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  1. Miura Y, Shahrizaila N, Yuki N
    Brain, 2015 Mar;138(Pt 3):e335.
    PMID: 25183714 DOI: 10.1093/brain/awu252
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  2. Tan CY, Shahrizaila N, Yeoh KY, Goh KJ, Tan MP
    Clin Auton Res, 2019 06;29(3):339-348.
    PMID: 29654380 DOI: 10.1007/s10286-018-0525-z
    OBJECTIVE: The current study aimed to investigate autonomic dysfunction in Guillain-Barré syndrome (GBS) patients and describe the results of computational heart rate variability (HRV)/baroreflex sensitivity (BRS) and autonomic challenge tests.

    METHODS: GBS patients were consecutively recruited and the results were compared to age- and gender-matched healthy controls. A series of autonomic function tests including computation-dependent tests (power spectrum analysis of HRV and BRS at rest) and challenge maneuvers (deep breathing, eyeball compression, active standing, the Valsalva maneuver, sustained handgrip, and the cold pressor test) were performed.

    RESULTS: Ten GBS patients (six men; mean age = 40.1 ± 13.9 years) and ten gender- and age-matched healthy controls were recruited. The mean GBS functional grading scale at disease plateau was 3.4 ± 1.0. No patients required intensive care unit admission or mechanical ventilation. Low-frequency HRV (p = 0.027), high-frequency HRV (p = 0.008), and the total power spectral density of HRV (p = 0.015) were significantly reduced in patients compared to controls. The mean up slope (p = 0.034), down slope (p = 0.011), and total slope (p = 0.024) BRS were significantly lower in GBS patients. The diastolic rise in blood pressure in the cold pressor test was significantly lower in GBS patients compared to controls (p = 0.008).

    INTERPRETATION: Computation-dependent tests (HRV and BRS) were more useful for detecting autonomic dysfunction in GBS patients, whereas the cold pressor test was the only reliable challenge test, making it useful as a bedside measure of autonomic function in GBS patients.

    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  3. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, et al.
    Nat Rev Neurol, 2019 11;15(11):671-683.
    PMID: 31541214 DOI: 10.1038/s41582-019-0250-9
    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 clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  4. Kumar V, Narayanan P, Shetty S, Mohammed AP
    BMJ Case Rep, 2021 Mar 01;14(3).
    PMID: 33649026 DOI: 10.1136/bcr-2020-240267
    COVID-19 is caused by the novel SARS-CoV-2 and is a potentially fatal disease that is of great global public health concern. In addition to respiratory symptoms, neurological manifestations have been associated with COVID-19. This is attributed to the neurotropic nature of coronaviruses. The authors present a case of Bell's palsy associated with COVID-19 in a term primigravida.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  5. Uncini A, Ippoliti L, Shahrizaila N, Sekiguchi Y, Kuwabara S
    Clin Neurophysiol, 2017 07;128(7):1176-1183.
    PMID: 28521265 DOI: 10.1016/j.clinph.2017.03.048
    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 evaluates duration of motor responses, sural sparing pattern and defines reversible conduction failure (RCF) in motor and sensory nerves at second study.

    RESULTS: At first study the misclassification error rates, compared to reference diagnoses, were: 15.3% for sparse LDA, 30% for our criteria, 45% for Rajabally's and 48% for Hadden's. Sparse LDA identified seven most powerful electrophysiological variables differentiating demyelinating and axonal subtypes and assigned to each patient the diagnostic probability of belonging to either subtype. At second study 46.6% of axonal GBS patients showed RCF in two motor and 8.8% in two sensory nerves.

    CONCLUSIONS: Based on a single study, sparse LDA showed the highest diagnostic accuracy. RCF is present in a considerable percentage of axonal patients.

    SIGNIFICANCE: Sparse LDA, a supervised statistical method of classification, should be introduced in the electrodiagnostic practice.

    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  6. Hiew FL, Ramlan R, Viswanathan S, Puvanarajah S
    Clin Neurol Neurosurg, 2017 Jul;158:114-118.
    PMID: 28514704 DOI: 10.1016/j.clineuro.2017.05.006
    OBJECTIVES: This study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes.

    PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al.'s vs recently proposed Rajabally et al.'s criteria. Functional severity and clinical outcome of various GBS subtypes were ascertained.

    RESULTS: All patients initially identified as GBS or related disorders can be sub-classified into having classical GBS (41, 67%), classic Miller-Fisher Syndrome (MFS) (6, 10%), Pharyngeal-cervical-brachial (PCB) (3, 5%), paraparetic GBS (4, 7%), bifacial weakness with paresthesia (3, 5%), acute ophthalmoparesis (AO) (1, 2%) and overlap syndrome (3, 5%): one (2%) with GBS/Bickerstaff brainstem encephalitis overlap and 2 (3%) with GBS/MFS overlap. Greater proportion of axonal classical GBS (67% vs 55%, p=0.372) seen with Rajabally et al.'s criteria and a predominantly axonal form of paraparetic variant (75%) independent of electrodiagnostic criteria were more representative of Asian GBS cohort. Classical GBS patients had lowest admission and discharge Medical Research Council Sum Score (MRCSS), greater functional disability and longest length of in-patient stay. Twenty (20/21, 95%) patients who needed mechanical ventilation had classical GBS. Patients required repeated dose of intravenous immunoglobulin (5/6, 3%) or plasma exchange (4/4, 100%) more frequently had axonal form of classical GBS.

    CONCLUSION: Phenotype recognition based on new GBS clinical classification, supported by electrodiagnostic study permits more precise clinical subtypes determination and outcome prognostication.

    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  7. Tan CY, Ahmad SB, Goh KJ, Latif LA, Shahrizaila N
    Neurol India, 2018 9 21;66(5):1475-1480.
    PMID: 30233023 DOI: 10.4103/0028-3886.241342
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  8. Shahrizaila N, Goh KJ, Kokubun N, Abdullah S, Yuki N
    J Neurol Sci, 2011 Oct 15;309(1-2):26-30.
    PMID: 21849173 DOI: 10.1016/j.jns.2011.07.042
    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 repeated NCS revealed the AMAN variant. This led us to investigate serial NCS in five patients with GBS, FS and FS/GBS overlap presenting over a period of a year. Three patients with AIDP showed a gradual increase in distal motor latencies during the acute phase of illness. NCS of two patients with FS and FS/GBS overlap showed no demyelinating features suggesting underlying axonal neuropathy in this group of patients. The importance of serial NCS in establishing the underlying pattern of neuropathy in GBS and FS is further emphasized in this study. Larger studies incorporating serial NCS are required to confirm the observations seen in our case series especially when pathological studies are often not justified in this group of patients.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  9. Hamidon BB
    Med J Malaysia, 2006 Jun;61(2):245-7.
    PMID: 16898323 MyJurnal
    Acute Guillain-Barre syndrome (GBS) is characterized by an acute onset of limb weakness and areflexia. There are a few rare variants that have been described and one of them is the pharyngeal-cervical-brachial (PCB) variant (oropharynx, neck, and proximal upper limb muscles). However, in this patient, the only presentation was bulbar involvement with fast recovery within days. This is likely to be the milder form of PCB that has rarely been described before. A 19-year-old Malay lady presented with progressive dysphagia associated with nasal voice for one week duration. There was no limb weakness. Examination showed generalized areflexia. Pharyngeal and palatal muscles were markedly weak. Cerebrospinal fluid (CSF) examination showed raised protein level. Nerve conduction studies revealed generalized demyelinating motor polyneuropathy consistent with GBS. The patient fully recovered within three days and was discharged well.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  10. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Peripher Nerv Syst, 2019 06;24(2):168-173.
    PMID: 31001904 DOI: 10.1111/jns.12320
    Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P 
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  11. Fong CY, Aung HWW, Khairani A, Gan CS, Shahrizaila N, Goh KJ
    Brain Dev, 2018 Jun;40(6):507-511.
    PMID: 29459060 DOI: 10.1016/j.braindev.2018.02.001
    Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  12. Ramanathan M
    Med J Malaysia, 2008 Dec;63(5):426-7.
    PMID: 19803310 MyJurnal
    This report deals with an elderly lady with Guillain-Barre Syndrome (GBS), who presented with features of unusually severe hyponatraemia. The hyponatraemia was probably due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The hyponatraemia resolved with water restriction and infusion of hypertonic saline; GBS was treated with human immunoglobulin (IVIG). This patient's experience stresses the importance of monitoring serum sodium levels as hyponatraemia has been identified to be a marker of poor prognosis in GBS.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  13. Shahrizaila N, Goh KJ, Abdullah S, Kuppusamy R, Yuki N
    Clin Neurophysiol, 2013 Jul;124(7):1456-9.
    PMID: 23395599 DOI: 10.1016/j.clinph.2012.12.047
    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.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  14. Tan CY, Razali SNO, Goh KJ, Shahrizaila N
    J Peripher Nerv Syst, 2020 09;25(3):256-264.
    PMID: 32511817 DOI: 10.1111/jns.12398
    We aimed to evaluate the key diagnostic features of Guillain-Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated. A total of 128 patients (95 GBS, 33 MFS) were included. In the GBS cohort, 92 (97%) patients presented with symmetrical limb weakness. Reflexes were depressed or absent in 90 (95%) patients. Almost all patients (94, 99%) followed a monophasic disease course, with 5 (5%) patients experiencing treatment-related fluctuations. Cerebrospinal fluid (CSF) albuminocytological dissociation was seen in 62/84 (73%) patients. Nerve conduction study (NCS) revealed neuropathy in 90/94 (96%) patients. In GBS patients with complete dataset (84), 56 (67%) patients reached level 1 of the Brighton criteria, 21 (25%) reached level 2, 3 (4%) reached level 3, and 4 (5%) reached level 4. In MFS, the clinical triad was present in 25 (76%) patients. All patients had a monophasic course. CSF albuminocytological dissociation was present in 10/25 (40%) patients. NCS was normal or showed sensory neuropathy in 25/33 (76%) patients. In MFS patients with complete dataset (25), 5 (20%) patients reached level 1 of the Brighton criteria, 14 (56%) reached level 2, 2 (8%) reached level 3, and 4 (16%) reached level 4. Inclusion of antiganglioside antibodies improved the sensitivity of the Brighton criteria in both cohorts. In the Malaysian cohort, the Brighton criteria showed a moderate to high sensitivity in reaching the highest diagnostic certainty of GBS, but the sensitivity was lower in MFS.
    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
  15. Tan CY, Sekiguchi Y, Goh KJ, Kuwabara S, Shahrizaila N
    Clin Neurophysiol, 2020 01;131(1):63-69.
    PMID: 31751842 DOI: 10.1016/j.clinph.2019.09.025
    OBJECTIVE: We aimed to develop a model that can predict the probabilities of acute inflammatory demyelinating polyneuropathy (AIDP) based on nerve conduction studies (NCS) done within eight weeks.

    METHODS: The derivation cohort included 90 Malaysian GBS patients with two sets of NCS performed early (1-20days) and late (3-8 weeks). Potential predictors of AIDP were considered in univariate and multivariate logistic regression models to develop a predictive model. The model was externally validated in 102 Japanese GBS patients.

    RESULTS: Median motor conduction velocity (MCV), ulnar distal motor latency (DML) and abnormal ulnar/normal sural pattern were independently associated with AIDP at both timepoints (median MCV: p = 0.038, p = 0.014; ulnar DML: p = 0.002, p = 0.003; sural sparing: p = 0.033, p = 0.009). There was good discrimination of AIDP (area under the curve (AUC) 0.86-0.89) and this was valid in the validation cohort (AUC 0.74-0.94). Scores ranged from 0 to 6, and corresponded to AIDP probabilities of 15-98% at early NCS and 6-100% at late NCS.

    CONCLUSION: The probabilities of AIDP could be reliably predicted based on median MCV, ulnar DML and ulnar/sural sparing pattern that were determined at early and late stages of GBS.

    SIGNIFICANCE: A simple and valid model was developed which can accurately predict the probability of AIDP.

    Matched MeSH terms: Guillain-Barre Syndrome/diagnosis*
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