Neurology Commons^™

Direct Oral Anticoagulant Failure In Stroke/Transient Ischaemic Attack: Neurologic And Pharmacokinetic Considerations, David Z. Rose, W. Scott Burgin

Neurology Faculty Publications

No abstract provided.

Test–Retest Reliability Of The Friedreich’S Ataxia Rating Scale, Christian Rummey, Theresa A. Zesiewicz, Santiago Perez-Lloret, Jennifer M. Farmer, Massimo Pandolfo, David R. Lynch

Neurology Faculty Publications

The modified Friedreich Ataxia Rating Scale (mFARS) is a disease specific, exam-based neurological rating scale commonly used as a outcome measure in clinical trials. While extensive clinimetric testing indicates it’s validity in measuring disease progression, formal test–retest reliability was lacking. To fill this gap, we acquired results from screening and baseline visits of several large clinical trials and calculated intraclass correlation coefficients, coefficients of variance, standard error, and the minimally detectable changes. This study demonstrated excellent test–retest reliability of the mFARS, and it’s upright stability subscore.

Untreated Stroke As Collateral Damage Of Covid-19: “Time Is Brain” Versus “Stay At Home, David Z. Rose, W. Scott Burgin, Swetha Renati

Neurology Faculty Publications

For decades, neurologists have been advocating that anyone with acute focal deficits report immediately to the closest hospital’s emergency room. Major advancements in the hyperacute diagnosis and treatment of stroke have justified our call-to-action slogan of “Time is Brain”—faster therapy leads to superior outcomes. However, this mantra has been recently usurped by the catchphrase “Stay at Home” during the coronavirus disease 2019 (COVID-19) pandemic. Fewer patients are presenting to hospitals with acute stroke; our census is down. Presumably the etiology of this phenomenon is either strict “social distancing” that some people may misperceive to exclude even emergent situations, or fears …

Direct Oral Anticoagulant Failure In Stroke/Transient Ischaemic Attack: Neurologic And Pharmacokinetic Considerations, David Z. Rose, W. Scott Burgin

Neurology Faculty Publications

No abstract provided.

A Case Report Of Extensive Cerebral Venous Sinus Thrombosis As A Presenting Sign Of Relapsing Nephrotic Syndrome, Janet K. Lee, Kathleen Murray, Swetha Renati

Neurology Faculty Publications

Nephrotic syndrome is defined by three characteristic features including proteinuria of >3 g in 24 hours, hypoalbuminemia of less than 3 g/dL, and peripheral edema. Multiple nephropathies can result in nephrotic syndrome. Most commonly, minimal change disease is seen in children under the age of 10, while adults are more commonly found to have membranous nephropathy. Hypercoagulability and thrombotic sequela can be seen in nephrotic syndrome, regardless of underlying etiology, and thrombosis is most commonly seen in deep veins of the lower extremities and renal veins. Our case identifies an adult with previously diagnosed and treated for minimal change disease …

Safety, Pharmacodynamics, And Potential Benefit Of Omaveloxolone In Friedreich Ataxia, David R. Lynch, Jennifer Farmer, Lauren Hauser, Ian A. Blair, Qing Qing Wang, Clementina Mesaros, Nathaniel Snyder, Sylvia Boesch, Melanie Chin, Martin B. Delatycki, Paola Giunti, Angela Goldsberry, Chad Hoyle, Michael G. Mcbride, Wolfgang Nachbauer, Megan O'Grady, Susan Perlman, S. H. Subramony, George R. Wilmot, Theresa A. Zesiewicz, Colin Meyer

Neurology Faculty Publications

Objective Previous studies have demonstrated that suppression of Nrf2 in Friedreich ataxia tissues contributes to excess oxidative stress, mitochondrial dysfunction, and reduced ATP production. Omaveloxolone, an Nrf2 activator and NF-kB suppressor, targets dysfunctional inflammatory, metabolic, and bioenergetic pathways. The dose-ranging portion of this Phase 2 study assessed the safety, pharmacodynamics, and potential benefit of omaveloxolone in Friedreich ataxia patients (NCT02255435).

Methods Sixty-nine Friedreich ataxia patients were randomized 3:1 to either omaveloxolone or placebo administered once daily for 12 weeks. Patients were randomized in cohorts of eight patients, at dose levels of 2.5–300 mg/day.

Results Omaveloxolone was well tolerated, and adverse …

Central Pain Mimicking Trigeminal Neuralgia As A Result Of Lateral Medullary Ischemic Stroke, Abinayaa Ravichandran, Kareem S. Elsayed, Hussam A. Yacoub

Neurology Faculty Publications

Background. Central pain mimicking trigeminal neuralgia (TN) as a result of lateral medullary infarction or Wallenberg syndrome has been rarely reported. Case Report. We discuss a patient who presented with a lateral medullary infarct and shortly after developed facial pain mimicking TN. We also elaborate on the anatomical pathway of the trigeminal nerve explaining facial pain as a result of a lateral medullary lesion. Discussion. Clinicians should be aware of this typical complication of lateral medullary infarct in order to attain proper management and work-up.

Protocol For Arest: Apixaban For Early Prevention Of Recurrent Embolic Stroke And Hemorrhagic Transformation—A Randomized Controlled Trial Of Early Anticoagulation After Acute Ischemic Stroke In Atrial Fibrillation, David Z. Rose, John N. Meriwether, Michael G. Fradley, Swetha Renati, Ryan C. Martin, Thomas Kasprowicz, Aarti Patel, Maxim Mokin, Ryan Murtagh, Kevin Kip, Andrea C. Bozeman, Tara Mctigue, Nicholas Hilker, Bonnie Kirby, Natasha Wick, Nhi Tran, W. Scott Burgin, Arthur J. Labovitz

Neurology Faculty Publications

Background: Optimal timing to initiate anticoagulation after acute ischemic stroke (AIS) from atrial fibrillation (AF) is currently unknown. Compared to other stroke etiologies, AF typically provokes larger infarct volumes and greater concern of hemorrhagic transformation, so seminal randomized trials waited weeks to months to begin anticoagulation after initial stroke. Subsequent data are limited and non-randomized. Guidelines suggest anticoagulation initiation windows between 3 and 14 days post-stroke, with Class IIa recommendations, and level of evidence B in the USA and C in Europe.

Aims: This open-label, parallel-group, multi-center, randomized controlled trial AREST (Apixaban for Early Prevention of Recurrent Embolic Stroke and …

Patient Perceived Changes In Sexual Dysfunction After Initiation Of Natalizumab For Multiple Sclerosis, Derrick Robertson, Angela Aungst, Ryan Collier, Jhulianna Vivar, Natalie Moreo, Lise Casady, Tuan Vu

Neurology Faculty Publications

Purpose Sexual dysfunction is a common but often overlooked secondary symptom of multiple sclerosis (MS) and can be associated with a decreased health-related quality of life (HRQoL). Natalizumab is a disease-modifying therapy approved for the treatment of relapsing forms of MS. In addition to its efficacy, those using natalizumab have shown improvement in HRQoL parameters, including fatigue and cognition. The idea that improvement in fatigue may also correlate with improvement in sexual dysfunction is the impetus for this study.

Methods A single-center, open-label, single-arm, 24-week study was performed to evaluate perceived change in sexual dysfunction in MS patients treated with …

A Novel Design Of A Phase Iii Trial Of Isradipine In Early Parkinson Disease (Steady-Pd Iii), Kevin M. Biglan, David Oakes, Anthony E. Lang, Robert A. Hauser, Karen Hodgeman, Brittany Greco, Jillian Lowell, Rebecca Rockhill, Ira Shoulson, Charles Venuto, Diony Young, Tanya Simuni

Neurology Faculty Publications

Objectives To describe the rationale for a novel study design and baseline characteristics of a disease-modifying trial of isradipine 10 mg daily in early Parkinson disease (PD).

Methods STEADY-PDIII is a 36-month, Phase 3, parallel group, placebo-controlled study of the efficacy of isradipine 10 mg daily in 336 participants with early PD as measured by the change in the Unified Parkinson Disease Rating Scale (UPDRS) Part I-III score in the practically defined ON state. Secondary outcome measures include clinically meaningful measures of disability progression in early PD: (1) Time to initiation and utilization of dopaminergic therapy; (2) Time to onset …

Carotid Endarterectomy In A Patient With Severe Internal Carotid Artery Stenosis With Persistent Trigeminal Artery And Ischemia Of The Anterior And Posterior Circulation, Melanie R. F. Greenway, Hussam A. Yacoub, Shweta Varade, Yevgeniy Isayev

Neurology Faculty Publications

Occurrence of cerebral ischemia in the posterior circulation as a result of severe internal carotid artery disease and persistent trigeminal artery is rare. An 81-year-old man with medical history of hypertension and ischemic stroke presented with dizziness, nausea, and mild dysarthria. Magnetic resonance imaging of the brain revealed acute infarcts in the left internal carotid artery territory. CT angiogram revealed a persistent trigeminal artery (PTA) and severe atherosclerosis. The patient developed new neurological symptoms and repeat imaging revealed new acute infarcts in the PTA distribution. After undergoing a left carotid endarterectomy with no complications, the patient was discharged to a …

Impact Of Diabetes In The Friedreich Ataxia Clinical Outcome Measures Study, Ashley Mccormick, Jennifer Farmer, Susan Perlman, Martin Delatycki, George Wilmot, Katherine Matthews, Grace Yoon, Chad Hoyle, Sub H. Subramony, Theresa Zesiewicz, David R. Lynch, Shana E. Mccormack

Neurology Faculty Publications

Objective Friedreich ataxia (FA) is a progressive neuromuscular disorder caused by GAA triplet repeat expansions or point mutations in the FXN gene. FA is associated with increased risk of diabetes mellitus (DM). This study assessed the age-specific prevalence of FA-associated DM and its impact on neurologic outcomes.

Research Design and Methods Participants were 811 individuals with FA from 12 international sites in a prospective natural history study (FA Clinical Outcome Measures Study, FACOMS). Physical function was assessed, using validated instruments. Multivariable regression analyses examined the independent association of DM with outcomes.

Results Mean age of participants was 30.1 years (SD …

Racial‐Ethnic Disparities In Acute Stroke Care In The Florida‐Puerto Rico Collaboration To Reduce Stroke Disparities Study, Ralph L. Sacco, Hannah Gardener, Kefeng Wang, Chuanhui Dong, Maria A. Ciliberti-Vargas, Carolina M. Gutierrez, Negar Asdaghi, W. Scott Burgin, Olveen Carrasquillo, Enid J. Garcia-Rivera, Ulises Nobo, Sofia Oluwole, David Z. Rose, Michael F. Waters, Juan Carlos Zevallos, Mary Robichaux, Salina P. Waddy, Jose G. Romano, Tatjana Rundek

Neurology Faculty Publications

Background: Racial‐ethnic disparities in acute stroke care can contribute to inequality in stroke outcomes. We examined race‐ethnic disparities in acute stroke performance metrics in a voluntary stroke registry among Florida and Puerto Rico Get With the Guidelines‐Stroke hospitals.

Methods and Results: Seventy‐five sites in the Florida Puerto Rico Stroke Registry (66 Florida and 9 Puerto Rico) recorded 58 864 ischemic stroke cases (2010–2014). Logistic regression models examined racial‐ethnic differences in acute stroke performance measures and defect‐free care (intravenous tissue plasminogen activator treatment, in‐hospital antithrombotic therapy, deep vein thrombosis prophylaxis, discharge antithrombotic therapy, appropriate anticoagulation therapy, statin use, smoking cessation counseling) …

Serotonin Syndrome Complicating Treatment Of Ifosfamide Neurotoxicity With Methylene Blue, Matthew Snyder, Suhas Gangadhara, Andrew S. Brohl, Steven Ludlow, Sowmya Nanjappa

Neurology Faculty Publications

Methylene blue is a widely used treatment for ifosfamide neurotoxicity. We present a case of severe encephalopathy complicating ifosfamide-based therapy for recurrent retroperitoneal leiomyosarcoma. After treatment with methylene blue, the patient experienced clinical decompensation and was diagnosed with serotonin syndrome based on a constellation of clinical findings. Withdrawal of methylene blue and other serotonergic medications led to clinical stabilization and ultimately neurological recovery. Our case highlights the challenge of diagnosing serotonin syndrome in the face of preexisting ifosfamide neurotoxicity, as there is significant clinical overlap between these 2 syndromes. Practitioners must remain vigilant of this potential life-threatening complication in this …

Incidence Of Seizure Exacerbation And Seizures Reported As Adverse Events During Adjunctive Treatment With Eslicarbazepine Acetate: A Pooled Analysis Of Three Phase Iii Controlled Trials, Mar Carreño, Selim Benbadis, Francisco Rocha, David Blum, Hailong Cheng

Neurology Faculty Publications

No abstract provided.

Progression Of Friedreich Ataxia: Quantitative Characterization Over 5 Years, Maya Patel, Charles J. Isaacs, Lauren Seyer, Karlla Brigatti, Sarah Gelbard, Cassandra Strawser, Debbie Foerster, Julianna Shinnick, Kimberly Schadt, Eppie M. Yiu, Martin B. Delatycki, Susan Perlman, George R. Wilmot, Theresa Zesiewicz, Katherine Mathews, Christopher M. Gomez, Grace Yoon, Sub H. Subramony, Alicia Brocht, Jennifer Farmer, David R. Lynch

Neurology Faculty Publications

Objective Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder of adults and children. This study analyzed neurological outcomes and changes to identify predictors of progression and generate power calculations for clinical trials.

Methods Eight hundred and twelve subjects in a natural history study were evaluated annually across 12 sites using the Friedreich Ataxia Rating Scale (FARS), 9-Hole Peg Test, Timed 25-Foot Walk, visual acuity tests, self-reported surveys and disability scales. Cross-sectional outcomes were assessed from recent visits, and longitudinal changes were gaged over 5 years from baseline.

Results Cross-sectional outcomes correlated with measures of disease severity. Age, genetic severity (guanine-adenine-adenine …

Application Of Pre-Participation Cardiovascular Screening Guidelines To Novice Older Runners And Endurance Athletes, Justin R. Abbatemarco, Courtney Bennett, Adrian J. Bell, Laura Dunne, Martin E. Matsumura

Neurology Faculty Publications

Objectives: Despite robust growth in participation in marathons and endurance sports among older individuals, guidance regarding pre-participation cardiovascular evaluation of these athletes is lacking. The objective of this study was to assess the utility of currently available pre-participation cardiovascular evaluation guidelines as applied to a cohort of older novice endurance athletes.

Methods: We applied data from 1457 novice runners and endurance athletes aged 35 years and older to two pre-participation screening tools, the American Heart Association/American College of Sports Medicine Pre-Participation Questionnaire and the 2001 Working Group recommendations for pre-participation screening of masters athletes (2001 Masters).

Results: Application of the …

Unusual Late Onset Of Parenchymal Neuro-Behçet Disease, Wai Wai Miller, Demetrios Konstas, Chetan Gandhy, Derrick Robertson

Neurology Faculty Publications

Neuro-Behçet disease (NBD) is a multisystem inflammatory disorder characterized by oral lesions, genital lesions, uveitis, and neurological deficits. If left untreated, it may lead to worsening neurological function and can be fatal. Here we present a case of a 52-year-old woman who was diagnosed with Behçet disease (BD) as a teenager and had a relatively mild disease course. Decades later after her initial DB diagnosis, she presented to our hospital with a chief complaint of headache. She did not have focal neurological deficits or any active mucosal lesions. Upon further investigation, the patient was found to have multiple inflammatory changes …

Frataxin Levels In Peripheral Tissue In Friedreich Ataxia, Michael Lazaropoulos, Yina Dong, Elisia Clark, Nathaniel R. Greeley, Lauren A. Seyer, Karlla W. Brigatti, Carlton Christie, Susan L. Perlman, George R. Wilmot, Christoper M. Gomez, Katherine D. Mathews, Grace Yoon, Theresa Zesiewicz, Chad Hoyle, Sub H. Subramony, Alicia F. Brocht, Jennifer M. Farmer, Robert B. Wilson, Eric C. Deutsch, David R. Lynch

Neurology Faculty Publications

No abstract provided.

Anti-Nmda Receptor Encephalitis In A Patient With Previous Psychosis And Neurological Abnormalities: A Diagnostic Challenge, R. David Heekin, Maria C. Catalano, Alfred T. Frontera, Glenn Catalano

Neurology Faculty Publications

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune disorder characterized by IgG autoantibodies directed against the NR1 subunit of the NMDA glutamate receptor. Psychiatric symptoms are common and include psychosis, mania, depressed mood, aggression, and speech abnormalities. Neurological symptoms such as seizures, decreased responsiveness, dyskinesias, and other movement abnormalities and/or autonomic instability are frequently seen as well. We present the case of a woman who was followed up at our facility for over 14 years for the treatment of multiple neuropsychiatric symptoms. Initially, she presented with paresthesias, memory loss, and manic symptoms. Nine years later, she presented to our facility again, …

Hippocampal Neurogenesis And The Brain Repair Response To Brief Stereotaxic Insertion Of A Microneedle, Shijie Song, Shuojing Song, Chuanhai Cao, Xiaoyang Lin, Kunyu Li, Vasyl Sava, Juan Sanchez-Ramos

Neurology Faculty Publications

We tested the hypothesis that transient microinjury to the brain elicits cellular and humoral responses that stimulate hippocampal neurogenesis. Brief stereotaxic insertion and removal of a microneedle into the right hippocampus resulted in (a) significantly increased expression of granulocyte-colony stimulating factor (G-CSF), the chemokine MIP-1a, and the proinflammatory cytokine IL12p40; (b) pronounced activation of microglia and astrocytes; and (c) increase in hippocampal neurogenesis. This study describes immediate and early humoral and cellular mechanisms of the brain’s response to microinjury that will be useful for the investigation of potential neuroprotective and deleterious effects of deep brain stimulation in various neuropsychiatric disorders.

Receptor Antagonism And Dyskinesia In Parkinson’S Disease, Micaela Morelli, Fabio Blandini, Nicola Simola, Robert A. Hauser

Neurology Faculty Publications

Dyskinesia, a major complication of treatment of Parkinson’s disease (PD), involves two phases: induction, which is responsible for dyskinesia onset, and expression, which underlies its clinical manifestation. The unique cellular and regional distribution of adenosine receptors in basal ganglia areas that are richly innervated by dopamine, and their antagonistic role towards dopamine receptor stimulation, have positioned receptor antagonists as an attractive nondopaminergic target to improve the motor deficits that characterize PD. In this paper, we describe the biochemical characteristics of receptors and the effects of adenosine antagonists in rodent and primate models of PD on L-DOPA-induced dyskinesia, together with relevant …

A Comparative Analysis Of Structural Brain Mri In The Diagnosis Of Alzheimer’S Disease, Jason Appel, Elizabeth Potter, Qian Shen, Gustavo Pantol, Maria T. Greig, David Loewenstein, Ranjan Duara

Neurology Faculty Publications

Dementia is a debilitating and life-altering disease which leads to both memory impairment and decline of normal executive functioning. While causes of dementia are numerous and varied, the leading cause among patients 60 years and older is Alzheimer’s disease. The gold standard for Alzheimer’s diagnosis remains histological identification of amyloid plaques and neurofibrillary tangles within the medial temporal lobe, more specifically the entorhinal cortex and hippocampus. Although no definitive cure for Alzheimer's disease currently exists, there are treatments targeted at preserving cognition and memory while delaying continued loss of function. Alzheimer's disease exists along a spectrum of cognitive decline and …

Foreign Accent Syndrome Mimicked By Garcin Syndrome With Spontaneous Resolution, Michael Hoffmann

Neurology Faculty Publications

An English speaking women developed a French accent, without any aphasic syndromes, in conjunction with multiple left sided cranial nerve deficits, temporally related to cranial trauma. Extensive testing with multimodality magnetic resonance imaging, cerebrospinal fluid and laboratory analysis was unremarkable. She was followed over a 3 year period during which her French accent resolved as did the majority of her multiple unilateral cranial neuropathies. The neurological diagnoses included a foreign accent syndrome attributed to a reversible Garcin syndrome.

Isolated Right Temporal Lobe Stroke Patients Present With Geschwind Gastaut Syndrome, Frontal Network Syndrome And Delusional Misidentification Syndromes, Michael Hoffmann

Neurology Faculty Publications

Background: Right temporal lobe lesion syndrome elicitation presents a clinical challenge. Aside from occasional covert quadrantanopias, heralding elementary neurological deficits are absent.Aim: Isolated right and left temporal lobe stroke patients were analyzed for the panoply of known temporal and frontal cognitive and neuropsychiatric syndromes.Methods: Temporal lobe stroke patients were analyzed, derived from a dedicated cognitive stroke registry. Patients were screened by a validated bedside cognitive battery and a neuropsychological test battery, including the Bear Fedio Inventory for diagnosis of the Geschwind Gastaut (GG) syndrome, frontal network syndrome testing (FNS), emotional intelligence testing and delusional misidentification syndromes (DMIS). NIH …

Etiology Of Frontal Network Syndromes In Isolated Subtentorial Stroke, Michael Hoffmann, Lourdes Benes Cases

Neurology Faculty Publications

Background: The neurobiology of the frontal network syndrome (FNS) that may occur with isolated subtentorial stroke is unknown.Aim: Evaluate for frontal network syndromes in young people post subtentorial stroke who have recovered neurologically and compare to a stroke lesion group least likely to manifest frontal network syndromes.Methods: Young people (18–49 years) with isolated cerebellar or brainstem subtentorial stroke (ST) that had recovered to independency (Rankin score ࣘ 2) with minimal or no residual neurological deficit (NIHSS ࣘ 4) with neurological recovery enabling resumption of former employment. Comparison was made to age and education matched young people with posterior …