Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients

Hu, Qiaoxia; Li, Hongfu; Tian, Jun; Zhang, Baorong

doi:10.1007/s10072-017-3184-2

Cited by 5 publications

(2 citation statements)

References 24 publications

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“…Few studies have addressed the AGAbs of overlapping MFS/GBS, most of which are case reports or small studies. Sekiguchi et al indicated that the IgG of anti-GQ1b, anti-GM1, and/or anti-GD1a existed in 73.9% (17/23) and in 21% (5/23) of overlapping MFS/GBS patients, respectively, in a study with a sample size of 33 [ 9 ]. A study from the Lisette Bazán-Rodríguez group showed that anti-GQ1b antibodies were positive in 60% (2/4) of patients with overlapping MFS/GBS [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

Only anti-GM4 antibody positivity in a Chinese girl with overlapping MFS/GBS: a case report

Chen,

Tian,

Shu

2024

Neurol Sci

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Background Guillain-Barré syndrome (GBS), as the most common cause of acute flaccid paralysis worldwide, is considered a part of a clinical spectrum in which discrete, complete, or incomplete forms of GBS and overlapping syndromes lie on the basis of their clinical features. The term overlapping Miller Fisher syndrome (MFS)/GBS is used when patients with MFS also suffer from progressive motor weakness of the limbs. Anti-ganglioside GQ1b has been specifically associated with MFS and ophthalmoplegia. Case description Here, we report a Chinese girl who was diagnosed with overlapping MFS/GBS showing acute flaccid paralysis of all four limbs, sensory symptoms, cranial nerve dysfunction, autonomic involvement, ophthalmoplegia, and ataxia. She had high serum and cerebrospinal fluid titres of monospecific anti-GM4 IgG antibody instead of anti-GQ1b antibody in the acute phase. Conclusion Anti-GM4 antibodies usually coexist with other antiganglioside antibodies, leading to missed diagnoses. The findings of the present study show that antibodies to ganglioside GM4 may in overlapping MFS/GBS as the lone immunological factors.

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Section: Discussionmentioning

confidence: 99%

Only anti-GM4 antibody positivity in a Chinese girl with overlapping MFS/GBS: a case report

Chen,

Tian,

Shu

2024

Neurol Sci

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“…Since the mEGRIS was developed based on this more representative cohort, it has a broader applicability and can also be used in mildly affected patients and GBS variants. Although, the need for MV in MFS patients is exceptional, it is not fully ruled out when these patients develop severe bulbar palsy or transit to MFS-GBS overlap (limb weakness),29 which are both represented in the mEGRIS.…”

Section: Discussionmentioning

confidence: 99%

Modified Erasmus GBS Respiratory Insufficiency Score: a simplified clinical tool to predict the risk of mechanical ventilation in Guillain-Barré syndrome

Luijten

Doets

Arends

et al. 2022

J Neurol Neurosurg Psychiatry

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BackgroundThis study aimed to determine the clinical and diagnostic factors associated with mechanical ventilation (MV) in Guillain-Barré syndrome (GBS) and to simplify the existing Erasmus GBS Respiratory Insufficiency Score (EGRIS) for predicting the risk of MV.MethodsData from the first 1500 patients included in the prospective International GBS Outcome Study (IGOS) were used. Patients were included across five continents. Patients <6 years and patients from Bangladesh were excluded. Univariable logistic and multivariable Cox regression were used to determine which prespecified clinical and diagnostic characteristics were associated with MV and to predict the risk of MV at multiple time points during disease course.Results1133 (76%) patients met the study criteria. Independent predictors of MV were a shorter time from onset of weakness until admission, the presence of bulbar palsy and weakness of neck flexion and hip flexion. The modified EGRIS (mEGRIS) was based on these factors and accurately predicts the risk of MV with an area under the curve (AUC) of 0.84 (0.80–0.88). We internally validated the model within the full IGOS cohort and within separate regional subgroups, which showed AUC values of 0.83 (0.81–0.88) and 0.85 (0.72–0.98), respectively.ConclusionsThe mEGRIS is a simple and accurate tool for predicting the risk of MV in GBS. Compared with the original model, the mEGRIS requires less information for predictions with equal accuracy, can be used to predict MV at multiple time points and is also applicable in less severely affected patients and GBS variants. Model performance was consistent across different regions.

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Atypical clinical manifestations of Miller Fisher syndrome

Jung

Shin

et al. 2018

Neurol Sci

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Miller Fisher syndrome (MFS) is characterized by a clinical triad of ophthalmoplegia, ataxia, and areflexia, and is closely associated with serum anti-GQ1b antibody. Although the clinical triad is the cardinal diagnostic clue, a variety of other symptoms and signs beyond the triad have been reported. To elucidate the frequency and characteristics of atypical clinical manifestations of MFS, we recruited 38 patients with MFS and evaluated the symptoms or signs beyond the classic triad. Eleven (29%) of 38 patients had atypical clinical manifestations of MFS such as headache (n = 6), delayed facial palsy (n = 3), divergence insufficiency (n = 2), and taste impairment (n = 2). Headache was localized to the periorbital (n = 3), temporal (n = 2), or whole (n = 1) area. Only one of them showed bilateral papilledema and an elevated opening pressure in cerebrospinal fluid analysis. Delayed facial palsy developed after the other signs have reached nadir (n = 1) or started to improve (n = 2), and did not follow a pattern of descending paralysis with other cranial neuropathies. Two patients showed divergence insufficiency without external ophthalmoplegia, and another two had taste impairment over the entire tongue without the other signs of facial and glossopharyngeal nerve involvements. Our study shows that approximately 30% of MFS patients can have atypical clinical manifestations beyond the classic triad. These results reflect the broad clinical spectrum of MFS, and might be associated with the presence of additional antiganglioside antibodies besides anti-GQ1b in patients with MFS.

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Bulbar paralysis associated with Miller-Fisher syndrome and its overlaps in Chinese patients

Cited by 5 publications

References 24 publications

Only anti-GM4 antibody positivity in a Chinese girl with overlapping MFS/GBS: a case report

Only anti-GM4 antibody positivity in a Chinese girl with overlapping MFS/GBS: a case report

Modified Erasmus GBS Respiratory Insufficiency Score: a simplified clinical tool to predict the risk of mechanical ventilation in Guillain-Barré syndrome

Atypical clinical manifestations of Miller Fisher syndrome

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