Neurology Commons^™

A Rare Case Of Myasthenia Gravis With Coexisting Muscular Dystrophy, Sommayya Aftab, M Haroon Hamid, Tipu Sultan

Pakistan Journal of Neurological Sciences (PJNS)

Myasthenia gravis (MG) is an autoimmune disease in which antibodies are directed against postsynaptic membrane of neuromuscular junction, resulting in muscle weakness and fatigability. We report a rare case of an 11 years old boy who was a known case of myasthenia gravis presented with progressive weakness and wasting of facial and limb musculature and was found to have coexisting muscular dystrophy most like facioscapulohumeral muscular dystrophy (FSHD).

Brain Blast 2014 Poster, Annie Leslie

Brain Blast

Announcement poster for UNE's Brain Blast 2014, a pechakucha-style brain health and safety awareness and learning event.

Hallervorden Spatz Disease – A Rare Clinicoradiological Diagnosis, Anam Abrar, Arsalan Ahmad, Ejaz A Khan

Pakistan Journal of Neurological Sciences (PJNS)

Hallervorden Spatz disease, also known as pantothenate kinase associated neuro-degeneration, is a rare, progressive neurological disorder usually seen in first decade of life. It is associated with extrapyramidal effects, dysarthria and dementia. Hallervorden Spatz Disease is also associated with psychiatric symptoms, depression and behavioral changes. Affected patients are disabled predominantly by dystonia. MRI, in later stage of the disease, shows “eye of the tiger’ appearance which is fairly diagnostic of Hallervorden Spatz Disease. Response to drugs is often poor and of limited value to these patients. This report highlights a classical case of Hallervorden Spatz disease that presented as an …

Brain Blast 2014 Speakers Poster, Annie Leslie

Brain Blast

Poster from UNE's Brain Blast 2014 listing the presenters at this event.

Stroke In Women: Burden, Challenges And Future For Pakistan, Maria Khan, Mohammad Wasay

Pakistan Journal of Neurological Sciences (PJNS)

The burden of stroke is increasing around the world in both men and women. South Asia is home to 20% of the world’s population but harbors almost 80% of world strokes. Previously sex specific data on stroke incidence, prevalence, severity and mortality was not available. Now over recent years this aspect has received attention. A systematic review in 2009 concluded that although stroke in women is less common compared to men, it has a greater severity and case fatality. Although this kind of data is not available from Pakistan, some preliminary reports suggest that this might be the case in …

Clinical Predictors Of Mechanical Ventilation In Guillain-Barré Syndrome (Gbs), Sumaira Nabi, Sadaf Khattak, Muhammad Irshad Awan

Pakistan Journal of Neurological Sciences (PJNS)

Guillain-Barre’ Syndrome is the most common cause of acute flaccid paralysis worldwide. One of the most serious complications is respiratory failure warranting mechanical ventilation which is required in about 30% of the patients. Various studies have been carried out to identify clinical factors which could help identify patients with impending respiratory failure and facilitate elective mechanical ventilation in these patients to reduce the associated mortality. Material and methods: 92 patients diagnosed with GBS were enrolled from February 6, 2013 to August 5, 2013 in department of Neurology, PIMS, Islamabad. Each patient was then be managed accordingly and monitored for therequirement …

Impact Of Guillain Barre Syndrome On Psychosocial Functionings Of Patients In Islamabad, Muhammad Naveed Babur, Farrukh Shahzad, Waqar Ahmed Awan

Pakistan Journal of Neurological Sciences (PJNS)

GuillainBarre Syndrome (GBS) is a rare autoimmune disease of unknown causes that affects peripheral nervous system. Objectives: To review the impacts of the GuillainBarre Syndrome on the psychosocial functioning of the patients and to assess the relationship between GuillainBarre Syndrome and the psychosocial functioning Methodology: Comparative cross sectional survey was conducted on 100 participants (50 GBS patients from Shifa International Hospitals and 50 normal participants from Islamabad in 6 months time from February to July 2013. Data collected through Structured Questionnaire in hospital settings for GBS patients and normal persons from Islamabad through Psychosocial functioning scale and social functioning scale …

Variants And Variations Among Gullian Barre Syndrome Presenting As Acute Flaccid Paralysis, Alam Ibrahim Siddiqui, Ghulam Rasool Bouk, Lal Chand, Sarfraz Ahmed Mahesar, Ghulam Mustafa Tunio

Pakistan Journal of Neurological Sciences (PJNS)

Acute flaccid paralysis (AFP) is a clinical syndrome characterized by rapid onset of weakness that frequently includes respiratory and bulbar weakness. An accurate and early diagnosis of the cause has important bearing on the management and prognosis. Guillian Barre Syndrome (GBS) is a post infectious polyradiculoneuropathy involving mainly motor but sometimes sensory and autonomic nerves.

Neuroradiological Manifestations Of Tuberculous Meningitis, Sumaira Nabi, Sadaf Khattak, Mazhar Badshah, Haris Majid Rajput

Pakistan Journal of Neurological Sciences (PJNS)

Tuberculous meningitis (TBM) represents the most severe form of extra pulmonarytuberculosis (1).The early and exact diagnosis of TBM is important but difficult due to time consuming definitive microbiological procedures (2).Neuroimaging is an important initial investigation in tuberculous meningitis(3).This study was conducted to evaluate the neuroradiological findings in patients with tuberculous meningitis, as a useful modality for itsearly diagnoses and prompt treatment.

Spontaneous Intracranial Hypotension, Mehwish Sayed, Ahmed Asif, Bushra Reha, Amanullah Khan

Pakistan Journal of Neurological Sciences (PJNS)

Spontaneous intracranial hypotension (SIH) is uncommon and usually undiagnosed entity which present most commonly with postural headache. The diagnosis is difficult because of the nonspecific symptoms and lack of awareness about the disease .It is important to recognize SIH as if the disease goes undiagnosed it can cause a significant delay in initiation of the treatment and exposing patient to the risks associated with the treatment for the disorders mimicking intracranial hypotension . We describe a patient with SIH and outline the important clinical and radiological features of this syndrome. The aim of presenting this case report is to emphasize …

Radiosurgery: Current Recommendations For Intracranial Lesions And Practices In Pakistan, Hussain Shallwani, Sohaib Tariq, Muhammad E Bari

Pakistan Journal of Neurological Sciences (PJNS)

Radiosurgery is defined as the use of high-dose ionizing radiation for precise and total destruction of a chosen target, avoiding concomitant or delayed harm to adjacent tissues. The three types of radiations include gamma rays (GammaKnife) by Cobalt-60, X8 rays using linear accelerator (LINAC) system, and charged-particles generated from synchocyclotron. Recommendations: Based on current evidence, radiosurgery has proven benefits as the treatment of certain brain metastases, vestibular schwannomas, meningiomas, and some skull base tumors. Conclusion: This review describes the current recommendations for use of radiosurgery for intracranial pathologies. Radiosurgery is a relatively newer modality in the developing country of Pakistan. …

Isoflavones And Alzheimer’S Disease: The Effects Of Soy In Diet, Sara Habib, Asad Ali Khan Afridi, Mohammad Wasay, Romaina Iqbal

Pakistan Journal of Neurological Sciences (PJNS)

Pathologically, Alzheimer’s disease is a result of aggregation of amyloid peptides and protein tau in the brain forming neurofibrillary tangles which are highly toxic to neuronal circuits in the brain. Recent evidences report that apart from aging, estrogen deficiency is one of the risk factors predisposing to the development of Alzheimer’s disease. Isoflavones, also known as phytoestrogens, are metabolized by the body forming compounds that are known to interfere with neurotoxic pathways and through their anti-fibrillization effects they play a role in reducing apoptosis of neurons and glial cells and promote axonal regeneration. Experimental studies on transgenic models with Alzheimer’s …

Novel Anticancer Agents In Clinical And Preclinical Trials, Adnan Salim

Section of Neurosurgery

No abstract provided.

International Telemedicine Consultations For Neurodevelopmental Disabilities, Phillip L. Pearl, Craig A. Sable, Sarah Helen Evans, Joseph Knight, Parker Cunningham, Gaetano R. Lotrecchiano, Andrea L. Gropman, Sheela Stuart, Penny J. Glass, Anne Conway, Issam Ramadan, Tania Paiva, Mark L. Batshaw, Roger J. Packer

Neurology Faculty Publications

Background: A telemedicine program was developed between the Children's National Medical Center (CNMC) in Washington, DC, and the Sheikh Khalifa Bin Zayed Foundation in the United Arab Emirates (UAE). A needs assessment and a curriculum of on-site training conferences were devised preparatory to an ongoing telemedicine consultation program for children with neurodevelopmental disabilities in the underserved eastern region of the UAE.

Materials and Methods: Weekly telemedicine consultations are provided by a multidisciplinary faculty. Patients are presented in the UAE with their therapists and families. Real-time (video over Internet protocol; average connection, 768 kilobits/s) telemedicine conferences are held weekly following previews …

Current Pathophysiological Concepts In Cerebral Small Vessel Disease., Fred Rincon, Clinton B Wright

Department of Neurology Faculty Papers

The association between cerebral small vessel disease (SVD) - in the form of white matter lesions, infarctions, and hemorrhages - with vascular cognitive impairment (VCI), has mostly been deduced from observational studies. Pathological conditions affecting the small vessels of the brain and leading to SVD have suggested plausible molecular mechanisms involved in vascular damage and their impact on brain function. However, much still needs to be clarified in understanding the pathophysiology of VCI, the role of neurodegenerative processes such as Alzheimer's disease, and the impact of aging itself. In addition, both genetic predispositions and environmental exposures may potentiate the development …

Autoantibodies To Agrin In Myasthenia Gravis Patients, Bin Zhang, Chengyong Shen, Beverly Bealmear, Samia Ragheb, Wen-Cheng Xiong, Richard A. Lewis, Robert P. Lisak, Lin Mei

Immunology and Microbiology Faculty Research Publications

To determine if patients with myasthenia gravis (MG) have antibodies to agrin, a proteoglycan released by motor neurons and is critical for neuromuscular junction (NMJ) formation, we collected serum samples from 93 patients with MG with known status of antibodies to acetylcholine receptor (AChR), muscle specific kinase (MuSK) and lipoprotein-related 4 (LRP4) and samples from control subjects (healthy individuals and individuals with other diseases). Sera were assayed for antibodies to agrin. We found antibodies to agrin in 7 serum samples of MG patients. None of the 25 healthy controls and none of the 55 control neurological patients had agrin antibodies. …

Distribution, Severity And Radiologic Features Of Intracranial Stenosis In Asymptomatic Pakistanis., Ayeesha Kamran Kamal, Farzin Majeed, Muhammad S. Ilyas, Munawar Hussain, Kamran Masood, Bilal Ahmed, Hasan Rehman,, Zafar Sajjad, Scott E. Kasner8

Department of Medicine

Background: Intracranial atherosclerotic disease (ICAD) is the most common ischemic stroke subtype globally. It accounts for 30–50% of all ischemic strokes in Asians. Aims—The aim of the study is to report the frequency of asymptomatic ICAD and its associated Magnetic Resonance Imaging (MRI) findings.

Methods: 200 adult participants were recruited from the Radiology Departments of two major diagnostic centers in Karachi. Eligible participants were confirmed for the absence of stroke symptoms via the Questionnaire for Verifying Stroke Free Status (QVSFS). QVSFS negative subjects underwent MRI on a 1.5 Tesla scanner. Images were centrally reviewed on Di com Viewer 3.0 with …

Microstructural Abnormalities In Language And Limbic Pathways In Orphanage-Reared Children: A Diffusion Tensor Imaging Study, Ajay Kumar, Michael E. Behen, Piti Singsoonsud, Amy L. Veenstra

University Faculty Publications and Creative Works

This study utilized diffusion tensor imaging fiber tractography to examine the miscrostructural integrity of limbic and paralimbic white matter tracts in 36 children (age M = 124 months) with histories of early deprivation, raised from birth in orphanages and subsequently adopted into the United States, compared to 16 age-matched typically developing children. We found increased mean diffusivity bilaterally in the arcuate fasciculus and increased mean diffusivity and reduced fractional anisotropy bilaterally in the uncinate fasciculus and cingulum in children with early deprivation. Microstructural integrity of the left arcuate fasciculus and right cingulum was related to language and behavioral functioning, respectively. …

Temporal Lobe Epilepsy And Surgery Selectively Alter The Dorsal, Not The Ventral, Default-Mode Network., Gaelle Eve Doucet, Christopher T Skidmore, James J. Evans, Ashwini Sharan Md, Michael R Sperling, Dorian Pustina, Joseph I Tracy

Department of Neurology Faculty Papers

The default-mode network (DMN) is a major resting-state network. It can be divided in two distinct networks: one is composed of dorsal and anterior regions [referred to as the dorsal DMN (dDMN)], while the other involves the more posterior regions [referred to as the ventral DMN (vDMN)]. To date, no studies have investigated the potentially distinct impact of temporal lobe epilepsy (TLE) on these networks. In this context, we explored the effect of TLE and anterior temporal lobectomy (ATL) on the dDMN and vDMN. We utilized two resting-state fMRI sessions from left, right TLE patients (pre-/post-surgery) and normal controls (sessions …

Is Surgical Spinal Decompression For Supratentorial Gbm Symptomatic Drop Down Metastasis Warranted? A Case Report And Review Of Literature, Muhammad Babar Khan, Muhammad Riaz, Muhammad Ehsan Bari

Medical College Documents

Background: Symptomatic spinal metastasis from an intracranial primary
glioblastoma multiforme (GBM) is very rare. Our literature search identified a total
of 42 such patients of which 11 were treated with surgical decompression for spinal metastasis with only one such report from the pediatric age group. Previous studies have reported variable outcomes after surgical management.

Case Description: We report the case of a 16‑year‑old boy who underwent
surgical spinal decompression for spinal metastasis after intracranial GBM. The
patient regained motor and autonomic function following surgery and reported
improvement in pain. We also present findings from a literature review using …

Headstrong Intervention For Pediatric Migraine Headache: A Randomized Clinical Trial., Michael A. Rapoff, Mark Connelly, Jennifer Bickel, Scott W. Powers, Andrew D. Hershey, Janelle R. Allen, Cynthia W. Karlson, Catrina C. Litzenburg, John M. Belmont

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The purpose of this study was to evaluate the efficacy of a self-guided CD-ROM program ("Headstrong") containing cognitive-behavioral self-management strategies versus an educational CD-ROM program for treating headaches, headache-related disability, and quality of life.

METHODS: Participants were 35 children ages 7-12 years with migraine recruited from one university medical center and two children's hospital headache clinics. Participants were randomly assigned to complete the Headstrong or educational control CD-ROM program over a 4-week period. Data on headache frequency, duration, and severity, migraine-related disability, and quality of life (QOL) were obtained at baseline, post-intervention, and 3-months post-intervention.

RESULTS: At post-intervention, Headstrong …

Neuronal Growth Cones And Regeneration: Gridlock Within The Extracellular Matrix, Diane M. Snow

Spinal Cord and Brain Injury Research Center Faculty Publications

The extracellular matrix is a diverse composition of glycoproteins and proteoglycans found in all cellular systems. The extracellular matrix, abundant in the mammalian central nervous system, is temporally and spatially regulated and is a dynamic "living" entity that is reshaped and redesigned on a continuous basis in response to changing needs. Some modifications are adaptive and some are maladaptive. It is the maladaptive responses that pose a significant threat to successful axonal regeneration and/or sprouting following traumatic and spinal cord injuries, and has been the focus of a myriad of research laboratories for many years. This review focuses largely on …

Proteoglycans: Road Signs For Neurite Outgrowth, Justin A. Beller, Diane M. Snow

Spinal Cord and Brain Injury Research Center Faculty Publications

Proteoglycans in the central nervous system play integral roles as "traffic signals" for the direction of neurite outgrowth. This attribute of proteoglycans is a major factor in regeneration of the injured central nervous system. In this review, the structures of proteoglycans and the evidence suggesting their involvement in the response following spinal cord injury are presented. The review further describes the methods routinely used to determine the effect proteoglycans have on neurite outgrowth. The effects of proteoglycans on neurite outgrowth are not completely understood as there is disagreement on what component of the molecule is interacting with growing neurites and …

Gaps And Opportunities In Refractory Status Epilepticus Research In Children: A Multi-Center Approach By The Pediatric Status Epilepticus Research Group (Pserg), Ivan Sanchez Fernandez, Nicholas S. Abend, Satish Agadi, Sookee An, Ravindra Arya, Jessica L. Carpenter, Kevin E. Chapman, William D. Gaillard, Tracy A. Glauser, David B. Golstein, Joshua L. Goldstein, Howard P. Goodkin, Cecil D. Hahn, Erin L. Heinzen, Mohamad A. Mikati, Katrina Peariso, John P. Pestian, Margie Ream, James J. Riviello, Robert C. Tasker, Korwyn Williams, Tobias Loddenkemper

Neurology Faculty Publications

PURPOSE:

Status epilepticus (SE) is a life-threatening condition that can be refractory to initial treatment. Randomized controlled studies to guide treatment choices, especially beyond first-line drugs, are not available. This report summarizes the evidence that guides the management of refractory convulsive SE (RCSE) in children, defines gaps in our clinical knowledge and describes the development and works of the 'pediatric Status Epilepticus Research Group' (pSERG).

METHODS:

A literature review was performed to evaluate current gaps in the pediatric SE and RCSE literature. In person and online meetings helped to develop and expand the pSERG network.

RESULTS:

The care of pediatric …

Expression Of Mir-15/107 Family Micrornas In Human Tissues And Cultured Rat Brain Cells, Wang-Xia Wang, Robert J. Danaher, Craig S. Miller, Joseph R. Berger, Vega G. Nubia, Bernard R. Wilfred, Janna H. Neltner, Christopher M. Norris, Peter T. Nelson

Sanders-Brown Center on Aging Faculty Publications

The miR-15/107 family comprises a group of 10 paralogous microRNAs (miRNAs), sharing a 5' AGCAGC sequence. These miRNAs have overlapping targets. In order to characterize the expression of miR-15/107 family miRNAs, we employed customized TaqMan Low-Density micro-fluid PCR-array to investigate the expression of miR-15/107 family members, and other selected miRNAs, in 11 human tissues obtained at autopsy including the cerebral cortex, frontal cortex, primary visual cortex, thalamus, heart, lung, liver, kidney, spleen, stomach and skeletal muscle. miR-103, miR-195 and miR-497 were expressed at similar levels across various tissues, whereas miR-107 is enriched in brain samples. We also examined the expression …

Spectrum Of Acquired Demyelinating Disorders Of The Central Nervous System In Adults In A Tertiary Care Hospital, Muhammad Athar Javed, Dr Qamar Zaman, Dr Naveed Alam, Ather Enam

Pakistan Journal of Neurological Sciences (PJNS)

Acquired demyelinating disorders of the central nervous system are diverse group of conditions characterized by destruction or disturbance of myelin sheath in the central nervous system i.e. brain and spinal cord. These vary in their causes, clinical presentations, and prognosis.

Utilizing Fast Spin Echo Mri To Reduce Image Artifacts And Improve Implant/Tissue Interface Detection In Refractory Parkinson’S Patients With Deep Brain Stimulators, Subhendra N. Sarkar, Pooja R. Sarkar, Efstathios Papavassiliou, Rafael Rojas

Publications and Research

Introduction. In medically refractory Parkinson’s disease (PD) deep-brain stimulation (DBS) is an effective therapeutic tool. Postimplantation MRI is important in assessing tissue damage and DBS lead placement accuracy. We wanted to identify which MRI sequence can detectDBS leads with smallest artifactual signal void, allowing better tissue/electrode edge conspicuity.

Methods. Using an IRB approved protocol 8 advanced PDpatientswere imagedwithinMRconditional safety guidelines at lowRF power (SAR ≤ 0.1 W/kg) in coronal plane at 1.5T by various sequences.The image slices were subjectively evaluated for diagnostic quality and the lead contact diameters were compared to identify a sequence least affected by metallic leads.

Results …

A Prospective, Multicenter, Phase I Matched-Comparison Group Trial Of Safety, Pharmacokinetics, And Preliminary Efficacy Of Riluzole In Patients With Traumatic Spinal Cord Injury., Robert G Grossman, Michael G Fehlings, Ralph F Frankowski, Keith D Burau, Diana S L Chow, Charles Tator, Angela Teng, Elizabeth G Toups, James Harrop, Bizhan Aarabi, Christopher I Shaffrey, Michele M Johnson, Susan J Harkema, Maxwell Boakye, James D Guest, Jefferson R Wilson

Department of Neurosurgery Faculty Papers

A prospective, multicenter phase I trial was undertaken by the North American Clinical Trials Network (NACTN) to investigate the pharmacokinetics and safety of, as well as obtain pilot data on, the effects of riluzole on neurological outcome in acute spinal cord injury (SCI). Thirty-six patients, with ASIA impairment grades A-C (28 cervical and 8 thoracic) were enrolled at 6 NACTN sites between April 2010 and June 2011. Patients received 50 mg of riluzole PO/NG twice-daily, within 12 h of SCI, for 14 days. Peak and trough plasma concentrations were quantified on days 3 and 14. Peak plasma concentration (Cmax) and …

Two Distinct Dynamic Modes Subtend The Detection Of Unexpected Sounds, Jean-Rémi King, Alexandre Gramfort, Aaron Schurger, Lionel Naccache, Stanislas Dehaene

Psychology Faculty Articles and Research

The brain response to auditory novelty comprises two main EEG components: an early mismatch negativity and a late P300. Whereas the former has been proposed to reflect a prediction error, the latter is often associated with working memory updating. Interestingly, these two proposals predict fundamentally different dynamics: prediction errors are thought to propagate serially through several distinct brain areas, while working memory supposes that activity is sustained over time within a stable set of brain areas. Here we test this temporal dissociation by showing how the generalization of brain activity patterns across time can characterize the dynamics of the underlying …

Pb Neurotoxicity: Neuropsychological Effects Of Lead Toxicity, Lisa H. Mason, Jordan P. Harp, Dong Y. Han

Neurology Faculty Publications

Neurotoxicity is a term used to describe neurophysiological changes caused by exposure to toxic agents. Such exposure can result in neurocognitive symptoms and/or psychiatric disturbances. Common toxic agents include heavy metals, drugs, organophosphates, bacterial, and animal neurotoxins. Among heavy metal exposures, lead exposure is one of the most common exposures that can lead to significant neuropsychological and functional decline in humans. In this review, neurotoxic lead exposure's pathophysiology, etiology, and epidemiology are explored. In addition, commonly associated neuropsychological difficulties in intelligence, memory, executive functioning, attention, processing speed, language, visuospatial skills, motor skills, and affect/mood are explored.