, Julia Jacobs, Lara Jehi, Philippe Kahane, Hui Ming Khoo, Sam Lhatoo, Louis Maillard, Daniel Mansilla, Aileen Mcgonigal, Ioana Mindruta, Dang Khoa Nguyen, Piero Perucca, Ivan Rektor, Sylvain Rheims, Philippe Ryvlin, Stephan Schuele, Andreas Schulze-Bonhage, Margitta Seeck, Jaysingh Singh, Michael Sperling, Rainer Surges, Laura Tassi, Eugen Trinka, Manjari Tripathi, Shuang Wang, Samuel Wiebe, Gregory Worrell, Sandor Beniczky

Department of Neurology Faculty Papers

OBJECTIVE: Stereo-electroencephalography (SEEG) is an intracranial EEG methodology used in presurgical epilepsy evaluation to precisely delineate the epileptogenic zone and associated networks. Although established for decades, its global adoption has accelerated due to a favorable safety profile and clinical effectiveness. However, inconsistent terminology limits comparability of findings and multicenter collaboration. This guideline addresses this gap by providing standardized SEEG terminology based on international consensus.

METHODS: A working group of 41 experts appointed by the International Federation of Clinical Neurophysiology (IFCN) Executive Committee used a modified Delphi process to develop standardized terminology. The process included two meetings and four Delphi rounds, …

Remarkable Seizure Reduction With Trofinetide Treatment In An Adult With Rett Syndrome: A Case Report, Semihan Adegbite, Pooneh Memar Ardestani, Maromi Nei

Department of Neurology Faculty Papers

No abstract provided.

Immune Reconstitution And Tolerance-Inducing Therapies As Promising Therapeutic Approaches In Multiple Sclerosis, Alireza Beheshti Maal, Mandana Kazem Arki, Seyed Massood Nabavi, Mostafa Rezaei-Tavirani, Elham Rismani, Massoud Vosough, Gholamreza Azizi, Nikoo Hossein-Khannazer

Department of Neurology Faculty Papers

OBJECTIVES: Multiple sclerosis (MS) is a complex, progressive neurodegenerative autoimmune disease and a major cause of neurological disability worldwide. MS affects approximately 2.8-3 million people, predominantly presenting as relapsing-remitting MS (RRMS) that frequently converts to secondary progressive disease, while effective options for progressive phenotypes remain limited.

METHODS: This review reframes MS immunotherapy through a tolerance-centric paradigm, distinguishing continuous maintenance disease-modifying therapies (DMTs) from immune reconstitution therapies (IRTs) and emerging antigen-specific tolerance strategies.

RESULTS: Classical immune reconstitution therapies, including alemtuzumab, cladribine, and autologous hematopoietic stem cell transplantation (aHSCT), have demonstrated durable disease control and prolonged periods of no evidence of disease …

Selective Loss Of Primary Cilia And Neurotrophic Signaling In G51d Α-Synuclein Mice Highlights A Common Pathway To Parkinson’S Disease, Yu-En Lin, Ebsy Jaimon, Youngdoo Kim, Annabeth Loftman, Aaran Vijayakumaran, Benjamin D W Belfort, Claire Y Chiang, Benjamin R Arenkiel, Huda Y Zoghbi, Suzanne R Pfeffer

Duncan NRI Faculty and Staff Publications

Parkinson's disease is characterized by dopaminergic neuron loss and accumulation of α-synuclein aggregates in the brain. G51D α-synuclein knock-in mice provide a genetically and clinically relevant model of disease, exhibiting early olfactory deficits, age-dependent motor impairment, and progressive phospho-α-synuclein accumulation. In multiple Parkinson's disease models, striatal cholinergic and parvalbumin interneurons, as well as astrocytes, lose primary cilia and the neurotrophic signaling needed to sustain dopaminergic neurons. We show here that G51D α-synuclein mice share these phenotypes. Phospho-Ser129 α-synuclein accumulation correlates with cilia loss in cholinergic interneurons but not in spiny projection neurons that accumulate higher phospho-α-synuclein levels. In the piriform …

Lineage Tracing Reveals A Shared Cellular Origin For Supraclavicular Brown And Inguinal Beige Adipocytes, Yali Ran, Kai Zhang, Yi-Ting Shen, Qianxing Mo, Ziyi Wang, Hari Krishna Yalamanchili, Sharon John, Mari Kogiso, Xia Gao, Chunmei Wang, Tanvi Sinha, Brian L Black, Miao-Hsueh Chen

Duncan NRI Faculty and Staff Publications

The metabolic importance of brown adipose tissue (BAT) has been recognized, but its origins, particularly supraclavicular BAT (scBAT), remain unclear. Here, we traced scBAT to Mef2c-anterior heart field (AHF)-marked cells. Mef2c-AHF-marked cells isolated from scBAT can spontaneously differentiate into brown adipocytes, express mesenchymal stem cell markers, and can be isolated from the stromal-vascular fraction (SVF) of wild-type scBAT as [CD31

Digital Markers For Passive Remote Monitoring Of Bipolar Disorder: Systematic Review, Thomas P Kutcher, Isha Chakraborty, Kristin Kostick-Quenet, Akane Sano, Nidal Moukaddam, Jeffrey A Herron, Wayne K Goodman, Sameer A Sheth, Ashutosh Sabharwal, Nicole R Provenza

Duncan NRI Faculty and Staff Publications

Background: Bipolar disorder (BD) features episodic shifts among mania, hypomania, depression, mixed states, and euthymia. Timely detection of mood transitions is difficult due to infrequent clinical touchpoints. Digital health technologies, including wearables and smartphones, offer a unique opportunity to passively and continuously monitor behavior and physiology that could reflect underlying mood dynamics in real-world settings.

Objective: This study aimed to systematically review passively collected digital markers for BD mood states, characterize devices/modalities and analytic approaches, appraise risk of bias, and identify design gaps and priorities for clinical translation.

Methods: Following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) …

A Rare Sight: Isolated Third Nerve Palsy As The Sole Presentation Of A Non-Functional Pituitary Adenoma, Jorge A. Aboytes, Jose J. Loayza, Ivan Mogollon, Ismael Cantu

Research Colloquium

Introduction: Hypopituitarism results from insufficient pituitary hormone production, often due to adenomas—accounting for ~15% of intracranial tumors. Non-functional adenomas, typically affecting older adults, may present with visual field deficits, headaches, or cranial nerve palsies when extending into the cavernous sinus. Isolated oculomotor (third nerve) palsy is a rare initial manifestation.

Case: A 46-year-old man with uncontrolled type 2 diabetes presented with acute right-sided headache, diplopia, ptosis, and right leg weakness. He had right ptosis, a fixed dilated pupil, facial droop, and right leg weakness. Given concern for stroke, he received thrombolytic therapy with partial leg improvement, but persistent diplopia and …

Th17 Effector Cytokines Induce Shared And Distinct Microglial And Endothelial Cell Responses In A Mouse Model For Post-Streptococcal Encephalitis, Charlotte Wayne, Uğur Akcan, Travis Faust, Violeta Durán-Laforet, Danny Jamoul, Luca Bremner, Nicole Ampatey, Büşra Akcan, Sarah Ho, Bogoljub Ciric, Shannon Delaney, Wendy Vargas, Susan Swedo, Vilas Menon, Dorothy Schafer, Tyler Cutforth, Dritan Agalliu

Department of Neurology Faculty Papers

Group A Streptococcus (GAS) infections cause neuropsychiatric complications in children, but the mechanisms linking peripheral infection to brain dysfunction remain unclear. Using mouse genetics, single-cell RNA sequencing, and spatial transcriptomics, we show that GAS infections induce inflammatory transcriptional programs in microglia and brain endothelial cells (BECs), accompanied by loss of blood-brain barrier (BBB) gene expression in female mice. Spatial transcriptomic analyses reveal that GAS-responsive microglia localize near infiltrating CD4+ T cells. Several microglial chemokines induced in mice are elevated in sera from affected patients. Deletion of GM-CSF in CD4⁺ T cells partially reduces microglial chemokine gene expression, without restoring BBB …