Neurology Commons^™

Psychiatric Manifestations Of Dengue Fever- A Case Report From Pakistan, Ifrah Hambal, Samrah Nasir, Tania Nadeem

Pakistan Journal of Neurological Sciences (PJNS)

A 29-year-old male presented to Aga Khan University Hospital with complaints of fever, body-aches, vomiting and one episode of gum bleeding. A Dengue antigen test done prior to presentation was positive. He was admitted for medical management. During hospital stay, the patient developed manic symptoms including over-talkativeness, expansive mood, disinhibited behavior, excessive planning, paranoia and aggression. He remained oriented to time, place and person and neurological examination was unremarkable. He was managed with anti-psychotics and benzodiazepines, and his psychiatric symptoms improved with improvement in his medical condition. This case highlights the possibility of psychiatric manifestations occurring in context of a …

Sars-Cov-2 Infection With Polymyositis- A Case Report, Muhammad Hassan, Fatima Mustafa, Naveed Ullah Khan, Taimoor Hassan, Mansoor Iqbal Chaudhry, Mazhar Badshah

Pakistan Journal of Neurological Sciences (PJNS)

Among the most prevalent and least addressed symptoms of SARS-CoV-2 infection are myalgia, muscle weakness, and body aches. Only a few studies have reported polymyositis in patients with Covid-19 infection. At least one-third of patients with Covid-19 syndrome had elevated CPK levels. The cause of elevated CPK can be a cytokine storm or viral infection of the muscles. We present a case in which our patient developed features of myositis following the resolution of an acute viral prodrome caused by SARS-CoV-2. Our patient had both radiological and biopsy-proven neuromuscular manifestations of SARS-CoV-2 associated with polymyositis. Proximal muscle weakness began shortly …

48 Hours Holter Monitoring In Detecting Occult Atrial Fibrillation In Young Patients With Possible Cardioembolic Stroke, Bushra Khalid, Soban Khan, Muhammad Tariq, Sajid Ali, Ijaz Ali, Zaid Waqar

Pakistan Journal of Neurological Sciences (PJNS)

ABSTRACT

Background and objective: Atrial fibrillation (AF) is a common cause of ischemic stroke. AF is usually paroxysmal and could escape detection via single lead electrocardiogram ECG recorder. Holter monitoring is useful in this scenario. The objective of this study was to assess the role of 48 hours Holter monitoring in identifying clinically inapparent AF in young patients with ischemic stroke

Methods: A retrospective cross-sectional study was conducted with patient data from November 2019 to February 2021. It was conducted in Neurology department of Pakistan Institute of Medical Sciences, Islamabad, Pakistan. Study contained medical records of 60 patients which were …

Short, Medium And Long Term Neurological Manifestations And Outcome In Covid-19 Patients, Sajid Hameed, Bashir Soomro, Samar Iltaf, Abdul Malik, Anjum Farooq, Alam Ibrahim Siddiqi, Safia Awan, Mohammad Wasay

Pakistan Journal of Neurological Sciences (PJNS)

Background and objective: There is a need to improve the understanding of Covid-19 neurological complications in a temporal manner. The objective of our study was to find out temporal relationship of neurological manifestations and outcome in Covid-19 Patients.

Methods: This was a multi-center observational study from six centers in Pakistan. Data of covid patients with six months follow up was retrospectively collected from the hospital records. Time periods were divided into short-term (< 1 month), medium-term (2-3 months) and long-term (>4 months). SPSS version 26.0 (SPSS Inc., Chicago, IL, USA) was used to analyze the data.

Results: Neurological manifestations were reported in 208 out of 905 covid …

Decompressive Hemicraniectomy For Patients With Malignant Cerebral Venous Sinus Thrombosis, Sadaf Majid, Waseem Tariq Malik, Asfandyar Khan Niazi, Ehsan Ul Haq, Hina Yusuf, Raja Farhat Shoaib, Maimoona Siddiqui Siddiqui

Pakistan Journal of Neurological Sciences (PJNS)

Background and objective: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke and may lead to the development of raised intracranial pressure due to edema, infarct or bleed. These patients may require decompressive hemicraniectomy to reduce the raised intracranial pressure. However, the evidence for hemicraniectomy in these patients is scanty. Our objective was to perform a retrospective chart review to describe the clinical characteristics of patients who underwent decompressive hemicraniectomy for malignant CVST and their outcomes.

Methods: We retrospectively analyzed the medical records of patients with CVST who underwent decompressive hemicraniectomy between 2013 and 2020 at Shifa International …

Development Of Myelinating Glia: An Overview, Carlo D Cristobal, Hyun Kyoung Lee

Duncan NRI Faculty and Staff Publications

Myelin is essential to nervous system function, playing roles in saltatory conduction and trophic support. Oligodendrocytes (OLs) and Schwann cells (SCs) form myelin in the central and peripheral nervous systems respectively and follow different developmental paths. OLs are neural stem-cell derived and follow an intrinsic developmental program resulting in a largely irreversible differentiation state. During embryonic development, OL precursor cells (OPCs) are produced in distinct waves originating from different locations in the central nervous system, with a subset developing into myelinating OLs. OPCs remain evenly distributed throughout life, providing a population of responsive, multifunctional cells with the capacity to remyelinate …

Massive Macroglossia, A Rare Side Effect Of Covid-19: Clinical, Histologic, And Genomic Findings In Covid-19-Positive Versus Covid-19-Negative Patients, Victoria A Mañón, David Chubb, Laura S Farach, Rachid Karam, Mary C Farach-Carson, Karan Saluja, Mark Wong, James C Melville

Faculty, Staff and Student Publications

PURPOSE: The primary purpose of this study is to identify if there is an underlying genetic predisposition for COVID-related macroglossia and if this susceptibility is higher among individuals of African heritage. Secondary objectives include determining if genetic testing of COVID-infected patients who are intubated and prone could identify patients with higher susceptibility to the development of macroglossia.

METHODS: A retrospective chart review was completed for each patient, and prospectively, genetic and histopathologic analyses were completed. Whole-exome sequencing was completed on two patients; immunohistochemistry was completed on the COVID-positive tissue samples.

RESULTS: Histopathology of the COVID-positive patient revealed significant peri-lymphocytic infiltrate, …

Extensive Accumulation Of Misfolded Protein Aggregates During Natural Aging And Senescence, Enrique Armijo

Faculty, Staff and Student Publications

Accumulation of misfolded protein aggregates is a hallmark event in many age-related protein misfolding disorders, including some of the most prevalent and insidious neurodegenerative diseases. Misfolded protein aggregates produce progressive cell damage, organ dysfunction, and clinical changes, which are common also in natural aging. Thus, we hypothesized that aging is associated to the widespread and progressive misfolding and aggregation of many proteins in various tissues. In this study, we analyzed whether proteins misfold, aggregate, and accumulate during normal aging in three different biological systems, namely senescent cells,

Endovascular Repair Of Intercostal Patch Aneurysms In A Patient With Loyes-Dietz Syndrome, Thanila A Macedo

Faculty, Staff and Student Publications

No abstract provided.

A Substring Replacement Approach For Identifying Missing Is-A Relations In Snomed Ct

Faculty, Staff and Student Publications

Biomedical ontologies provide formalized information and knowledge in the biomedical domain. Over the years, biomedical ontologies have played an important role in facilitating biomedical research and applications. Common quality issues of biomedical ontologies include inconsistent naming of concepts, redundant concepts, redundant relations, incomplete/incorrect concept definitions, and incomplete/incorrect class hierarchies. In this work, we focus on addressing the incompleteness of the class hierarchy in SNOMED CT. We develop a substring replacement approach, leveraging concepts’ lexical features and existing IS-A relations to identify potential missing IS-A relations in SNOMED CT. To evaluate the effectiveness of our approach, we performed both automated and …

Predictors For Outcomes Of Noninvasive, Individualized Transcranial Magnetic Neuromodulation In Multiple Sclerosis Women With Neurogenic Voiding Dysfunction, Darshil Choksi

Faculty, Staff and Student Publications

Purpose:

Multiple sclerosis (MS) is a multifocal demyelinating disease that affects the central nervous system (CNS) and commonly leads to neurogenic lower urinary tract dysfunction (NLUTD). Proper storage and release of urine relies on synchronized activity of the LUT, which is meticulously regulated by supraspinal circuits, making it vulnerable to diseases such as MS. NLUTD, characterized by voiding dysfunction (VD), storage issues, or a combination of both is a common occurrence in MS. Unfortunately, there are limited treatment options for NLUTD, making the search for alternative treatments such as transcranial rotating permanent magnet stimulation (TRPMS) of utmost importance. To assess …

Neurobehavioral Deficits And A Progressive Ictogenesis In The Tetrodotoxin Model Of Epileptic Spasms, John T Le, Carlos J Ballester-Rosado, James D Frost, John W Swann

Duncan NRI Faculty and Staff Publications

Objective: Our goal was to determine whether animals with a history of epileptic spasms have learning and memory deficits. We also used continuous (24/7) long-term electroencephalographic (EEG) recordings to evaluate the evolution of epileptiform activity in the same animals over time.

Methods: Object recognition memory and object location memory tests were undertaken, as well as a matching to place water maze test that evaluated working memory. A retrospective analysis was undertaken of long-term video/EEG recordings from rats with epileptic spasms. The frequency and duration of the ictal events of spasms were quantified.

Results: Rats with a history of epileptic spasms …

Inpatient Teleneurology Follow-Up Has Comparable Outcomes To In-Person Neurology Follow-Up, Michele M Joseph, Tzu-Ching Wu, Amanda Jagolino-Cole

Faculty, Staff and Student Publications

Background and Objectives

Community emergency departments often transfer patients for lack of neurology coverage, potentially burdening patients and accepting facilities. Telestroke improves access to acute stroke care, but there is a lack of data on inpatient teleneurology and telestroke care.

Methods

From our prospective telestroke registry, we retrospectively reviewed 3702 consecutive patients who were seen via telestroke between September 2015 and December 2018. Patients who required transfer after initial telestroke evaluation or who were kept at hospitals without consistent neurology coverage were excluded from analysis. We compared baseline demographics, clinical characteristics, and hospital outcomes in patients who were subsequently followed …

Amplification Of The Plag-Family Genes-Plagl1 And Plagl2-Is A Key Feature Of The Novel Tumor Type Cns Embryonal Tumor With Plagl Amplification, Michaela-Kristina Keck, Martin Sill, Andrea Wittmann, Piyush Joshi, Damian Stichel, Pengbo Beck, Konstantin Okonechnikow, Philipp Sievers, Annika K. Wefers, Nasir Uddin

Department of Pathology and Laboratory Medicine

Pediatric central nervous system (CNS) tumors represent the most common cause of cancer-related death in children aged 0-14 years. They differ from their adult counterparts, showing extensive clinical and molecular heterogeneity as well as a challenging histopathological spectrum that often impairs accurate diagnosis. Here, we use DNA methylation-based CNS tumor classification in combination with copy number, RNA-seq, and ChIP-seq analysis to characterize a newly identified CNS tumor type. In addition, we report histology, patient characteristics, and survival data in this tumor type. We describe a biologically distinct pediatric CNS tumor type (n = 31 cases) that is characterized by focal …

Notch Missense Mutations In Drosophila Reveal Functions Of Specific Egf-Like Repeats In Notch Folding, Trafficking, And Signaling, Hilman Nurmahdi, Mao Hasegawa, Elzava Yuslimatin Mujizah, Takeshi Sasamura, Mikiko Inaki, Shinya Yamamoto, Tomoko Yamakawa, Kenji Matsuno

Duncan NRI Faculty and Staff Publications

Notch signaling plays various roles in cell-fate specification through direct cell–cell interactions. Notch receptors are evolutionarily conserved transmembrane proteins with multiple epidermal growth factor (EGF)-like repeats. Drosophila Notch has 36 EGF-like repeats, and while some play a role in Notch signaling, the specific functions of most remain unclear. To investigate the role of each EGF-like repeat, we used 19 previously identified missense mutations of Notch with unique amino acid substitutions in various EGF-like repeats and a transmembrane domain; 17 of these were identified through a single genetic screen. We assessed these mutants’ phenotypes in the nervous system and hindgut during …

Impact Of Solid State Roadway Lighting On Melatonin In Humans, Ronald B Gibbons, Rajaram Bhagavathula, Benjamin Warfield, George Brainard, John P Hanifin

Department of Neurology Faculty Papers

Introduction: In 2009, the World Health Organization identified vehicle crashes, both injury-related and fatal, as a public health hazard. Roadway lighting has long been used to reduce crashes and improve the safety of all road users. Ocular light exposure at night can suppress melatonin levels in humans. At sufficient light levels, all visible light wavelengths can elicit this response, but melatonin suppression is maximally sensitive to visible short wavelength light. With the conversion of roadway lighting to solid state sources that have a greater short wavelength spectrum than traditional sources, there is a potential negative health impact through suppressed melatonin …

Influence Of Data Sampling Methods On The Representation Of Neural Spiking Activity In Vivo, Meike E Van Der Heijden, Amanda M Brown, Roy V Sillitoe

Duncan NRI Faculty and Staff Publications

No abstract provided.

Improving Translatability Of Spinal Cord Injury Research By Including Age As A Demographic Variable, Andrew N. Stewart, Linda A. T. Jones, John C. Gensel

Department of Physical Therapy Faculty Papers

Pre-clinical and clinical spinal cord injury (SCI) studies differ in study design, particularly in the demographic characteristics of the chosen population. In clinical study design, criteria such as such as motor scores, neurological level, and severity of injury are often key determinants for participant inclusion. Further, demographic variables in clinical trials often include individuals from a wide age range and typically include both sexes, albeit historically most cases of SCI occur in males. In contrast, pre-clinical SCI models predominately utilize young adult rodents and typically use only females. While it is often not feasible to power SCI clinical trials to …

Delta Oscillation Coupled Propagating Fast Ripples Precede Epileptiform Discharges In Patients With Focal Epilepsy, Shennan A. Weiss, Laurent Sheybani, Nitish Seenarine, Itzhak Fried, Chengyuan Wu, Ashwini Sharan, Jerome Engel Jr., Michael R. Sperling, Yuval Nir, Richard J. Staba

Department of Neurology Faculty Papers

Epileptiform spikes are used to localize epileptogenic brain tissue. The mechanisms that spontaneously trigger epileptiform discharges are not yet elucidated. Pathological fast ripple (FR, 200–600 Hz) are biomarkers of epileptogenic brain, and we postulated that FR network interactions are involved in generating epileptiform spikes. Using macroelectrode stereo intracranial EEG (iEEG) recordings from a cohort of 46 patients we found that, in the seizure onset zone (SOZ), propagating FR were more often followed by an epileptiform spike, as compared with non-propagating FR (p < 0.05). Propagating FR had a distinct frequency and larger power (p < 1e-10) and were more strongly phase coupled to the peak of iEEG delta oscillation, which likely correspond with the DOWN states during non-REM sleep (p < 1e-8), than non-propagating FR. While FR propagation was rare, all FR occurred with the highest probability within +/− 400 msec of epileptiform spikes with superimposed high-frequency oscillations (p < 0.05). Thus, a sub-population of epileptiform spikes in the SOZ, are preceded by propagating FR that are coordinated by the DOWN state during non-REM sleep.

Study Design And Baseline Characteristics For The Reflect Gene Therapy Trial Ofm.11778g>A/Nd4-Lhon, Prem S. Subramanian, Nancy J. Newman, Mark Moster, An-Guor Wang, Patrick Yu-Wai-Man, Sean Donahue, Bart P. Leroy, Valerio Carelli, Valerie Biousse, Catherine Vignal-Clermont, Robert C. Sergott, Alfredo A. Sadun, Gema Rebolleda, Bart K. Chwalisz, Rudrani Banik, Fabienne Bazin, Eric Cox, Michel Roux, Magali Taiel, Jose-Alain Sahel

Wills Eye Hospital Papers

Objective REFLECT is the first randomised, double-masked, placebo-controlled multicentre phase 3 clinical trial that evaluated the efficacy and safety of bilateral intravitreal (IVT) injection of lenadogene nolparvovec in subjects with Leber hereditary optic neuropathy carrying the m.11778G>A mutation.

Methods and analysis A total of 98 subjects were enrolled with vision loss of ≤12 months. The subjects were randomised to one of two treatment arms with all subjects receiving an intravitreal (IVT) injection of lenadogene nolparvovec in their first affected eye and the second-affected eye randomised to receive IVT of either lenadogene nolparvovec or placebo.

Results The majority of subjects …