Miller Fisher syndrome（MFS）is a clinical variant of Guillain-Barre syndrome（GBS）and has the main clinical features of ataxia，ophthalmoplegia，and tendon areflexia，with pupil changes and abnormal pupillary light reflex in rare cases. There are generally no symptom fluctuations，and positive anti-GQ1b IgG antibodies can be detected in some patients. This article reports a case of MFS with positive anti-GQ1b，anti-GT1a，and anti-sulfatide antibodies and fluctuating extraocular muscle paralysis as the initial presentation，accompanied by bilateral pupil dilation，delayed light reflex，and numbness and weakness in the limbs. The symptoms are rare and atypical，which may easily lead to misdiagnosis in clinical practice.
Miller Fisher Syndrome ; Ophthalmoplegia

A boy, aged 1 year and 7 months, was hospitalized due to weakness in both lower limbs and blepharoptosis, which showed progressive aggravation and developed into irregular breathing. Neurological examinations showed lethargy, blepharoptosis, grade 4 muscle strength of both upper limbs, grade 3 muscle strength of both lower limbs, and disappearance of tendon reflex. Laboratory tests revealed albuminocytological dissociation in cerebrospinal fluid, disappearance of H reflex, and positive serum anti-GD1b IgG. The boy was finally diagnosed with Guillain-Barré syndrome (GBS) overlapping with Miller-Fisher syndrome and Bickerstaff brainstem encephalitis. He recovered and was discharged after treatment including immunoglobulin, plasma exchange, and respiratory support. The GBS overlap syndromes in children have strong clinical heterogeneity due to the injury of both peripheral nerve and brainstem, among which anti-GD1b antibody-related GBS overlap syndromes have special clinical manifestations and complex neuroelectrophysiological changes and are thus difficult to diagnose. Nerve conduction velocity tests, especially H reflex test, should be performed for children with weakness in both lower limbs and blepharoptosis.
Blepharoptosis ; Child ; Encephalitis ; Guillain-Barre Syndrome ; Humans ; Lower Extremity ; Male ; Miller Fisher Syndrome

BACKGROUND AND PURPOSE: The most-common initial manifestation of Miller Fisher syndrome (MFS) is diplopia due to acute ophthalmoplegia. However, few studies have focused on ocular motility findings in MFS. This study aimed to determine the pattern of extraocular muscle (EOM) paresis in MFS patients. METHODS: We consecutively recruited MFS patients who presented with ophthalmoplegia between 2010 and 2015. The involved EOMs and the strabismus pattern in the primary position were analyzed. Antecedent infections, other involved cranial nerves, and laboratory findings were also reviewed. We compared the characteristics of the patients according to the severity of ophthalmoplegia between complete ophthalmoplegia (CO) and incomplete ophthalmoplegia (IO). RESULTS: Twenty-five patients (15 males and 10 females) with bilateral ophthalmoplegia were included in the study. The most-involved and last-to-recover EOM was the lateral rectus muscle. CO and IO were observed in 11 and 14 patients, respectively. The patients were aged 59.0±18.4 years (mean±SD) in the CO group and 24.9±7.4 years in the IO group (p<0.01), and comprised 63.6% and 21.4% females, respectively (p=0.049). Elevated cerebrospinal fluid protein was identified in 60.0% of patients with CO and 7.7% of patients with IO (p=0.019) for a mean follow-up time from the initial symptom onset of 3.7 days. CONCLUSIONS: The lateral rectus muscle is the most-involved and last-to-recover EOM in ophthalmoplegia. The CO patients were much older and were more likely to be female and have an elevation of cerebrospinal fluid protein than the IO patients.
Cerebrospinal Fluid ; Cranial Nerves ; Diplopia ; Female ; Follow-Up Studies ; Guillain-Barre Syndrome ; Humans ; Jupiter ; Male ; Miller Fisher Syndrome ; Ophthalmoplegia ; Paresis ; Strabismus

Guillain-Barré syndrome (GBS) is a representative form of post-infectious autoimmune neuropathy with heterogenous manifestations. It was originally considered as an ascending demyelinating polyneuropathy in Western countries. However, the discovery of anti-ganglioside antibodies on the basis of molecular mimicry theory could help us better understand various kinds of focal and regional variants as well as axonal type of GBS those were frequently found from Asian countries. Recent development of new techniques about anti-ganglioside complex antibodies is making more detailed descriptions for specific or unusual clinical manifestations. It has been regarded that GBS has good prognosis if treated properly as early as possible, but it still shows high mortality and morbidity rate with frequent long term neurologic and medical complications. Unfortunately, there are only two options for medical treatment, intravenous immunoglobulin and plasmapheresis, for the last 100 years. Several clinical studies on new immunotherapy targeting complement activating system with background of molecular mimicry using animal model are underway. We hope that these new treatments will be helpful for the future patients.
Antibodies ; Asian Continental Ancestry Group ; Axons ; Complement System Proteins ; Gangliosides ; Guillain-Barre Syndrome ; Hope ; Humans ; Immunoglobulins ; Immunotherapy ; Miller Fisher Syndrome ; Models, Animal ; Molecular Mimicry ; Mortality ; Plasmapheresis ; Polyneuropathies ; Prognosis

Bilateral internuclear ophthalmoplegia (INO) refers to a specific gaze abnormality of bilateral adduction deficits, often accompanied by dissociated abducting nystagmus, caused by medial longitudinal fasciculus lesions usually due to multiple sclerosis or stroke. We report a 63-year-old man with clinical features of Miller-Fisher syndrome (MFS), consisting of ataxia, areflexia, and external ophthalmoplegia which mimicked bilateral-INO without an identifiable central lesion. Although bilateral adduction deficits are usually caused by central lesions, peripheral nervous damage by MFS is needed to be considered.
Ataxia ; Humans ; Middle Aged ; Miller Fisher Syndrome ; Multiple Sclerosis ; Ocular Motility Disorders ; Ophthalmoplegia ; Stroke

BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.
Adult ; Antibodies ; Bulbar Palsy, Progressive ; Gangliosides ; Guillain-Barre Syndrome ; Humans ; Miller Fisher Syndrome

Introduction: Miller Fisher syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS) characterized by an immunemediated polyneuropathy. Diagnosis is largely clinical and spontaneous recovery is observed in most cases. Treatment options such as IVIg, plasmapheresis, and steroids have been studied as options to shorten the disease course, but with inconclusive results. Case: A 25-year-old male complained of sudden onset diplopia, gait instability and hand parasthesia. Diagnosis of MFS was done clinically; chest CT scan, nerve conduction studies, and MRI of brain and orbits were unremarkable. Anti-GQ1b determination was not performed. Low dose oral corticosteroid was initiated with gradual recovery of symptoms noted over two weeks and full recovery in two months. Discussion: Miller Fisher syndrome (MFS) is a rare entity and the least common of the GBS variants. Its incidence as a proportion of GBS accounts for one to five percent in Western countries. Most patients have evidence of an upper respiratory tract infection one to three weeks before symptom onset. MFS is largely considered to be a self-limiting condition, but case series have shown that patients return to normal activities approximately six months after neurological onset. The patient in this report was treated with low dose steroids, with gradual taper over two months. Significant improvement of symptoms was noted over two months, which is shorter than the six months recovery in literature. Conclusion Worldwide incidence of MFS can be underestimated as it is often overlooked during the initial work-up of the disease. The risks of treatment, therefore, should be weighed against the likelihood of spontaneous recovery. Although use of steroids in this case report have noticeably caused a shorter course of the disease, prospective studies are suggested to look into the role of low dose oral corticosteroids in shortening the onset-to-recovery course of this illness.
Miller Fisher Syndrome ; Diplopia ; Steroids

PURPOSE: This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination. METHODS: A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%). RESULTS: Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability. CONCLUSION: Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.
Adrenal Cortex Hormones ; Child* ; Demyelinating Diseases* ; Encephalomyelitis, Acute Disseminated ; Guillain-Barre Syndrome ; Hospitalization ; Humans ; Immunoglobulins ; Intensive Care Units ; Miller Fisher Syndrome ; Myelitis, Transverse ; Nervous System ; Neuroimaging ; Optic Neuritis ; Peripheral Nervous System ; Plasmapheresis ; Retrospective Studies ; Steroids

PURPOSE: In the present study, the clinical characteristics and prognosis of patients clinically diagnosed with classic Miller Fisher syndrome were evaluated. METHODS: We retrospectively investigated the clinical and laboratory findings as well as treatment outcomes using the medical records of patients diagnosed with Miller Fisher syndrome. Symptom triad including acute ophthalmoplegia, ataxia, and areflexia were evaluated. RESULTS: This study included 10 patients. Nine patients had antecedent infectious illness which took an average of 11 ± 9.7 days for onset of diplopia from antecedent infectious systemic illness. Seven patients showed bilateral paralytic strabismus. Specifically, 5 patients showed the involvement of vertical and horizontal extraocular muscles. Pupil impairment and blepharoptosis were observed in 4 patients, limb weakness in 3 patients, dysarthria in 3 patients and facial palsy in 1 patient. Two patients showed contrast enhancement of the abducens nerve on brain magnetic resonance imaging (MRI) and 2 patients showed albumin-cell dissociation on cerebrospinal fluid (CSF) analysis. Eight patients had anti-GQ1b antibodies in their blood serum analysis. Six patients were treated with intravenous immunoglobulins and the other patients were observed with regular follow-ups. The duration of diplopia was 2.9 ± 1.2 months in the treatment group and 3.1 ± 1.7 months in the control group (p > 0.05). The duration of ataxia was 1 ± 0.4 months in the treatment group and 1 ± 0.9 months in the control group (p > 0.05). CONCLUSIONS: Miller Fisher syndrome should be considered in patients with antecedent infection; acute ophthalmoplegia, ataxia and areflexia as well as anti-GQ1b antibody can be helpful for diagnosis. Final outcomes in the treated group were not significantly different from the control group and all patients showed good final outcomes.
Abducens Nerve ; Antibodies ; Ataxia ; Blepharoptosis ; Brain ; Cerebrospinal Fluid ; Diagnosis ; Diplopia ; Dysarthria ; Extremities ; Facial Paralysis ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; Magnetic Resonance Imaging ; Medical Records ; Miller Fisher Syndrome* ; Muscles ; Ophthalmoplegia ; Prognosis* ; Pupil ; Retrospective Studies ; Serum ; Strabismus

PURPOSE: To report a case of Miller Fisher syndrome in a pediatric patient with gastroenteritis associated with seroconversion of Campylobacter jejuni titer during the development of neurological symptoms and positive anti-GQ1b IgG. CASE SUMMARY: An 8-year-old male patient visited our clinic with bilateral ophthalmoplegia, diplopia, and ptosis of the right upper lid. He had experienced gastroenteritis one week previous, and antibodies to Campylobacter jejuni were detected in his plasma. Ophthalmic examination revealed a corrected visual acuity of 20/20 in both eyes. Ocular motor examination revealed limitations in all positions of gaze. Neurologic examination demonstrated areflexia and ataxia. The serologic anti-GQ1b IgG test was positive. Intravenous immunoglobulin and steroid pulse therapy were started. Extraocular movement, ptosis, and ataxia gradually improved after one month of treatment. CONCLUSIONS: We confirmed a case of Miller Fisher syndrome in a pediatric patient with bilateral ophthalmoplegia, ptosis, and a positive anti-GQ1b antibody test.
Antibodies ; Ataxia ; Campylobacter jejuni ; Child ; Diplopia ; Gastroenteritis ; Humans ; Immunoglobulin G* ; Immunoglobulins ; Male ; Miller Fisher Syndrome* ; Neurologic Examination ; Ophthalmoplegia ; Plasma ; Visual Acuity