 | Nortina Shahrizaila |
Prominent publications by Nortina Shahrizaila
Activating mutations in STIM1 and ORAI1 cause overlapping syndromes of tubular myopathy and congenital miosis
[ PUBLICATION ]
Signaling through the store-operated Ca(2+) release-activated Ca(2+) (CRAC) channel regulates critical cellular functions, including gene expression, cell growth and differentiation, and Ca(2+) homeostasis. Loss-of-function mutations in the CRAC channel pore-forming protein ORAI1 or the Ca(2+) sensing protein stromal interaction molecule 1 (STIM1) result in severe immune dysfunction and nonprogressive myopathy. Here, we identify gain-of-function mutations in the cytoplasmic domain of ...
| Also Ranks for: Congenital Miosis | crac channel | stormorken syndrome | stim1 orai1 | dyslexia erythrocytes |
In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. In the cases with Bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support of a central pathology whereas in Fisher syndrome, patients were areflexic in keeping with a ...
| Also Ranks for: Fisher Syndrome | brainstem encephalitis | gq1b antibody | acute ophthalmoparesis | antecedent infection |
Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study ...
| Also Ranks for: Barré Syndrome | child child | clinical presentation | electrophysiological subtype | outcome study |
Serial nerve conduction studies provide insight into the pathophysiology of Guillain–Barré and Fisher syndromes
[ PUBLICATION ]
The electrodiagnosis of Guillain-Barré syndrome (GBS) can be broadly divided into acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Fisher syndrome (FS) is a variant of GBS, although the underlying neuropathy of FS has yet to be established. Serial nerve conduction studies (NCS) can provide further insight into the likely pathophysiology by further subtyping of GBS and FS. We present a patient with an initial diagnosis of AIDP in whom ...
| Also Ranks for: Fisher Syndrome | patients gbs | conduction studies | serial ncs | acute phase |
Guillain-Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international ...
| Also Ranks for: Management Gbs | barré syndrome | clinical presentation | peripheral nerves | latin america |
Validation of the Malay Version of Addenbrooke’s Cognitive Examination III in Detecting Mild Cognitive Impairment and Dementia
[ PUBLICATION ]
Background/Aims: This study aimed to investigate the validity and reliability of the Malay version of Addenbrooke's Cognitive Examination III (ACE-III) for detecting mild cognitive impairment (MCI) and dementia.
Methods: A total of 152 participants (dementia = 53, MCI = 38, controls = 61) were recruited from two teaching hospitals. The Malay version of ACE-III was translated following the standard guidelines for cross-cultural adaptation of measure. All the participants were assessed ...
| Also Ranks for: Mild Cognitive Impairment | aceiii dementia | cognitive examination | iii ace | total score |
Two sets of nerve conduction studies may suffice in reaching a reliable electrodiagnosis in Guillain–Barré syndrome
[ PUBLICATION ]
OBJECTIVE: Recent studies have advocated the use of serial nerve conduction studies (NCS) in the electrodiagnosis of Guillain-Barré syndrome (GBS). The current study aims to elucidate when and how frequent NCS can be performed to reflect the disease pathophysiology.
METHODS: A prospective study of GBS patients documenting the initial and final electrodiagnoses following serial NCS performed at three time intervals: 1-2 weeks, 3-8 weeks and 8-12 weeks.
RESULTS: Twenty-one patients were ...
| Also Ranks for: Barré Syndrome | conduction studies | gbs patients | electrodiagnosis guillain | 2 years |
Peripheral neuropathy is a known manifestation of systemic lupus erythematosus. However, the association of primary autoimmune inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) with SLE is uncommon. We report a 26-year-old man who simultaneously presented with severe CIDP and photosensitive rash, but was unresponsive to intravenous immunoglobulin infusion and continued to progress. He was found to have underlying SLE and improved with combined ...
| Also Ranks for: Chronic Inflammatory | systemic lupus erythematosus | cidp sle | demyelinating polyneuropathy | immunosuppressive agents |
Antibodies to single glycolipids and glycolipid complexes in Guillain-Barré syndrome subtypes
[ PUBLICATION ]
OBJECTIVE: To comprehensively investigate the relationship between antibodies to single glycolipids and their complexes and Guillain-Barré syndrome subtypes and clinical features.
METHODS: In acute sera from 199 patients with Guillain-Barré syndrome, immunoglobulin G (IgG) antibodies to glycolipids and ganglioside complexes were tested using ELISA against individual antigens from single glycolipids including gangliosides (LM1, GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, GT1a, GT1b, GQ1b) and a ...
| Also Ranks for: Immunoglobulin Igg | ganglioside complexes | acute motor | axonal neuropathy | antibodies gm1 |
Guillain-Barré Syndrome Animal Model: The First Proof of Molecular Mimicry in Human Autoimmune Disorder
[ PUBLICATION ]
Molecular mimicry between self and microbial components has been proposed as the pathogenic mechanism of autoimmune diseases, and this hypothesis is proven in Guillain-Barré syndrome. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, sometimes occurs after Campylobacter jejuni enteritis. Gangliosides are predominantly cell-surface glycolipids highly expressed in nervous tissue, whilst lipo-oligosaccharides are major components of the Gram-negative ...
| Also Ranks for: Molecular Mimicry | barré syndrome | human autoimmune | animal model | gm1 ganglioside |
Anti‐GQ1b antibody syndrome: anti‐ganglioside complex reactivity determines clinical spectrum
[ PUBLICATION ]
BACKGROUND AND PURPOSE: Anti-GQ1b antibodies have been found in patients with Miller Fisher syndrome as well as its related conditions. Our aim was to identify the mechanism by which autoantibodies produce various clinical presentations in 'anti-GQ1b antibody syndrome'.
METHODS: Immunoglobulin G antibodies to ganglioside complex (GSC) of GQ1b or GT1a with GM1, GD1a, GD1b or GT1b were tested in sera from patients with anti-GQ1b (n = 708) or anti-GT1a (n = 696) IgG antibodies. Optical ...
| Also Ranks for: Clinical Spectrum | anti gq1b | ganglioside complex | miller fisher syndrome | ophthalmoplegia ataxia |
Optimizing the electrodiagnostic accuracy in Guillain-Barré syndrome subtypes: Criteria sets and sparse linear discriminant analysis
[ PUBLICATION ]
OBJECTIVE: To optimize the electrodiagnosis of Guillain-Barré syndrome (GBS) subtypes at first study.
METHODS: The reference electrodiagnosis was obtained in 53 demyelinating and 45 axonal GBS patients on the basis of two serial studies and results of anti-ganglioside antibodies assay. We retrospectively employed sparse linear discriminant analysis (LDA), two existing electrodiagnostic criteria sets (Hadden et al., 1998; Rajabally et al., 2015) and one we propose that additionally ...
| Also Ranks for: Electrodiagnostic Accuracy | syndrome subtypes | criteria sets | serial studies | single study |
OBJECTIVE: To assess the longitudinal changes of nerve ultrasound in Guillain-Barré syndrome (GBS) patients.
METHODS: We prospectively recruited 17 GBS patients and 17 age and gender-matched controls. Serial studies of their nerve conduction parameters and nerve ultrasound, documenting the cross-sectional areas (CSA), were performed at admission and repeated at several time points throughout disease course.
RESULTS: Serial nerve ultrasound revealed significantly enlarged CSA in median, ...
| Also Ranks for: Nerve Ultrasound | barré syndrome | disease onset | conduction peripheral | guillain barre |
Different IVIG Glycoforms Affect In Vitro Inhibition of Anti-Ganglioside Antibody-Mediated Complement Deposition
[ PUBLICATION ]
Intravenous immunoglobulin (IVIG) is the first line treatment for Guillain-Barré syndrome and multifocal motor neuropathy, which are caused by anti-ganglioside antibody-mediated complement-dependent cytotoxicity. IVIG has many potential mechanisms of action, and sialylation of the IgG Fc portion reportedly has an anti-inflammatory effect in antibody-dependent cell-mediated cytotoxicity models. We investigated the effects of different IVIG glycoforms on the inhibition of antibody-mediated ...
| Also Ranks for: Complement Deposition | ivig treatment | intravenous immunoglobulin | anti ganglioside | sialylated igg |
Survival and prognostic factors of motor neuron disease in a multi-ethnic Asian population
[ PUBLICATION ]
Our objective was to determine the survival and prognostic factors of motor neuron disease (MND) in a multi-ethnic cohort of Malaysian patients. All patients seen at a university medical centre between January 2000 and December 2009 had their case records reviewed for demographic, clinical and follow-up data. Mortality data, if unavailable from records, were obtained by telephone interview of relatives or from the national mortality registry. Of the 73 patients, 64.4% were Chinese, 19.2% ...
| Also Ranks for: Motor Neuron Disease | age onset | asian population | mortality data | case records |
Nortina Shahrizaila: Influence Statistics
| Concept | World rank |
|---|---|
| grade cmtns | #1 |
| dti parameters cmt | #1 |
| grade sciatic | #1 |
| anisotropy charcot | #1 |
| grades cmtns | #1 |
| dti muscle atrophy | #1 |
| cmt correlations | #1 |
| muscle atrophy grade | #1 |
| cmtns 005 | #1 |
| dti cmt | #1 |
| muscle atrophy grades | #1 |
| relationship dti parameters | #1 |
| cmt dti | #1 |
| cmtns study | #1 |
| aoc emeac | #2 |
| paediatric bbe | #2 |
| p245l orai1 | #2 |
| megos egos | #2 |
| dsp chronic inflammatory | #2 |
| require concerns | #2 |
| 001 egos | #2 |
| outcome erasmus | #2 |
| mfs megos | #2 |
| egris predict | #2 |
| aoc neurology | #2 |
| structural child dna | #2 |
| nerve ultrasound neuropathies | #2 |
| months egos | #2 |
| egos mfs | #2 |
| admission egris | #2 |
| egos megos | #2 |
| miosis signaling | #2 |
| egris gbs patients | #2 |
| ddsp patients | #2 |
| mfs egris | #2 |
| markedly larger nerves | #2 |
| months megos | #2 |
| africa emeac | #2 |
| emeac aoc | #2 |
| miosis tubular | #2 |
| emeac neurology | #2 |
| aoc clinical neurophysiology | #2 |
| tubular myopathy patients | #2 |
| ifcn survey | #2 |
| oceania chapters | #2 |
| admission egos | #2 |
| emeac | #2 |
| megos mechanical | #2 |
| performed competence | #2 |
| egos patients | #2 |
Key People For Barré Syndrome
Nortina Shahrizaila:Expert Impact
Concepts for whichNortina Shahrizailahas direct influence:Barré syndrome, Amyotrophic lateral sclerosis, Fisher syndrome, Neuromuscular ultrasound, Nerve ultrasound, Brighton criteria, Guillainbarré syndrome, Chronic inflammatory.
Nortina Shahrizaila:KOL impact
Concepts related to the work of other authors for whichfor which Nortina Shahrizaila has influence:Barré syndrome, Amyotrophic lateral sclerosis, Zika virus, Chronic inflammatory, Patients gbs, Intravenous immunoglobulin, Molecular mimicry.
Tools
Is this your profile? Claim your profile Copy URL Embed Link to your profile |