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  1. Shahrizaila N, Goh KJ, Kokubun N, Tan AH, Tan CY, Yuki N
    Muscle Nerve, 2014 Apr;49(4):558-63.
    PMID: 23893512 DOI: 10.1002/mus.23973
    Differing patterns of neurophysiological abnormalities have been reported in patients with Fisher syndrome. Fisher syndrome is rare, and few series have incorporated prospective serial studies to define the natural history of nerve conduction studies in Guillain-Barré syndrome.
    Matched MeSH terms: Miller Fisher Syndrome/diagnosis*
  2. 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: Miller Fisher Syndrome/diagnosis
  3. Sim BNH, Joseph JP
    J R Coll Physicians Edinb, 2019 Dec;49(4):304-306.
    PMID: 31808458 DOI: 10.4997/JRCPE.2019.411
    Miller Fischer syndrome (MFS) is a variant of Guillain-Barré syndrome first described in 1956 and is characterised by the clinical triad of ophthalmoplegia, ataxia and areflexia. However, since its discovery, forme fruste and overlapping syndrome have been described. A forme fruste of MFS implies an attenuated form where not all of the clinical triad are present. In this report, a case of MFS is highlighted that was mistakenly treated as posterior circulation stroke, as well as the challenges faced in reaching the correct diagnosis and hence the appropriate treatment.
    Matched MeSH terms: Miller Fisher Syndrome/diagnosis*
  4. 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: Miller Fisher Syndrome/diagnosis*
  5. 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: Miller Fisher Syndrome/diagnosis*
  6. Al-Hakem H, Doets AY, Stino AM, Zivkovic SA, Andersen H, Willison HJ, et al.
    Neurology, 2023 Jun 06;100(23):e2386-e2397.
    PMID: 37076309 DOI: 10.1212/WNL.0000000000207282
    BACKGROUND AND OBJECTIVES: To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study.

    METHODS: Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/μL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%).

    RESULTS: In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/μL in 1,005 patients (83%), 5-49 cells/μL in 200 patients (16%), and ≥50 cells/μL in 13 patients (1%).

    DISCUSSION: ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/μL, is compatible with GBS after a thorough exclusion of alternative diagnoses.

    CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

    Matched MeSH terms: Miller Fisher Syndrome/diagnosis
  7. 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: Miller Fisher Syndrome/diagnosis
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