74 results match your criteria: "Western Australian Neuroscience Research Institute[Affiliation]"

Part 2: Making the "unproven" "proven".

Cytotherapy

January 2016

Center for Stem Cell Research (a unit of inStem, Bengaluru), Department of Hematology, Christian Medical College, Vellore, India. Member at large of the ISCT Presidential Task Force on the Use of Unproven Cellular Therapies.

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CT perfusion in acute stroke calls: A pictorial review and differential diagnoses.

J Med Imaging Radiat Oncol

April 2016

Neurological Intervention and Imaging Service of WA (NIISwa), Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.

CT perfusion is increasingly utilised in hyperacute stroke to facilitate diagnosis and patient selection for reperfusion therapies. This review article demonstrates eight examples of how CT perfusion can be used to diagnose stroke mimics and small volume infarcts, which can be easily missed on non-contrast CT, and to suggest the presence of an ischaemic penumbra. Radiologists involved in stroke management must understand the importance of rapid imaging acquisition and be confident in the prospective interpretation of this powerful diagnostic tool as we move into a new era of hyperacute stroke care.

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CT perfusion can delineate lacunar infarcts in acute stroke calls.

Neurol Clin Pract

December 2015

Neurological Intervention and Imaging Service of WA (NIISwa) (AHC, WM); Department of Neurology (GJH, DJB), Sir Charles Gairdner Hospital; School of Medicine & Pharmacology (GJH), University of Western Australia; and Western Australian Neuroscience Research Institute (DJB), Perth, Australia.

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The caudal zona incerta target within the posterior subthalamic area is an investigational site for deep brain stimulation (DBS) in Parkinson disease (PD) and tremor. The authors report on a patient with tremor-predominant PD who, despite excellent tremor control and an otherwise normal neurological examination, exhibited profound difficulty swimming during stimulation. Over the last 20 years, anecdotal reports have been received of 3 other patients with PD who underwent thalamic or pallidal lesioning or DBS surgery performed at the authors' center and subsequently drowned.

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NUT midline carcinoma (NMC) is a fatal cancer that arises in various tissues along the upper midline of the body. The defining molecular feature of NMC is a chromosomal translocation that joins (in the majority of cases) the nuclear testis gene NUT (NUTM1) to the bromodomain protein family member 4 (BRD4) and thereby creating a fusion oncogene that disrupts cellular differentiation and drives the disease. In this study, we report the case of an adolescent NMC patient presenting with severe facial pain, proptosis and visual impairment due to a mass arising from the ethmoid sinus that invaded the right orbit and frontal lobe.

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Asymptomatic progressive multifocal leukoencephalopathy during natalizumab therapy with treatment.

J Clin Neurosci

March 2016

Centre for Neuromuscular and Neurological Disorders, Western Australian Neuroscience Research Institute, The University of Western Australia, Sir Charles Gairdner Hospital, 4th Floor, A Block, QEII Medical Centre, Verdun Street, Nedlands, WA 6009, Australia; Institute of Immunology and Infectious Diseases, Murdoch University, Murdoch, WA 6150, Australia. Electronic address:

We report a case of asymptomatic progressive multifocal leukoencephalopathy (PML) detected on regular MRI screening in a 40-year-old patient with subsequent benign course with 12 months follow-up. The patient had a history of aggressive inflammatory multiple sclerosis, prior mitoxantrone therapy, Stratify John Cunningham Virus test positivity (Quest Diagnostics, Madison, NJ, USA), and 5 years of natalizumab monotherapy. The initial MRI detection of PML was both atypical and subtle.

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The Acquisition of Target Dependence by Developing Rat Retinal Ganglion Cells.

eNeuro

October 2015

School of Anatomy, Physiology, and Human Biology, The University of Western Australia , Perth, Western Australia 6009, Australia ; Western Australian Neuroscience Research Institute, Nedlands, Western Australia 6009, Australia.

Similar to neurons in the peripheral nervous system, immature CNS-derived RGCs become dependent on target-derived neurotrophic support as their axons reach termination sites in the brain. To study the factors that influence this developmental transition we took advantage of the fact that rat RGCs are born, and target innervation occurs, over a protracted period of time. Early-born RGCs have axons in the SC by birth (P0), whereas axons from late-born RGCs do not innervate the SC until P4-P5.

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Amplitude-integrated EEG for detection of neonatal seizures: a systematic review.

Seizure

December 2015

Department of Neonatology, King Edward Hospital for Women and Princess Margaret Hospital for Children, Subiaco, Western Australia 6008, Australia; Center for Neonatal Research and Education, School of Pediatrics and Child Health, University of Western Australia, Crawley, Western Australia 6009, Australia. Electronic address:

Purpose: Amplitude-integrated electroencephalogram (aEEG) is being used increasingly for monitoring seizures in neonatal units. Its accuracy, compared with "the gold-standard" conventional elecroencephalogram (cEEG) is still not well established. We aimed to conduct a systematic review to evaluate the diagnostic accuracy of aEEG when compared with cEEG, for detection of neonatal seizures.

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Neuroprotective peptides fused to arginine-rich cell penetrating peptides: Neuroprotective mechanism likely mediated by peptide endocytic properties.

Pharmacol Ther

September 2015

Centre for Neuromuscular and Neurological Disorders, The University of Western Australia, Nedlands, Australia; Department of Neurosurgery, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, Western Australia, Australia; Western Australian Neuroscience Research Institute, Nedlands, Australia.

Several recent studies have demonstrated that TAT and other arginine-rich cell penetrating peptides (CPPs) have intrinsic neuroprotective properties in their own right. Examples, we have demonstrated that in addition to TAT, poly-arginine peptides (R8 to R18; containing 8-18 arginine residues) as well as some other arginine-rich peptides are neuroprotective in vitro (in neurons exposed to glutamic acid excitotoxicity and oxygen glucose deprivation) and in the case of R9 in vivo (after permanent middle cerebral artery occlusion in the rat). Based on several lines of evidence, we propose that this neuroprotection is related to the peptide's endocytosis-inducing properties, with peptide charge and arginine residues being critical factors.

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Lipid lowering for primary prevention of stroke in older adults?

BMJ

May 2015

School of Medicine and Pharmacology, University of Western Australia, Harry Perkins Institute of Medical Research, QEII Medical Centre, Nedlands, Perth, Australia 6009 Department of Neurology, Sir Charles Gairdner Hospital, Nedlands, Perth, Australia Western Australian Neuroscience Research Institute, Nedlands, Perth, Australia

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Introduction: Postural instability is a major source of disability in idiopathic Parkinson's disease (IPD). Deep brain stimulation of the globus pallidus internus (GPI-DBS) improves clinician-rated balance control but there have been few quantitative studies of its interactive effects with levodopa (L-DOPA). The purpose of this study was to compare the short-term and interactive effects of GPI-DBS and L-DOPA on objective measures of postural stability in patients with longstanding IPD.

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Evidence for an excitatory GABAA response in human motor cortex in idiopathic generalised epilepsy.

Seizure

March 2015

Western Australian Neuroscience Research Institute, University of Western Australia, 4th Floor, A Block, QEII Medical Centre, Verdun Street, Nedlands, Perth, Western Australia 6009, Australia. Electronic address:

Purpose: Impaired GABAergic inhibition has been implicated in the pathophysiology of epilepsy. The possibility of a paradoxical excitatory effect of GABA in epilepsy has been suggested, but has not been investigated in vivo. We investigated pre- and post-synaptic GABAergic mechanisms in patients with idiopathic generalised epilepsy (IGE).

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Poly-arginine and arginine-rich peptides are neuroprotective in stroke models.

J Cereb Blood Flow Metab

June 2015

1] Centre for Neuromuscular and Neurological Disorders, The University of Western Australia, Nedlands, Western Australia, Australia [2] Department of Neurosurgery, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, Western Australia, Australia [3] Western Australian Neuroscience Research Institute, Nedlands, Western Australia, Australia.

Using cortical neuronal cultures and glutamic acid excitotoxicity and oxygen-glucose deprivation (OGD) stroke models, we demonstrated that poly-arginine and arginine-rich cell-penetrating peptides (CPPs), are highly neuroprotective, with efficacy increasing with increasing arginine content, have the capacity to reduce glutamic acid-induced neuronal calcium influx and require heparan sulfate preotoglycan-mediated endocytosis to induce a neuroprotective effect. Furthermore, neuroprotection could be induced with immediate peptide treatment or treatment up to 2 to 4 hours before glutamic acid excitotoxicity or OGD, and with poly-arginine-9 (R9) when administered intravenously after stroke onset in a rat model. In contrast, the JNKI-1 peptide when fused to the (non-arginine) kFGF CPP, which does not rely on endocytosis for uptake, was not neuroprotective in the glutamic acid model; the kFGF peptide was also ineffective.

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Background: The mirror neuron network provides a neural mechanism to prime the motor system through action observation in stroke survivors.

Objective: To examine whether action observation training with immediate physical practice improves upper-limb function in chronic stroke.

Methods: In a within-subject design, 14 chronic stroke survivors were assessed at baseline, then participated in 2 weeks of relaxation-sham plus physical practice (control) and reassessed.

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Helicobacter pylori infection as a protective factor against multiple sclerosis risk in females.

J Neurol Neurosurg Psychiatry

June 2015

Centre for Neuromuscular and Neurological Disorders, Western Australian Neuroscience Research Institute, The University of Western Australia, Sir Charles Gairdner Hospital, QEII Medical Centre, Nedlands, Australia Institute for Immunology and Infectious Diseases, Murdoch University, Murdoch, Australia.

Background: In recent years, a relationship between Helicobacter pylori and many disease conditions has been reported, however, studies in its relationship with multiple sclerosis (MS) have had contradictory results.

Objective: To determine the association between the H. pylori infection and MS.

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Is it a stroke?

BMJ

January 2015

School of Medicine and Pharmacology, University of Western Australia, Harry Perkins Institute of Medical Research, QEII Medical Centre, Nedlands, Perth, Western Australia, Australia 6009 Department of Neurology, Sir Charles Gairdner Hospital, Nedlands, Perth, Australia Western Australian Neuroscience Research Institute, Nedlands, Perth, Australia.

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Inclusion body myositis: a review of clinical and genetic aspects, diagnostic criteria and therapeutic approaches.

J Clin Neurosci

January 2015

Institute of Immunology and Infectious Diseases, Murdoch University, Murdoch, WA, Australia; Western Australian Neuroscience Research Institute, Queen Elizabeth II Medical Centre, Verdun Street, Nedlands, WA 6009, Australia.

Inclusion body myositis is the most common myopathy in patients over the age of 40 years encountered in neurological practice. Although it is usually sporadic, there is increasing awareness of the influence of genetic factors on disease susceptibility and clinical phenotype. The diagnosis is based on recognition of the distinctive pattern of muscle involvement and temporal profile of the disease, and the combination of inflammatory and myodegenerative changes and protein deposits in the muscle biopsy.

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An analysis of the sensitivity and specificity of MHC-I and MHC-II immunohistochemical staining in muscle biopsies for the diagnosis of inflammatory myopathies.

Neuromuscul Disord

December 2014

Section of Neuropathology, Department of Anatomical Pathology, PathWest Laboratory Medicine WA, Royal Perth Hospital, Perth, Western Australia, Australia; School of Pathology and Laboratory Medicine, University of Western Australia, Nedlands, Western Australia, Australia.

Although there have been several previous reports of immunohistochemical staining for MHC antigens in muscle biopsies, there appears to be a lack of consensus about its routine use in the diagnostic evaluation of biopsies from patients with suspected inflammatory myopathy. Positive MHC-I staining is nonspecific but is widely used as a marker for inflammatory myopathy, whilst the role of MHC-II staining is not clearly defined. We investigated the sensitivity and specificity of MHC-I and MHC-II immunostaining for the diagnosis of inflammatory myopathy in a large group of biopsies from a single reference laboratory.

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The role of interleukin-17 in immune-mediated inflammatory myopathies and possible therapeutic implications.

Neuromuscul Disord

November 2014

Institute for Immunology & Infectious Diseases (IIID), Murdoch University, Murdoch, WA, Australia; Western Australian Neuroscience Research Institute, Centre for Neuromuscular & Neurological Disorders, University of Western Australia, Australia.

The idiopathic inflammatory myopathies are a heterogeneous group of autoimmune muscle disorders with distinct clinical and pathological features and underlying immunopathogenic mechanisms. Traditionally, CD4(+) Th1 cells or CD8(+) cytotoxic effector T cells and type I/II interferons have been primarily implicated in the pathogenesis of the inflammatory myopathies. The presence of IL-17A producing cells in the inflamed muscle tissue of myositis patients and the results of in vitro studies suggest that IL-17A and the Th17 pathway may also have a key role in these diseases.

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Impaired functional communication between the L-type calcium channel and mitochondria contributes to metabolic inhibition in the mdx heart.

Proc Natl Acad Sci U S A

July 2014

School of Anatomy, Physiology and Human Biology, The University of Western Australia, Crawley, WA 6009, Australia;Victor Chang Cardiac Research Institute, Darlinghurst, NSW 2010, Australia

Duchenne muscular dystrophy is a fatal X-linked disease characterized by the absence of dystrophin. Approximately 20% of boys will die of dilated cardiomyopathy that is associated with cytoskeletal protein disarray, contractile dysfunction, and reduced energy production. However, the mechanisms for altered energy metabolism are not yet fully clarified.

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Dermatomyositis, polymyositis and immune-mediated necrotising myopathies.

Biochim Biophys Acta

April 2015

Western Australian Neuroscience Research Institute, University of Western Australia, Perth, Western Australia, Australia; Institute for Immunology & Infectious Diseases, Murdoch University, Perth, Western Australia, Australia. Electronic address:

Dermatomyositis, polymyositis and immune-mediated necrotising myopathy are major forms of idiopathic inflammatory myopathy. We review here recent developments in understanding the pathology and pathogenesis of these diseases, and characterisation of autoantibody biomarkers. Dermatomyositis is traditionally considered to be due to a complement-mediated microangiopathy but the factors responsible for complement activation remain uncertain.

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