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An Unusual Presentation Of Neurononopathic Gauchers Disease., Prem Chand, Shahnaz Ibrahim, Bushra Habib, Farida jan 2013 Aga Khan University Hospital Karachi,pakistan

An Unusual Presentation Of Neurononopathic Gauchers Disease., Prem Chand, Shahnaz Ibrahim, Bushra Habib, Farida Jan

Pakistan Journal of Neurological Sciences (PJNS)

A six years old male child presented with tremor, ataxia, speech apraxia, supranuclear gaze palsy and hepatosplenomegaly. There was no history of seizures and psycho-cognitive abnormalities. The clinical and bone marrow findings were consistent with Gaucher's disease type 3.

KEY WORDS: Gauchers disease, Enzyme replacement therapy, Gene therapy, Bone marrow transplant.


Dementia In Pakistan: National Guidelines For Clinicians, Arsalan Ahmad, Khurram Owais, Maimoona Siddiqui, Kayser Mamun, Faiza Rao, Abdul Wahab Yousufzai 2013 Shifa International Hospital,islamabad

Dementia In Pakistan: National Guidelines For Clinicians, Arsalan Ahmad, Khurram Owais, Maimoona Siddiqui, Kayser Mamun, Faiza Rao, Abdul Wahab Yousufzai

Pakistan Journal of Neurological Sciences (PJNS)

Dementia refers to a range of disorders characterized by declining memory, multiple cognitive deficits and eventual mortality due to progressive and irreversible brain damage1. Disease entities comprising dementia characteristically affect mainly the geriatric population. Together, they constitute a major component of global disease burden in general and geriatric disease burden in particular2. In 2005, it was estimated that 24.3 million people in the world were living with dementia; 4.6 million cases have been added each year ever since3. Overall, dementia contributes 11.3% of years of life lived with disability, making it a major contributor …


Dementia – The Next Impending Epidemic Knocking On Our Door – Are We Ready?, Ismail A. Khatri, Mohammad Wasay 2013 King Saud bin Abdulaziz University of Health Sciences in Riyadh.

Dementia – The Next Impending Epidemic Knocking On Our Door – Are We Ready?, Ismail A. Khatri, Mohammad Wasay

Pakistan Journal of Neurological Sciences (PJNS)

Late-onset dementias are life-shortening diseases of largely unknown cause. Predictions of the number of persons living with dementia by 2050 have spawned alarming headlines forecasting a “tsunami” of dementia cases that will overwhelm families and health care systems and impose unbearable economic burdens [Whalley].


Mri Guided Functional Neurosurgery At L.G.H: The Beginning Of A New Era, Khalid Mahmood 2013 Lahore General Hospital

Mri Guided Functional Neurosurgery At L.G.H: The Beginning Of A New Era, Khalid Mahmood

Pakistan Journal of Neurological Sciences (PJNS)

Lahore General Hospital has the credit of being pioneer of “Functional Neurosurgery in Pakistan” when Prof. Bashir Ahmad started the Department of Neurosurgery at LGH in 1966. Prof. BashirAhmad, Prof. Nazir Ahmad and Late Prof. Javed MajidMian had done lot of work and performedThalamotomiesfor Parkinsoniantremors


Astrocyte-Specific Overexpression Of Insulin-Like Growth Factor-1 Protects Hippocampal Neurons And Reduces Behavioral Deficits Following Traumatic Brain Injury In Mice, Sindhu K. Madathil, Shaun W. Carlson, Jennifer M. Brelsfoard, Ping Ye, A Joseph D'Ercole, Kathryn E. Saatman 2013 University of Kentucky

Astrocyte-Specific Overexpression Of Insulin-Like Growth Factor-1 Protects Hippocampal Neurons And Reduces Behavioral Deficits Following Traumatic Brain Injury In Mice, Sindhu K. Madathil, Shaun W. Carlson, Jennifer M. Brelsfoard, Ping Ye, A Joseph D'Ercole, Kathryn E. Saatman

Spinal Cord and Brain Injury Research Center Faculty Publications

Traumatic brain injury (TBI) survivors often suffer from long-lasting cognitive impairment that stems from hippocampal injury. Systemic administration of insulin-like growth factor-1 (IGF-1), a polypeptide growth factor known to play vital roles in neuronal survival, has been shown to attenuate posttraumatic cognitive and motor dysfunction. However, its neuroprotective effects in TBI have not been examined. To this end, moderate or severe contusion brain injury was induced in mice with conditional (postnatal) overexpression of IGF-1 using the controlled cortical impact (CCI) injury model. CCI brain injury produces robust reactive astrocytosis in regions of neuronal damage such as the hippocampus. We exploited …


A Descriptive Study To Find Possible Correlation Between Mri Findings Of Pituitary Gland And Serum Prolactin Level, Muhammad Azeemuddin, Rohana Naqi, Mohammad Wasay 2013 Aga Khan University

A Descriptive Study To Find Possible Correlation Between Mri Findings Of Pituitary Gland And Serum Prolactin Level, Muhammad Azeemuddin, Rohana Naqi, Mohammad Wasay

Department of Medicine

Objective: To explore equation, if any, between findings of magnetic resonance imaging of pituitary gland and serum prolactin level.

Methods: The retrospective, descriptive study was conducted at the Department of Radiology, Aga Khan University Hospital, Karachi, and related to patients\' records from April 19, 2006 to April 23, 2009. Seventy patients underwent magnetic resonance imaging of brain for pituitary gland. Inclusion criteria were all patients referred with relevant clinical symptoms or deranged serum prolactin level. Patients who were claustrophobic or had a pacemaker, aneurysm clip, metallic foreign body in the orbit or with no laboratory investigation were excluded from the …


Benefit Of Neuroprotection In Acute Ischaemic Stroke, Shall We Dare To Hope?, Anjum Akhtar, Ayeesha Kamran Kamal 2013 Aga Khan University

Benefit Of Neuroprotection In Acute Ischaemic Stroke, Shall We Dare To Hope?, Anjum Akhtar, Ayeesha Kamran Kamal

Department of Medicine

No abstract provided.


Botulism In Children: A Diagnostic Dilemma In Developing Countries, Muhammad Rehan Khan, Prem Kumar, Shahnaz Shahid, Anwarul Haque 2013 Aga Khan University

Botulism In Children: A Diagnostic Dilemma In Developing Countries, Muhammad Rehan Khan, Prem Kumar, Shahnaz Shahid, Anwarul Haque

Department of Paediatrics and Child Health

Botulism is a well-known disease of the neuromuscular junction. It is a rare but curable cause of paralysis in paediatric population. In addition to classical clinical signs and symptoms, the diagnosis of botulism requires laboratory confirmation of intoxication by various biological tests. These include demonstration of botulinum toxin in serum or isolation of the Clostridium botulinum from stool/gastric aspirates. However, it is not always possible to confirm intoxication due to unavailability of technical facilities, especially in resource limited countries like Pakistan. Under these circumstances, electrophysiological studies serve as an excellent diagnostic tool. These studies can provide quick diagnosis of botulism …


My Patient Has Intracranial Stenosis, Does He Need An Intracerebral Stent? Results Of The Sammpris Study., Muhammad Faisal Wadiwala,, Ayeesha Kamran Kamal 2013 Aga Khan University

My Patient Has Intracranial Stenosis, Does He Need An Intracerebral Stent? Results Of The Sammpris Study., Muhammad Faisal Wadiwala,, Ayeesha Kamran Kamal

Department of Medicine

No abstract provided.


Hyper-Arousal Decreases Human Visual Thresholds, Adam J. Woods, John W. Philbeck, Philip Wirtz 2013 University of Pennsylvania

Hyper-Arousal Decreases Human Visual Thresholds, Adam J. Woods, John W. Philbeck, Philip Wirtz

Neurological Surgery Faculty Publications

Arousal has long been known to influence behavior and serves as an underlying component of cognition and consciousness. However, the consequences of hyper-arousal for visual perception remain unclear. The present study evaluates the impact of hyper-arousal on two aspects of visual sensitivity: visual stereoacuity and contrast thresholds. Sixty-eight participants participated in two experiments. Thirty-four participants were randomly divided into two groups in each experiment: Arousal Stimulation or Sham Control. The Arousal Stimulation group underwent a 50-second cold pressor stimulation (immersing the foot in 0–2° C water), a technique known to increase arousal. In contrast, the Sham Control group immersed their …


Converging Evidence For The Processing Costs Associated With Ambiguous Quantifier Comprehension., Corey T McMillan, Danielle Coleman, Robin Clark, Tsao-Wei Liang, Rachel G Gross, Murray Grossman 2013 Frontotemporal Degeneration Center, Department of Neurology, Perelman School of Medicine, University of Pennsylvania

Converging Evidence For The Processing Costs Associated With Ambiguous Quantifier Comprehension., Corey T Mcmillan, Danielle Coleman, Robin Clark, Tsao-Wei Liang, Rachel G Gross, Murray Grossman

Department of Neurology Faculty Papers

Traditional neuroanatomic models of language comprehension have emphasized a core language network situated in peri-Sylvian cortex. More recent evidence appears to extend the neuroanatomic network beyond peri-Sylvian cortex to encompass other aspects of sentence processing. In this study, we evaluate the neuroanatomic basis for processing the ambiguity in doubly-quantified sentences. For example, a sentence like "All the dogs jumped in a lake" can be interpreted with a collective interpretation (e.g., several dogs jumping into a single lake) or a distributive interpretation (e.g., several dogs each jumping into a different lake). In Experiment 1, we used BOLD fMRI to investigate neuroanatomic …


Asymptomatic Unilateral Cerebellar Hypoplasia In An Adult Patient - A Unique Presentation., Pari Gul, Bushra Rehan, Saleha Anwar, Ambreen Majeed, Ayub Mansoor 2013 Liaquat National Hospital, Karachi, Pakistan

Asymptomatic Unilateral Cerebellar Hypoplasia In An Adult Patient - A Unique Presentation., Pari Gul, Bushra Rehan, Saleha Anwar, Ambreen Majeed, Ayub Mansoor

Pakistan Journal of Neurological Sciences (PJNS)

Unilateral cerebellar hypoplasia is a relatively rare malformation. We report a case of 54 year old female patient who presented with single episode of sudden unconsciousness and slurring of speech. Imaging finding suggests the diagnosis of unilateral cerebellar hypoplasia. The patient recovered with conservative management probably because the symptoms were due to Transient ischemic attack not due to hypoplasia.


Tacrolimus Induced Reversible Encephalopathy, Nirmal Surya, Devshi Visana 2013 Bombay Hospital, Mumbai, India.

Tacrolimus Induced Reversible Encephalopathy, Nirmal Surya, Devshi Visana

Pakistan Journal of Neurological Sciences (PJNS)

Tacrolimus is a novel immunosuppressant used to prevent graft rejection after allogenic organ transplantation. Serious neurological complications of tacrolimus are rare. We present a lady who developed confusion, paresthesiae and visual hallucinations while on tacrolimus following a renal transplant.Her tacrolimus level was high and she improved on stopping the drug. This case highlights the reversible encephalopathy induced by tacrolimus.


Efficacy Of Interferon In Multiple Sclerosis, Evangelos A, , Nikolaos Ch. Syrmos, Anastasios Orologas 2013 Neurological Clinic, Laboratory of Neuropathology, AHEPA Hospital

Efficacy Of Interferon In Multiple Sclerosis, Evangelos A, , Nikolaos Ch. Syrmos, Anastasios Orologas

Pakistan Journal of Neurological Sciences (PJNS)

Introduction of interferon b-1b in the therapeutic strategy against Multiple Sclerosis has resulted in a great amelioration of treatment in various terms. Relapse rates, delaying progression of the activity, improvement of imaging outcomes as well as reduction of disease activity itself consist great achievements against a pathological condition that challenged medical effectiveness throughout decades. Two recombinant human IFN-1a and an IFN-1b preparations have been proved effected and well tolerated. We reviewed the most important literature concerning pharmacodynamics, medical efficacy, tolerability and adverse effects of interferon-b usage. In parallel, despite the wide range of trials referring to INF-b treatment, we tried …


My Patient Had A Small Vessel Stroke, Are Two Antiplatelet Agents Better Than One?, Muhammad Faisal Wadiwala, Ayeesha Kamran Kamal 2013 Aga Khan University

My Patient Had A Small Vessel Stroke, Are Two Antiplatelet Agents Better Than One?, Muhammad Faisal Wadiwala, Ayeesha Kamran Kamal

Department of Medicine

No abstract provided.


Clinical Profile And Outcome Of Children Admitted With Status Epileptics In Picu Of A Developing Country, Khalid Ahmed, Sidra Kaleem Jafr, Faria Bhatti, Arshad Rafique, Anwa rul Haque Haque 2013 Aga Khan University Hospital Karachi,pakistan

Clinical Profile And Outcome Of Children Admitted With Status Epileptics In Picu Of A Developing Country, Khalid Ahmed, Sidra Kaleem Jafr, Faria Bhatti, Arshad Rafique, Anwa Rul Haque Haque

Pakistan Journal of Neurological Sciences (PJNS)

Status epilepticus (SE) is a common, life-threatening neurologic disorder. The exact incidence is not known. The frequency of occurrence of status epilepticus is 17-23 per 100, 000 persons per year, with the higher incidences occurring in developing countries. In children the mortality from SE ranges from 3-10%.

Objective: To describe the etiology, clinical profile and immediate-outcome of children with status epilepticus at a Tertiary Care Hospital in Karachi.


Epilepsy: The Pakistan Perspective Some Suggestions, Fowzia Siddiqi 2013 Aga Khan University Hospital Karachi,pakistan

Epilepsy: The Pakistan Perspective Some Suggestions, Fowzia Siddiqi

Pakistan Journal of Neurological Sciences (PJNS)

Epilepsy is a disease that affects more than 65 million people around the world. In Pakistan prevalence of epilepsy is 9.99/1000 people, thus around 2million people in Pakistan suffering from epilepsy1,2.The prevalence in children is much higher 14.6/10003,4,5.Prof. Charles Newton in the lancet quotes 50,000 deaths from epilepsy annually in the US6. The death rate in the developing countries is much higher. The exact figures are difficult to assess due to lack of epidemiological surveys and incomplete data collection. However it is thought that the number will be greater and considered at least twice …


Abstracts From The 7th Annual Neurology Research Day, Shifa International Hospital, Islamabad, Syed Hussain Shah, Noreen Akhtar, Akhtar Waheed, Nida Fatima 2013 Armed Forces Institute of Rehabilitation Medicine, Rawalpindi, Punjab, Pakistan.

Abstracts From The 7th Annual Neurology Research Day, Shifa International Hospital, Islamabad, Syed Hussain Shah, Noreen Akhtar, Akhtar Waheed, Nida Fatima

Pakistan Journal of Neurological Sciences (PJNS)

:It's a cross sectional study conducted at CP clinic of Armed Forces institute of Rehabilitation medicine Rawalpindi from Jan 2012 to Jan 2013. All cerebral palsy children above 2 years of age and both genders were included in study. SPSS version 17 was used for data analysis.


Cerebral Sinus Thrombosis Secondary To Epidural Anaesthesia, Modar Khalil, Mohamed El-Sayed, Fayyaz Ahmed, Alec Ming 2013 Hull Royal Infirmary, Kingston-Upon-Hull, England

Cerebral Sinus Thrombosis Secondary To Epidural Anaesthesia, Modar Khalil, Mohamed El-Sayed, Fayyaz Ahmed, Alec Ming

Pakistan Journal of Neurological Sciences (PJNS)

Cerebral venous sinus thrombosis (CVST) is an uncommon, and yet potentially fatal, condition; we present a rare case of CVST as a complication of epidural anaesthesia used for caesarean section, we review different aspects

of CVST in terms of epidemiology, aetiology, investigations, and treatment, as well as most acceptable theory explaining the pathogenesis in our case report


Neuroinflammation And Psychiatric Illness, Souhel Najjar, Daniel M. Pearlman, Kenneth Alper, Amanda Najjar, Orrin Devinsky 2013 New York University School of Medicine

Neuroinflammation And Psychiatric Illness, Souhel Najjar, Daniel M. Pearlman, Kenneth Alper, Amanda Najjar, Orrin Devinsky

Dartmouth Scholarship

Multiple lines of evidence support the pathogenic role of neuroinflammation in psychiatric illness. While systemic autoimmune diseases are well-documented causes of neuropsychiatric disorders, synaptic autoimmune encephalitides with psychotic symptoms often go under-recognized. Parallel to the link between psychiatric symptoms and autoimmunity in autoimmune diseases, neuroimmunological abnormalities occur in classical psychiatric disorders (for example, major depressive, bipolar, schizophrenia, and obsessive-compulsive disorders). Investigations into the pathophysiology of these conditions traditionally stressed dysregulation of the glutamatergic and monoaminergic systems, but the mechanisms causing these neurotransmitter abnormalities remained elusive. We review the link between autoimmunity and neuropsychiatric disorders, and the human and experimental evidence …


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