Neurology Commons^™

Development And Validation Of Juvenile Criminal Thinking Styles’ Inventory, Fatima Sana, Muhammad Rafiq

Pakistan Journal of Neurological Sciences (PJNS)

The current research explored the styles of criminal thinking among juvenile delinquents in Pakistani cultural context. Initially, 40 juvenile delinquents were interviewed individually to generate an item pool of 34 distinct thought statements. After excluding repetitive items a list of 19 items was piloted on 30 juvenile delinquents as a self-report measure of 5-point rating scale (Juvenile Criminal Thinking Styles’ Inventory). Finally, a sample of 211 juvenile delinquents were given the final list of 19 items, Measure of Criminal Social Identity (Boduszek, Adamson, Shevlin, & Hyland, 2012; Shagufta, 2015), and a demographic form. Principal Component factor analysis determined a three …

Msto1 Mutations Cause Mtdna Depletion, Manifesting As Muscular Dystrophy With Cerebellar Involvement., S Donkervoort, R Sabouny, P Yun, L Gauquelin, K R Chao, Y Hu, I Al Khatib, A Töpf, P Mohassel, B B Cummings, R Kaur, D Saade, S A Moore, L B Waddell, M A Farrar, J K Goodrich, P Uapinyoying, S H S Chan, A Javed, M E Leach, P Karachunski, J Dalton, L Medne, A Harper, C Thompson, Isabelle Thiffault, S Specht, R E Lamont, Carol J. Saunders, H Racher, F P Bernier, D Mowat, N Witting, J Vissing, R Hanson, Keith A. Coffman, Meagan K. Hainlen, J S Parboosingh, A Carnevale, G Yoon, R E Schnur, Care4rare Canada Consortium, K M Boycott, J K Mah, V Straub, A Reghan Foley, A M Innes, C G Bönnemann, T E Shutt

Manuscripts, Articles, Book Chapters and Other Papers

MSTO1 encodes a cytosolic mitochondrial fusion protein, misato homolog 1 or MSTO1. While the full genotype-phenotype spectrum remains to be explored, pathogenic variants in MSTO1 have recently been reported in a small number of patients presenting with a phenotype of cerebellar ataxia, congenital muscle involvement with histologic findings ranging from myopathic to dystrophic and pigmentary retinopathy. The proposed underlying pathogenic mechanism of MSTO1-related disease is suggestive of impaired mitochondrial fusion secondary to a loss of function of MSTO1. Disorders of mitochondrial fusion and fission have been shown to also lead to mitochondrial DNA (mtDNA) depletion, linking them to the mtDNA …

Brief History Of Spinal Neurosurgical Societies In The United States: Part 1., Sasha Vaziri, Daniel K. Resnick, Christopher P. Ames, James S. Harrop, Christopher I. Shaffrey, Kern Singh, Justin S. Smith, Daniel J. Hoh

Department of Neurosurgery Faculty Papers

No abstract provided.

Markers Of Bone And Lipid Metabolism With Eslicarbazepine Acetate Monotherapy., Scott Mintzer, Tawnya Constantino, Barry Gidal, Parul Bhargava, Todd Grinnell, David Blum

Department of Neurology Faculty Papers

OBJECTIVE: To evaluate the impact of eslicarbazepine acetate (ESL) monotherapy on markers of bone and lipid metabolism.

METHODS: We conducted a post-hoc analysis of data pooled from two Phase III, dose-blind, conversion-to-ESL (1600 mg and 1200 mg) monotherapy studies in patients with focal seizures. Laboratory measurements included lipids (total cholesterol [TC]; high-density lipoprotein cholesterol [HDL-C]; low-density lipoprotein cholesterol; and triglycerides) and markers of bone metabolism (alkaline phosphatase; 25-hydroxyvitamin D; osteocalcin; and parathyroid hormone [PTH]); measurements were taken at baseline, Week 18, and Month 12, and analyzed according to enzyme-inducing antiepileptic drugs (EIAEDs) use at baseline (+EIAED and -EIAED subgroups).

RESULTS: …

Mr Perfusion Imaging, Techniques And Role In Differentiating Radiation Necrosis And Tumor Recurrence, Raima Zakaria, Fatima Mubarak, Muhammad Shahzad Shamim

Department of Radiology

High grade brain tumours are treated with surgery, chemotherapy and radiation therapy and despite such aggressive treatment, can recur in a short span of time. MRI scan has been the conventional diagnostic modality to diagnose recurrence, although at times it becomes difficult for the neuroradiologists to differentiate between tumour recurrence and radiation necrosis. Herein lies the emergent need to explore the efficacy of functional imaging to assist in this diagnostic challenge. Recent studies have sought to do so with promising implications, which we have attempted to summarize in this review.

Gna11 Brain Somatic Pathogenic Variant In An Individual With Phacomatosis Pigmentovascularis, Joseph M Sliepka, Sarah C Mcgriff, Linda Z Rossetti, Peyman Bizargity, Haley Streff, Yi-Shan Lee, Hongzheng Dai, Satyamaanasa Polubothu, Grace Lee, Vicky Ren, Jill V Hunter, Daniel J Curry, Fernando Scaglia, Adekunle M Adesina, Irfan Ali, Veronica Kinsler, Lindsay C Burrage, Dana Marafi

Faculty, Staff and Students Publications

OBJECTIVE: To describe the findings of histopathology and genotyping studies in affected brain tissue from an individual with phacomatosis pigmentovascularis (PPV).

METHODS: A retrospective chart review of a 2-year 10-month-old male with a clinical diagnosis of PPV cesiomarmorata (or type V) was performed. Clinical features, brain imaging and histopathology findings, and genotyping studies in his affected brain tissue are summarized.

RESULTS: The proband had a clinically severe neurologic phenotype characterized by global developmental delay, generalized hypotonia, and recurrent episodes of cardiac asystole in the setting of status epilepticus. A somatic pathogenic variant in

CONCLUSIONS: The finding of this variant in …

Pediatric Brainstem Gliomas: An Institutional Experience, Altaf Ali Laghari, Mirza Zain Baig, Muhammad Ehsan Bari, Aneela Darbar, Naureen Mushtaq, Umme E Hani Abdullah, Daniya Aziz Khan

Section of Neurosurgery

Objective: The aim of this study was to analyze the clinical profiles and outcomes of pediatric brainstem gliomas treated at our institute.
Methodology: We reviewed the files of 18 pediatric age group patients diagnosed with brainstem glioma at our institution. The following variables were recorded: age, sex, duration of symptoms, date of diagnosis, main clinical symptoms, Karnofsky performance status score, magnetic resonance imaging findings, histopathology findings, details of the treatment given, disease progression, and date of mortality/last follow-up. This data were then transferred to SPSS version 23 which was used for further analysis.
Results: The mean age of our cohort …

Rapidly Recurrent Basilar Artery Thrombosis Treated With Emergent Mechanical Thrombectomies And Stenting, Mark Cohen, Amna Imran, Ankur Garg

Neurology

Acute basilar artery thrombosis (BAT) is a life-threatening condition that can be treated by emergent mechanical thrombectomy (EMT). Recurrent acute BAT is a rare phenomenon. Here we describe a case of rapid recurrent acute BAT that was treated each time by EMT and finally by emergent basilar artery stenting with good outcome

Outcomes In Patients With Gunshot Wounds To The Brain, Leigh Anna Robinson, Lauren M. Turco, Bryce Robinson, Joshua G. Corsa, Michael Mount, Amy V. Hamrick, John Berne, Dalier R. Mederos, Allison G. Mcnickle, Paul J. Chestovich, Jason Weinberger, Areg Grigorian, Jeffry Nahmias, Jane K. Lee, Kevin L. Chow, Erik J. Olson, Jose L. Pascual, Rachele Solomon, Danielle A. Pigneri, Husayn A. Ladhani, Joanne Fraifogl, Terry Curry, Todd W. Costantini, Manasnun Kongwibulwut, Haytham Kaafarani, Janika San Roman, Craig Schreiber, Anna Goldenberg-Sandau, Parker Hu, Patrick Bosarge, Rindi Uhlich

School of Medicine Faculty Research

Introduction: Gunshot wounds to the brain (GSWB) confer high lethality and uncertain recovery. It is unclear which patients benefit from aggressive resuscitation, and furthermore whether patients with GSWB undergoing cardiopulmonary resuscitation (CPR) have potential for survival or organ donation. Therefore, we sought to determine the rates of survival and organ donation, as well as identify factors associated with both outcomes in patients with GSWB undergoing CPR. Methods: We performed a retrospective, multicenter study at 25 US trauma centers including dates between June 1, 2011 and December 31, 2017. Patients were included if they suffered isolated GSWB and required CPR at …

Interleukin 1 Alpha Administration Is Neuroprotective And Neuro-Restorative Following Experimental Ischemic Stroke, Kathleen E. Salmeron, Michael E. Maniskas, Danielle N. Edwards, Raymond Wong, Ivana Rajkovic, Amanda L. Trout, Abir A. Rahman, Samantha Hamilton, Justin F. Fraser, Emmanuel Pinteaux, Gregory J. Bix

Sanders-Brown Center on Aging Faculty Publications

BACKGROUND: Stroke remains a leading cause of death and disability worldwide despite recent treatment breakthroughs. A primary event in stroke pathogenesis is the development of a potent and deleterious local and peripheral inflammatory response regulated by the pro-inflammatory cytokine interleukin-1 (IL-1). While the role of IL-1β (main released isoform) has been well studied in stroke, the role of the IL-1α isoform remains largely unknown. With increasing utilization of intravenous tissue plasminogen activator (t-PA) or thrombectomy to pharmacologically or mechanically remove ischemic stroke causing blood clots, respectively, there is interest in pairing successful cerebrovascular recanalization with neurotherapeutic pharmacological interventions (Fraser et …

Is It Fair To Compare? A Patient And Family Experience Of Two Healthcare Systems And Neurosurgical Teams Within A Two-Week Period, Laura Miller Cpxp

Patient Experience Journal

As the mother of a 28-year-old son with cerebral palsy and hydrocephalus, and as a healthcare consultant focused on patient experience and professional development, I have a unique perspective and skill set. Recently he experienced symptoms that included an excruciating headache, neck pain and lethargy. Fearing his ventriculoperitoneal shunt had malfunctioned, he went to the emergency room and was later admitted on the neuro inpatient floor for a three-day hospitalization. His original shunt had been placed in 1991, and he never had an issue with until August 2018. While in the hospital, he was informed that he was no longer …

Awakening From A Medical Mystery: One Patient’S Experience Of Being Undiagnosed, Dwane Unruh

Patient Experience Journal

This personal narrative pleads for a supportive and comprehensive system or sub-system similar to that which exists for cancer patients, to deal with undiagnosed illnesses. By describing the torment of living with a debilitating illness that medicine could not easily recognize, then by contrasting this experience with my wife’s experience of the cancer care system, and by referring along the way to lessons learned many years ago from reading the works of the inciteful neurologist, Dr. Oliver Sacks, I hope to inspire the medical system to develop a separate, supportive and comprehensive system to deal with the undiagnosed. As it …

Progression-Free But No Overall Survival Benefit For Adult Patients With Bevacizumab Therapy For The Treatment Of Newly Diagnosed Glioblastoma: A Systematic Review And Meta-Analysis., Nagham Kaka, Karim Hafazalla, Haider Samawi, Andrew Simpkin, James Perry, Arjun Sahgal, Sunit Das

Department of Neurosurgery Faculty Papers

Glioblastoma (GBM) is the most common high-grade primary brain tumor in adults. Standard multi-modality treatment of glioblastoma with surgery, temozolomide chemotherapy, and radiation results in transient tumor control but inevitably gives way to disease progression. The need for additional therapeutic avenues for patients with GBM led to interest in anti-angiogenic therapies, and in particular, bevacizumab. We sought to determine the efficacy of bevacizumab as a treatment for newly diagnosed GBM. We conducted a literature search using the PubMed database and Google Scholar to identify randomized controlled trials (RCTs) since 2014 investigating the safety and efficacy of bevacizumab in the treatment …

Pediatric Moyamoya Presenting As A Subarachnoid Hemorrhage From A Ruptured Anterior Cerebral Artery Aneurysm., Mohammad Hassan A. Noureldine, Ibrahim Saikali, Anis Nassif, Rita Chahinian, Ahmad Sweid, Raghid Kikano, Michel Mawad

Department of Neurosurgery Faculty Papers

BACKGROUND: The incidence of Moyamoya disease (MMD)-associated intracranial aneurysms ranges from 3% to 14% in adult patients, whereas this complication has rarely been reported in children.

CASE DESCRIPTION: We herein report the first case, to our knowledge, of an extremely rare subarachnoid hemorrhage presentation of a child with a ruptured anterior cerebral artery dissecting aneurysm secondary to a newly discovered, unilateral Moyamoya-like pathology.

CONCLUSIONS: MMD-associated aneurysms are extremely rare in children, and hemorrhage may be the initial presentation of the disease. Prompt intervention is essential to exclude the ruptured aneurysm that is at risk of rebleeding because of persistent hemodynamic …

Use Of Befasstt Acronym To Improve Brainstem Stroke Recognition - A Case Study, Marietta Sperry, Amber Wilson

Articles, Abstracts, and Reports

Purpose: To increase recognition of lower brain stem strokes through the use of the BeFASSTT acronym to improve patient outcomes.

Biological Intratumoral Therapy For The High-Grade Glioma Part I: Intratumoral Delivery And Immunotoxins., Joshua Loya, Charlie Zhang, Emily J Cox, Achal Singh Achrol, Santosh Kesari

Articles, Abstracts, and Reports

Management of high-grade gliomas remains a complex challenge. Standard of care consists of microsurgical resection, chemotherapy and radiation, but despite these aggressive multimodality therapies the overall prognosis remains poor. A major focus of ongoing translational research studies is to develop novel therapeutic strategies that can maximize tumor cell eradication while minimizing collateral side effects. Particularly, biological intratumoral therapies have been the focus of new translational research efforts due to their inherent potential to be both dynamically adaptive and target specific. This two-part review will provide an overview of biological intratumoral therapies and summarize key advances and remaining challenges in intratumoral …

Biological Intratumoral Therapy For The High-Grade Glioma Part Ii: Vector- And Cell-Based Therapies And Radioimmunotherapy., Joshua Loya, Charlie Zhang, Emily J Cox, Achal Singh Achrol, Santosh Kesari

Articles, Abstracts, and Reports

Management of high-grade gliomas (HGGs) remains a complex challenge with an overall poor prognosis despite aggressive multimodal treatment. New translational research has focused on maximizing tumor cell eradication through improved tumor cell targeting while minimizing collateral systemic side effects. In particular, biological intratumoral therapies have been the focus of novel translational research efforts due to their inherent potential to be both dynamically adaptive and target specific. This two part review will provide an overview of biological intratumoral therapies that have been evaluated in human clinical trials in HGGs, and summarize key advances and remaining challenges in the development of these …

Post-Acquisition Processing Confounds In Brain Volumetric Quantification Of White Matter Hyperintensities, Ahmed A. Bahrani, Omar M. Al-Janabi, Erin L. Abner, Shoshana H. Bardach, Richard J. Kryscio, Donna M. Wilcock, Charles D. Smith, Gregory A. Jicha

Neurology Faculty Publications

BACKGROUND: Disparate research sites using identical or near-identical magnetic resonance imaging (MRI) acquisition techniques often produce results that demonstrate significant variability regarding volumetric quantification of white matter hyperintensities (WMH) in the aging population. The sources of such variability have not previously been fully explored.

NEW METHOD: 3D FLAIR sequences from a group of randomly selected aged subjects were analyzed to identify sources-of-variability in post-acquisition processing that can be problematic when comparing WMH volumetric data across disparate sites. The methods developed focused on standardizing post-acquisition protocol processing methods to develop a protocol with less than 0.5% inter-rater variance.

RESULTS: A series …

Utilization And Outcomes With Low Dose Tissue Plasminogen Activator As Intravenous Thrombolytic Therapy For Ischaemic Stroke At Aga Khan University Hospital, Karachi: A Retrospective Analysis, Masood Uz Zaman Babar, Ammar Zaman Khan, Haris Hakeem, Jaleed Gilani, Mohammad Wasay

Section of Neurology

Objective: To determine the utilisation of intravenous tissue plasminogen activator at a certain dose for ischaemic stroke thrombolysis and to compare the outcomes with those of a different dosage mentioned in literature.
Methods: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised medical records from January, 2007, to October, 2016, of all patients having received intravenous tissue plasminogen activator for ischaemic stroke thrombolysis. Primary safety outcome variables included symptomatic intracerebral haemorrhage after the start of treatment (0.6mg/kg) and death within three months as per the modified Rankin scale 6. Secondary efficacy outcome variable was functional …

Current State Of Knowledge Of Basic Life Support In Health Professionals Of The Largest City In Pakistan: A Cross-Sectional Study, Babar Irfan, Ibrahim Zahid, Muhammad Sharjeel Khan, Omar Abdul Aziz Khan, Shayan Zaidi, Safia Awan, Sobia Bilal, Omar Irfan

Section of Neurology

Background: Basic Life Support (BLS) is the recognition of sudden cardiac arrest and activation of the emergency response system, followed by resuscitation, and rapid defibrillation. According to WHO, Pakistan has one of the highest mortality rates from accidental deaths therefore assessment and comparison of BLS knowledge in health professionals is crucial. We thereby aim to assess and compare the knowledge of BLS in doctors, dentists and nurses.

Methods: A multi-centric cross-sectional survey was conducted in Karachi at different institutions belonging to the private as well as government sector from January to March 2018. We used a structured questionnaire which was …