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Hdqlife: Development And Assessment Of Health-Related Quality Of Life In Huntington Disease (Hd), N E Carlozzi, S G Schilling, J-S Lai, J S Paulsen, E A Hahn, J S Perlmutter, C A Ross, N R Downing, A L Kratz, M K McCormack, M A Nance, K A Quaid, J C Stout, R C Gershon, R E Ready, J A Miner, S K Barton, S L Perlman, S M Rao, S Frank, I Shoulson, H Marin, M D Geschwind, P Dayalu, S M Goodnight, D Cella 2016 University of Michigan - Ann Arbor

Hdqlife: Development And Assessment Of Health-Related Quality Of Life In Huntington Disease (Hd), N E Carlozzi, S G Schilling, J-S Lai, J S Paulsen, E A Hahn, J S Perlmutter, C A Ross, N R Downing, A L Kratz, M K Mccormack, M A Nance, K A Quaid, J C Stout, R C Gershon, R E Ready, J A Miner, S K Barton, S L Perlman, S M Rao, S Frank, I Shoulson, H Marin, M D Geschwind, P Dayalu, S M Goodnight, D Cella

Rowan-Virtua School of Osteopathic Medicine Departmental Research

PURPOSE: Huntington disease (HD) is a chronic, debilitating genetic disease that affects physical, emotional, cognitive, and social health. Existing patient-reported outcomes (PROs) of health-related quality of life (HRQOL) used in HD are neither comprehensive, nor do they adequately account for clinically meaningful changes in function. While new PROs examining HRQOL (i.e., Neuro-QoL-Quality of Life in Neurological Disorders and PROMIS-Patient-Reported Outcomes Measurement Information System) offer solutions to many of these shortcomings, they do not include HD-specific content, nor have they been validated in HD. HDQLIFE addresses this by validating 12 PROMIS/Neuro-QoL domains in individuals with HD and by using established PROMIS …


Awake Craniotomy For Brain Tumours In Pakistan: An Initial Case Series From A Developing Country, Saad Akhtar Khan, Karim Rizwan Nathani, Badar Uddin Ujjan, Muhammad Danish Barakzai, Ather Enam, Faraz Shafiq 2016 Aga Khan University

Awake Craniotomy For Brain Tumours In Pakistan: An Initial Case Series From A Developing Country, Saad Akhtar Khan, Karim Rizwan Nathani, Badar Uddin Ujjan, Muhammad Danish Barakzai, Ather Enam, Faraz Shafiq

Section of Neurosurgery

Awake craniotomy offers safe resection of brain tumours in eloquent area. Aga Khan University Hospital, Karachi, recently started the programme in Pakistan, and the current study was planned to assess our experience of the first 16 procedures. The retrospective study comprised all such procedures done from November 2015 to May 2016. Pre-operative and post-operative variables were analysed. Of the 16 patients, 11(68.75%) were males and 5(31.25%) were females. The overall median age was 37 years (interquartile range[IQR]: 23-62 years). The most common presenting complaint was seizures 8(50%), followed by headache6(38%). The common pathologies operated include oligodendroglioma and glioblastoma. Pre-operative mean …


Paediatric Traumatic Brain Injury: Presentation, Prognostic Indicators And Outcome Analysis From A Tertiary Care Center In A Developing Country, Badar Uddin Ujjan, Muhammad Waqas, Muhammad Babar Khan, Dr Saqib Kamran Bakhshi, Muhammad Ehsan Bari 2016 Aga Khan University

Paediatric Traumatic Brain Injury: Presentation, Prognostic Indicators And Outcome Analysis From A Tertiary Care Center In A Developing Country, Badar Uddin Ujjan, Muhammad Waqas, Muhammad Babar Khan, Dr Saqib Kamran Bakhshi, Muhammad Ehsan Bari

Section of Neurosurgery

Traumatic brain injury (TBI) is the leading cause of morbidity and mortality in children worldwide. This study was conducted to report the presentation, management, outcomes and prognostic indicators in a large series of patients from a tertiary care centre in a developing country. It is a review of prospectively collected data of paediatric patients with TBI admitted at our centre between July 2010 and December 2013. A total of 291 patients with a mean age of 7.2±5.0 years were dichotomised into survivors and non-survivors, and variables were compared between the two groups. The mean post-resuscitation Glasgow coma scale (GCS) score …


Exploring Patient And Family Satisfaction In Pediatric Neurological Surgery, Randa R. Barsoom, Todd A. Maugans, James F. Burrows, Paul Rosen 2016 Thomas Jefferson University

Exploring Patient And Family Satisfaction In Pediatric Neurological Surgery, Randa R. Barsoom, Todd A. Maugans, James F. Burrows, Paul Rosen

Student Papers, Posters & Projects

Introduction Patient and family satisfaction during outpatient visits is correlated with a continuance of care and likelihood to recommend the practice to others. Additionally, patient-family satisfaction can determine the success of the practice and influence medical outcomes. Utilizing a well-validated surveys instrument, patient and family satisfaction can be explored in the office setting. Methods During a consecutive 36 month period, a standardized and validated patient satisfaction survey instrument was provided to the family members of patients who presented to two pediatric neurosurgery clinics associated with Nemours Children's Health System. The completed surveys were analyzed statistically to identify correlations between overall …


A Fourier Description Of Covariance, And Separation Of Simultaneously Encoded Slices With In-Plane Acceleration In Fmri, Mary C. Kociuba 2016 Marquette University

A Fourier Description Of Covariance, And Separation Of Simultaneously Encoded Slices With In-Plane Acceleration In Fmri, Mary C. Kociuba

Dissertations (1934 -)

Functional magnetic resonance imaging (fMRI) studies aim to identify localized neural regions associated with a cognitive task performed by the subject. An indirect measure of the brain activity is the blood oxygenation level dependent (BOLD) signal fluctuations observed within the complex-valued spatial frequencies measured over time. The standard practice in fMRI is to discard the phase information after image reconstruction, even with evidence of biological task-related change in the phase time-series. In the first aim of this dissertation, a complex-valued time-series covariance is derived as a linear combination of second order temporal Fourier frequency coefficients. As opposed to magnitude-only analysis, …


Co-Existing Spinal Intradural Ependymal Cyst And Sacral Tarlov Cyst In Adult-Onset Tethered Cord Syndrome With Syringomyelia: Case Report And Literature Review, Hamid H. Rai, Muhammad F. Khan, Ather Enam, Imtiaz Hashmi 2016 Aga Khan University

Co-Existing Spinal Intradural Ependymal Cyst And Sacral Tarlov Cyst In Adult-Onset Tethered Cord Syndrome With Syringomyelia: Case Report And Literature Review, Hamid H. Rai, Muhammad F. Khan, Ather Enam, Imtiaz Hashmi

Section of Neurosurgery

Background: Synchronous spinal intradural ependymal cysts and sacral Tarlov cysts in adult onset tethered cord syndrome are extremely rare.
Case description: A 23-year-old male presented with back pain radiating into both lower extremities, accompanied by acute onset of gait difficulty and sphincter dysfunction. Magnetic resonance imaging identified a low lying conus medullaris, syringomyelia with septations extending from T12 to S1, a tethered cord, and a thickened filum terminale with a sacral Tarlov cyst. The patient underwent a L3-4 laminectomy for decompression of syringomyelia and excision/biopsy of a space occupying lesion along with S1-2 laminectomy for cord untethering and Tarlov cyst …


Prevalence Of Stroke In Children Admitted With Sickle Cell Anaemia To Mulago Hospital, Samden Lhatoo, Martha Sajatovic 2016 Case Western Reserve University

Prevalence Of Stroke In Children Admitted With Sickle Cell Anaemia To Mulago Hospital, Samden Lhatoo, Martha Sajatovic

Faculty Scholarship

Background: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. Methods: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in …


Role Of Diffusion Tensor Imaging In Prognostication And Treatment Monitoring In Niemann-Pick Disease Type C1, Meghann Lau, Ryan Lee, Robin MIyamoto, Eun Sol Jung, Nicole Farhat, Shoko Yoshida, Susumu Mori, Andrea L. Gropman, Eva Baker, Forbes Porter 2016 George Washington University

Role Of Diffusion Tensor Imaging In Prognostication And Treatment Monitoring In Niemann-Pick Disease Type C1, Meghann Lau, Ryan Lee, Robin Miyamoto, Eun Sol Jung, Nicole Farhat, Shoko Yoshida, Susumu Mori, Andrea L. Gropman, Eva Baker, Forbes Porter

Neurology Faculty Publications

Niemann-Pick Disease, type C1 (NPC1) is a rapidly progressive neurodegenerative disorder characterized by cholesterol sequestration within late endosomes and lysosomes, for which no reliable imaging marker exists for prognostication and management. Cerebellar volume deficits are found to correlate with disease severity and diffusion tensor imaging (DTI) of the corpus callosum and brainstem, which has shown that microstructural disorganization is associated with NPC1 severity. This study investigates the utility of cerebellar DTI in clinical severity assessment. We hypothesize that cerebellar volume, fractional anisotropy (FA) and mean diffusivity (MD) negatively correlate with NIH NPC neurological severity score (NNSS) and motor severity subscores. …


Brainstem Tuberculoma As A Manifestation Of Paradoxical Reaction In A Patient With Tuberculous Meningitis., Farheen Niazi, Mazhar Badshah 2016 PAEC General hospital, Islamabad.

Brainstem Tuberculoma As A Manifestation Of Paradoxical Reaction In A Patient With Tuberculous Meningitis., Farheen Niazi, Mazhar Badshah

Pakistan Journal of Neurological Sciences (PJNS)

Tuberculosis commonly involves the CNS, During drug treatment of CNS tuberculosis paradoxical response (PR) may occasionally occur. The term “paradoxicalresponse”refers to the development of previously nonexistent tuberculosis lesions or worsening of preexisting lesions during antituberculosis treatment( ATT). Etiology is still not clear but delayed type hypersensitivity and alteration in immune system is thought to be a cause. Continued treatment improves PR without severe sequelae. We report a 41 year-old female patient with tuberculous meningitis(TBM) who developed brainstem tuberculomaduring the course of ATT, after about 9 months of treatment as a paradoxical response to ATT. Early identification, continuation of ATT with …


In-Hospital Outcome In Patients With Spontaneous Intracerebral Hemorrhage (Ich), Faraz Ahmed, Mazhar Badshah, Sumaira Nabi 2016 Pakistan institute of medical sciences, Islamabad

In-Hospital Outcome In Patients With Spontaneous Intracerebral Hemorrhage (Ich), Faraz Ahmed, Mazhar Badshah, Sumaira Nabi

Pakistan Journal of Neurological Sciences (PJNS)

Spontaneous intracerebral hemorrhage (ICH) is associated with a high in-hospital mortality and morbidity. One radiological parameter which was found significant in predicting mortality was the presence of hydrocephalus on CT brain (plain). The aim of this study was to determine the frequency of hydrocephalus in patients with spontaneous ICH and to determine the in-hospital outcome in patients of spontaneous ICH with hydrocephalus.MATERIALS AND METHODS: This was a descriptive case series carried out in Department of Neurology, Pakistan institute of Medical Sciences, Islamabad from December 30, 2013 to June 29, 2014. All patients who meet the inclusion criteria were selected for …


The Rise And Rise Of Neurosurgery: Academic Journey Of A Young Department In A Developing Country, Muhammad Shahzad Shamim, Hamid Hussain Rai Hussain Rai 2016 Aga Khan University Hospital, Karachi

The Rise And Rise Of Neurosurgery: Academic Journey Of A Young Department In A Developing Country, Muhammad Shahzad Shamim, Hamid Hussain Rai Hussain Rai

Pakistan Journal of Neurological Sciences (PJNS)

The Section of Neurosurgery, part of the Department of Surgery, and the Mind and Brain Service Line at the Aga Khan University Hospital Karachi, has come a long way since its recognition in mid-90s. Although the program itself was recognized in late 90’s and the first resident graduated in 2004, it wasn't until 2008 that it assumed its current shape of a formal, structured training program. Currently it has six full-time faculty members, one part-time faculty member, two instructors, and a team of 12 residents. Following are few of the innovations introduced within the program over the last few years.


Awareness About The Signs, Symptoms And The Risk Factors Of Stroke In Patients With Stroke: A Multicentre Study, Nausheen Farooq, Haider Darain 2016 Physiotherapist Saudi Arabia

Awareness About The Signs, Symptoms And The Risk Factors Of Stroke In Patients With Stroke: A Multicentre Study, Nausheen Farooq, Haider Darain

Pakistan Journal of Neurological Sciences (PJNS)

The aim of this survey was to determine the levels of awareness regarding symptoms, warning signs and risk factors of stroke in patients with stroke. For the latter purpose, a total of 500 participants who had stroke were recruited from four different hospitals of Rawalpindi and Islamabad. Patients who were already diagnosed by physicians with either type of stroke (haemorrhagic and ischemic) were included in this trial. Data was collected through a questionnaire and was analysed in SPSS version 20. Both male and female population were included in this study. The minimum age for the patients to be included was …


Subarachnoid Hemorrhage:Unusual Situations Leading To Sah And Underlying Principles Of Physics Behind Its Complications, Ali Zohair Nomani, Sumaira Nabi 2016 Pakistan Institute of Medical Sciences, Islamabad, Pakistan.

Subarachnoid Hemorrhage:Unusual Situations Leading To Sah And Underlying Principles Of Physics Behind Its Complications, Ali Zohair Nomani, Sumaira Nabi

Pakistan Journal of Neurological Sciences (PJNS)

Multiple authors have identified the most unusual novel associations as precipitant factors of subarachnoid hemorrhage and the knowledge of these and pathogenesis in background is necessary to suspect and therefore timely diagnose subarachnoid bleed and understand the mechanism of its subsequent complications. Objective: We herein describe unusual causes of subarachnoid bleed reported in various case reports with a comprehensive but elaborative review describing underlying pathogenesis and physiological mechanisms behind these precipitants and complications. Evidence Review: We sorted unusual causes of subarachnoid hemorrhage from literature review. By conducting meticulous scrutinization on search engines likePubmed®, Medline ®, Medline Plus ®, PubMed Central …


Adem, Diagnostic Dilemma, A Tertiary Care Hospital Experience:, Rajesh Kumar, Hazim Brohi 2016 LNH Karachi

Adem, Diagnostic Dilemma, A Tertiary Care Hospital Experience:, Rajesh Kumar, Hazim Brohi

Pakistan Journal of Neurological Sciences (PJNS)

Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease of the central nervous system that usually occurs following a viral infection or vaccination, bacterial or parasitic infection, or even spontaneously. ADEM is a monophasic; poly symptomatic disorder but it may present with various combinations of motor, sensory, visual and cognitive symptoms. Some case reports of ADEM presented as psychiatric disorders primarily as confusion psychosis and at times dissociative disorder. At times, the diagnosis becomes a dilemma due to multifaceted picture. Due to confusion physician may go to extreme range of tests with no specific conclusion. Debates regarding exact diagnosis …


Management And Outcomes Of Intramedullary Spinal Cord Tumors: A Single Center Experience From A Developing Country, Dr Saqib Kamran Bakhshi, Muhammad Waqas, Baila Shakaib, Ather Enam 2016 Aga Khan University

Management And Outcomes Of Intramedullary Spinal Cord Tumors: A Single Center Experience From A Developing Country, Dr Saqib Kamran Bakhshi, Muhammad Waqas, Baila Shakaib, Ather Enam

Section of Neurosurgery

Background: Intraoperative neurophysiology, high magnification microscopes, and ultrasonic aspirators are considered essential aid for the safe resection of intramedullary spinal cord tumors (IMSCTs). Most centers in developing countries such as Pakistan still lack these facilities. The purpose of this study was to review the management of IMSCTs at our hospital and to determine factors associated with the outcomes of surgery.
Methods: This was a retrospective review of medical records of adult patients undergoing surgery for IMSCT over 12 years. The institutional ethical review committee approved this study. Data were collected regarding demographics, clinical and radiological features, and surgical details. Modified …


Association Of Iron Deficiency Anemia In Children With Febrile Convulsions, Vikash Lal, Haresh Kumar, Shahina Hanif, Om Parkash, Suneel Arwani 2016 Dow University of Health Sciences, Karachi, Pakistan.

Association Of Iron Deficiency Anemia In Children With Febrile Convulsions, Vikash Lal, Haresh Kumar, Shahina Hanif, Om Parkash, Suneel Arwani

Pakistan Journal of Neurological Sciences (PJNS)

:To determine the association of iron deficiency anemia in children presented with febrile convulsion. Study Design: Case control study Setting: The pediatric unit III,Civil Hospital,Karachi. Duration: Six months from 30th April 2013 to 1st November 2013 Material and Methods: History regarding age, sex, developmental milestones, family history of febrile seizures or epilepsy, mean of the temperature peak at admission, and the underlying illness were recorded for all cases and controls, as well as details of seizure history, duration and frequency. Blood samples were collected in the Paediatric wardsfor measurements of hemoglobin, serum ferritin, MCV, MCH and MCHC, serum electrolytes, serum …


Magnetic Resonance Imaging Of Intracranial Extramedullary Haematopoiesis In Β-Thalassemia: A Case Report, Mahnoor Hafeez, Ateeque Ahmed Khan 2016 Civil Hospital & Dow University of Health Sciences (DUHS), Karachi, Pakistan.

Magnetic Resonance Imaging Of Intracranial Extramedullary Haematopoiesis In Β-Thalassemia: A Case Report, Mahnoor Hafeez, Ateeque Ahmed Khan

Pakistan Journal of Neurological Sciences (PJNS)

We report the Magnetic Resonance Imaging of intracranial extramedullaryhematopoiesis in 21 years old β-thalassemic patient who presented to the Neurology Department of Civil Hospital Karachi with seizures. The MR exam revealed multiple intracranialextra-axial masses;isointense on T1W sequence and hypointenseon T1W, T2W, FLAIR, DWI sequence and ADC (b value= 1000 sec/mm2) with avid post contrast enhancement. Plain CT of the Head documented the presence of multiple hyperdenseextra-axial masses with mean value of 45 HU. Extramedullary hematopoiesis is the formation and development of blood cells in tissues outside of the medulla of the bone. The most common sites of involvement are the …


“More Than Meets The Eye” Non Secretory Myeloma Presenting As Cidp In A Patient With Longstanding Diabetic Polyneuropathy. A Diagnostic And Therapeutic Challenge., Farheen Niazi, Arsalan Ahmad 2016 PAEC General hospital, Islamabad, Pakistan

“More Than Meets The Eye” Non Secretory Myeloma Presenting As Cidp In A Patient With Longstanding Diabetic Polyneuropathy. A Diagnostic And Therapeutic Challenge., Farheen Niazi, Arsalan Ahmad

Pakistan Journal of Neurological Sciences (PJNS)

Diagnosing chronic inflammatory polyradiculopathy in patients with pre-existing diabetic sensorimotor polyneuropathy is a diagnostic challenge. We present a case of a 69 years old who presented with weakness of legs for two months,he was diagnosed as having CIDP on the background of diabetic sensorimotor polyneuropathy, further and extensive workup revealed the final diagnosis of nonsecretory myeloma. Diagnosing non secretory myeloma is itself a diagnostic challenge and usually first line investigations for the workup of myeloma are negative as was the case in our patient. Our patient with CIDP had raised free light chains of kappa which made the final diagnosis …


An Overview Of Pathophysiology, Assessment And Management Strategies Of Post Stroke Shoulder Subluxation, Sarah Razaq, Farooq Azam Rathore 2016 e,Pakistan Aeronautical Complex Hospital, Kamra, Pakistan

An Overview Of Pathophysiology, Assessment And Management Strategies Of Post Stroke Shoulder Subluxation, Sarah Razaq, Farooq Azam Rathore

Pakistan Journal of Neurological Sciences (PJNS)

Shoulder subluxation is a common complication after stroke. It can result in soft tissue damage, stretching of the joint capsule, rotator cuff injury, and in some cases pain. An estimated 17-81 % of stroke patients have shoulder subluxation. It limits the use of affected extremity in addition to the weakness resulting from stroke itself. It can delay the neurological recovery in hemiplegic stroke patients. The etiology is multifactorial and the underlying mechanisms alongwith its association with shoulder pain are not clearly understood. This makes management of post stroke shoulder- subluxation a challenge for stroke management team. This review provides an …


Knowledge, Attitude And Practice Of Epilepsy Among Schoolteachers In Pakistan, Syed Wasim Akhtar Wasim Akhtar, Zarine Mogal 2016 Abbasi Shaheed Hospital, Karachi, Pakistan

Knowledge, Attitude And Practice Of Epilepsy Among Schoolteachers In Pakistan, Syed Wasim Akhtar Wasim Akhtar, Zarine Mogal

Pakistan Journal of Neurological Sciences (PJNS)

Assessment of Knowledge, Attitude and Practice (KAP) concerning epilepsy among schoolteachers in Pakistan. No such study has previously been conducted in this country and results would help formulate a comprehensive plan of action to address this important issue.


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