Neurology Commons^™

Case Report: Pafah1b1 Mutation And Posterior Band Heterotopia With Focal Temporal Lobe Epilepsy Treated By Responsive Neurostimulation, Frank G. Gilliam, Paddy Ssentongo, Michael Sather, Yuka I. Kawasawa

School of Medicine Publications

Subcortical band heterotopia (SBH), also known as double cortex syndrome, is a malformation of cortical development caused by inherited or somatic gene variants. We present a case of a young adult with posterior SBH and electroclinical features of focal neocortical temporal lobe epilepsy. Genomic blood analysis identified a pathogenic somatic mosaicism duplication variant of the PAFAH1B1 gene. Despite bilateral cortical MRI abnormalities, the interictal and ictal EEG findings indicated a focal epileptogenic region in the left posterior temporal region. Chronic responsive cortical neurostimulation across two four-contact depth electrodes placed 5mm on either side of the maximal interictal spiking identified during …

Frontotemporal Dementias In Latin America: History, Epidemiology, Genetics, And Clinical Research, Jorge J. Llibre-Guerra, Maria Isabel Behrens, Mirna Lie Hosogi, Lucia Montero, Teresa Torralva, Nilton Custodio, Erika Mariana Longoria-Ibarrola, Margarita Giraldo-Chica, David Aguillón, Gladys E. Maestre

School of Medicine Publications

Introduction: The historical development, frequency, and impact of frontotemporal dementia (FTD) are less clear in Latin America than in high-income countries. Although there is a growing number of dementia studies in Latin America, little is known collectively about FTD prevalence studies by country, clinical heterogeneity, risk factors, and genetics in Latin American countries.

Methods: A systematic review was completed, aimed at identifying the frequency, clinical heterogeneity, and genetics studies of FTD in Latin American populations. The search strategies used a combination of standardized terms for FTD and related disorders. In addition, at least one author per Latin American country summarized …

Serotonergic Therapy In Epilepsy, Frank Gilliam, Hrvoje Hećimović, Matthew S. Gentry

School of Medicine Publications

Purpose of review

The serotonergic system is implicated in multiple aspects of epilepsy, including seizure susceptibility, sudden unexpected death in epilepsy (SUDEP), and comorbid depression. Despite the complexity of serotonin’s effects on various neuronal networks, ongoing research provides considerable insight into the role of serotonin in human epilepsy. This review explores the potential roles of serotonergic therapies to improve clinical outcomes in epilepsy.

Recent findings

In recent decades research has markedly increased our knowledge of the diverse effects of serotonin on brain function. Animal models of epilepsy have identified the influence of serotonin on seizure threshold in specific brain regions, …

Neurodegeneration Difference Between The Non-Lesioned And Lesioned Hemisphere In Stroke Patient, N. Cortez, Elle Wynn, Kelsey Baker

School of Medicine Publications

Stroke is the 4th leading cause of death in Hispanics. It has generally been suggested that neurodegeneration is isolated to the lesioned hemisphere. However, recent evidence has suggested that the non-lesioned side of the brain may also undergo neurodegeneration. Here, we sought to evaluate the amount of neurodegeneration in the lesioned and non-lesioned hemisphere in chronic stroke patients and identify the impact of stroke size and location

Treating Ms After Surviving Pml: Discrete Strategies For Rescue, Remission, And Recovery Patient 1: From The National Multiple Sclerosis Society Case Conference Proceedings, Nidhiben Anadani, Megan Hyland, Roberto A. Cruz, Robert Lisak, Kathleen Costello, Eugene O. Major, Yasir Jassam, Ethan Meltzer, Thomas C. Varkey, Matthew S. Parsons

School of Medicine Publications

A 38-year-old woman with MS receiving natalizumab presented to the neurology clinic with the complaint of a new neurologic symptom.

Mitigating Alemtuzumab-Associated Autoimmunity In Ms: A "Whack-A-Mole" B-Cell Depletion Strategy, Ethan Meltzer, Sarah Campbell, Benjamin Ehrenfeld, Roberto A. Cruz, Lawrence Steinman, Matthew S. Parsons, Scott S. Zamvil, Elliot M. Frohman, Teresa C. Frohman

School of Medicine Publications

Objective: To determine whether the punctuated administration of low-dose rituximab, temporally linked to B-cell hyperrepopulation (defined when the return of CD19+ B cells approximates 40%-50% of baseline levels as measured before alemtuzumab treatment inception), can mitigate alemtuzumab-associated secondary autoimmunity.

Methods: In this hypothesis-driven pilot study, 10 patients received low-dose rituximab (50-150 mg/m2), a chimeric anti-CD20 monoclonal antibody, after either their first or second cycles of alemtuzumab. These patients were then routinely assessed for the development of autoimmune disorders and safety signals related to the use of dual monoclonal antibody therapy.

Results: Five patients received at least 1 IV infusion of …

Part Ii. High-Dose Methotrexate With Leucovorin Rescue For Severe Covid-19: An Immune Stabilization Strategy For Sars-Cov-2 Induced 'Panic' Attack, Elliot M. Frohman, Nicole R. Villemarette-Pittman, Roberto A. Cruz, Reid Longmuir, Vernon Rowe, Elizabeth S. Rowe, Thomas C. Varkey, Lawrence Steinman, Scott S. Zamvil, Teresa C. Frohman

School of Medicine Publications

Here, in Part II of a duology on the characterization and potential treatment for COVID-19, we characterize the application of an innovative treatment regimen for the prevention of the transition from mild to severe COVID-19, as well as detail an intensive immunotherapy intervention hypothesis. We propose as a putative randomized controlled trial that high-dose methotrexate with leucovorin (HDMTX-LR) rescue can abolish 'PANIC', thereby 'left-shifting' severe COVID-19 patients to the group majority of those infected with SARS-CoV-2, who are designated as having mild, even asymptomatic, disease. HDMTX-LR is endowed with broadly pleiotropic properties and is a repurposed, generic, inexpensive, and widely …

Ssa Positivity As Predictor Of Relapse In Patients With Neuromyelitis Optica Spectrum Disorder, Roberto A. Cruz, Alexander B. Ramos, Bridget A. Bagert, Jesus F. Lovera

School of Medicine Publications

No abstract provided.

Dexamethasone As Abortive Treatment For Refractory Seizures Or Status Epilepticus In The Inpatient Setting, Alexander B. Ramos, Roberto A. Cruz, Nicole R. Villemarette-Pittman, Piotr W. Olejniczak, Edward C. Mader Jr.

School of Medicine Publications

Refractory seizures or status epilepticus (RS/SE) continues to be a challenge in the inpatient setting. Failure to abort a seizure with antiepileptic drugs (AEDs) may lead to intubation and treatment with general anesthesia exposing patients to complications, extending hospitalization, and increasing the cost of care. Studies have shown a key role of inflammatory mediators in seizure generation and termination. We describe 4 patients with RS/SE that was aborted when dexamethasone was added to conventional AEDs: a 61-year-old female with temporal lobe epilepsy who presented with delirium, nonconvulsive status epilepticus, and oculomyoclonic status; a 56-year-old female with history of traumatic left …

Association Of Office And Ambulatory Blood Pressure With Mortality And Cardiovascular Outcomes, Wen-Yi Yang, Jesus D. Melgarejo, Lutgarde Thijs, Zhen-Yu Zhang, José Boggia, Fang-Fei Wei, Tine W. Hansen, Kei Asayama, Gladys E. Maestre, Takayoshi Ohkubo

School of Medicine Publications

Importance Blood pressure (BP) is a known risk factor for overall mortality and cardiovascular (CV)-specific fatal and nonfatal outcomes. It is uncertain which BP index is most strongly associated with these outcomes.

Objective To evaluate the association of BP indexes with death and a composite CV event.

Design, Setting, and Participants Longitudinal population-based cohort study of 11 135 adults from Europe, Asia, and South America with baseline observations collected from May 1988 to May 2010 (last follow-ups, August 2006-October 2016).

Exposures Blood pressure measured by an observer or an automated office machine; measured for 24 hours, during the day or …

Incidence Of Dementia In Elderly Latin Americans: Results Of The Maracaibo Aging Study, Gladys E. Maestre, Luis J. Mena, Jesus D. Melgarejo, Daniel C. Aguirre-Acevedo, Gloria Pino-Ramírez, Milady Urribarrí, Inara J. Chacon, Carlos A. Chavez, Luis Falque-Madrid, Ciro A. Gaona

School of Medicine Publications

Introduction—There are few longitudinal studies of dementia in developing countries. We used longitudinal data from the Maracaibo Aging Study (MAS) to accurately determine the age- and sex-specific incidence of dementia in elderly Latin Americans.

Methods—The DSM IV-R was used to diagnose dementia, which was classified as Alzheimer’s disease (AD), vascular dementia (VaD), or other. Age- and sex-specific incidence was estimated as the number of new cases of dementia divided by person-years of follow-up (p-y).

Results—The incidence of all dementia diagnoses was 9.10 per 1000 p-y (95% CI 7.13–11.44; 8026 total p-y), 5.18 for AD (95% CI 3.72–7.03; …