Here we present a case of a child presenting with incomplete Parinaud’s syndrome as the initial manifestation of MFS. [ejnpn.springeropen.com]

Case presentation A 76-year-old white man presented to our emergency department (ED) with recent onset of unsteady gait, poor oral intake, dysarthria, and dizziness for 1 week prior to presentation. [jmedicalcasereports.biomedcentral.com]

An elevated protein value was present in 134 patients (64.4%); cerebrospinal fluid findings were normal in 56 patients. Eighteen patients showed a mild pleocytosis. [collections.lib.utah.edu]

Very rarely, MFS can present with a recurrent course. [ncbi.nlm.nih.gov]

• Disability

  In the episode reported here, our patient showed disturbed consciousness and an extensor response to cutaneous plantar stimulation was observed (BBE symptoms), with severe disability and requirement for artificial respiration management. [ncbi.nlm.nih.gov]

  The prognosis of the disease is often benign, but disabling or even fatal outcome is possible. Corticosteroid treatment, possibly because of antiinflammatory and/or immunosuppressive action, could be effective in some patients. [moh-it.pure.elsevier.com]

  Twenty-eight days after her admission, she recovered to the point of walking without any aid and was discharged with minimal disability. [jkna.org]

  He was seriously disabled, mainly because of truncal and limb ataxia. Somatosensory evoked-potential examination revealed no response to electric stimulation. [ajnr.org]

  Treatment and prognosis Although most cases of Miller Fisher syndrome have a good prognosis, occasional cases with permanent neurological disability are encountered. Fatal progression has also been described but is uncommon (<5%) 1,7. [radiopaedia.org]

• Streptococcal Infection

  J Infect Dis 176(Suppl 2): S150–S153 Google Scholar 29. Yuki N, Hirata K (1998) Fisher’s syndrome and group A streptococcal infection. J Neurol Sci 160: 64–66 CrossRef PubMed Google Scholar 30. [link.springer.com]

  Fisher’s syndrome and group A streptococcal infection. J Neurol Sci 1998;160:64–66. ↑ Bennetto LP, Lyons P. Miller Fisher syndrome associated with Pasteurella multocida infection. J Neurol Neurosurg Psychiatry 2004;75:1786–1797 ↑ Takano H, Yuki N. [eyewiki.org]

• Hodgkin Lymphoma

  lymphoma Rarely, influenza vaccinations or childhood vaccinations COVID-19 virus COVID-19 Johnson & Johnson and AstraZeneca vaccine Complications Guillain-Barre syndrome affects your nerves. [mayoclinic.org]

• Quickening

  Retrospective research has found that intravenous immunoglobulin (IVIG) does not have a major impact on outcomes, though it does quicken the onset of recovery from ophthalmoplegia by a small margin (12.0 days after symptom onset compared to 13.5 days) [eyewiki.org]

• Diplopia

  Miller Fisher syndrome (MFS) is a rare immune-mediated neuropathy that commonly presents with diplopia after the acute onset of complete bilateral external ophthalmoplegia. [ncbi.nlm.nih.gov]

  Last neurological examination, performed 2 months earlier, revealed only mild diplopia on left lateral gaze after prolonged fixation. [frontiersin.org]

  On the fourth day, he woke up with binocular, oblique diplopia and was unable to move his eyes in any direction. Thinking the diplopia was related to starting the prednisone, he stopped taking it. [eyerounds.org]

• Blurred Vision

  Recently, we encountered a woman with MFS whose initial manifestation was blurred vision because of bilateral tonic pupils. [ncbi.nlm.nih.gov]

  Case presentation A previously healthy 13-year-old girl presented with complaints of blurred vision and diplopia in the 24 h preceding the initial examination. Her symptoms were not persistent and fluctuated during the day. [ejnpn.springeropen.com]

  Miller Fisher Syndrome (MFS), also called Fisher’s syndrome, usually begins with the rapid development, over days, of 3 problems: weak eye muscles, with double or blurred vision, and often drooping eyelids with facial weakness; poor balance and coordination [gbs-cidp.org]

• Photophobia

  Upon further questioning, the patient denied weakness in the face, pain with eye movement, pain with neck movement, halos, photophobia, hand weakness, numbness or tingling sensations in the extremities, and breathing difficulty. [consultant360.com]

  Other less frequent signs and symptoms of MFS include dysesthesias, ptosis, facial weakness, limb weakness, dysphagia, headache, optic neuritis, photophobia, blurred vision, and periocular pain. [eyerounds.org]

  Less common symptoms include consciousness disturbance, blepharoptosis, limb weakness, bulbar dysfunction, dysphagia, photophobia, dizziness, blurry vision, headache, and facial weakness.[2] Signs MFS classically presents with three signs: ophthalmoplegia [eyewiki.org]

  The patient returned five days later after completing his oral azithromycin course and stated he experienced no improvement in the distance diplopia; in addition, he experienced the onset of significant photophobia and periocular pain around both eyes [reviewofoptometry.com]

• Miosis

  Five days later, a neuro-ophthalmologic examination showed bilateral eye adduction weakness with an upgaze palsy, along with a mild left eye ptosis and miosis. Direct and consensual reaction of pupils was intact. [nnjournal.net]

  […] external ophthalmoplegia with positive anti-GQ1b antibodies.[11],[12],[13],[14],[15] Radziwill et al., demonstrated the presence of denervation supersensitivity of the pupillary sphincter muscles by testing with dilute pilocarpine (0.1%), which induced miosis [neurologyindia.com]

• Ataxia

  Neuro-ophthalmic manifestations of the syndrome of ophthalmoplegia, ataxia, and areflexia: observations on 20 patients. Acta Neurol Scand 1994;89:87-94. 11. Yip P. Bilateral ptosis, ataxia and areflexia – a variant of Fisher’s syndrome. [neuroophthalmology.ca]

  First symptoms were diplopia (38.6%) or ataxia (20.6%). An areflexia was present in 81.6% of cases. [collections.lib.utah.edu]

  Initially he presented with partial ophthalmoplegia, ataxia. Second episode was characterized by full-blown presentation characterized by ataxia, areflexia and ophthalmoplegia. CSF analysis was typical during both episodes. [ncbi.nlm.nih.gov]

• Stroke

  Circulating intravenous immunoglobulins combined with a low blood pressure was considered the most probable cause of his stroke. [ncbi.nlm.nih.gov]

  (Adams et al., Principles of Neurology, 6th ed, p1313; Neurology 1987 Sep;37(9):1493-8) Miller Fisher Syndrome: factsheet from NINDS, the National Institute of Neurological Disorders and Stroke (US site). [ucl.ac.uk]

  Neurological Disorders and Stroke » [brainfacts.org]

  Paralysis in other forms of GBS typically begins in the legs.1-3 Moreover, MFS is characterized by a triad of conditions: areflexia, ataxia, and ophthalmoplegia.2 The present report describes the case of a man with risk factors for stroke. [jaoa.org]

• Headache

  We report a 35-year-old man with anti-GQ(1b) antibody-confirmed Miller Fisher syndrome presenting with severe and persistent headache, and we propose that the headache is caused by antibody-mediated effects on the trigeminovascular pain pathway. [ncbi.nlm.nih.gov]

  […] our case does not enable us to consider diving a predisposing factor, we do think that it could facilitate and probably cause other associated symptoms (headache) that were unclear upon initial diagnosis. [elsevier.es]

  Early symptoms include fever, headache, and pneumonia. They are followed by facial paralysis, diplopia, external ophthalmoplegia, and paresthesia of the arms and trunk. Reported in male patients between 38 and 65 years of age. [whonamedit.com]

• Gait Ataxia

  A further review of the patient 3 months later showed complete resolution of the gait ataxia. [cerebellumandataxias.biomedcentral.com]

  We herein report the case of a 53-year-old man with cerebellar ataxia with anti-glutamic acid decarboxylase antibody (GAD-Ab) who mimicked Miller Fisher syndrome (MFS). He developed ophthalmoplegia, diplopia, and gait ataxia for one week. [ncbi.nlm.nih.gov]

  He was treated with two courses of intravenous immunoglobulins and made a good recovery, only displaying mild gait ataxia when reviewed in clinic 2.5 months later. [eprints.whiterose.ac.uk]

• Nystagmus

  However, nystagmus is rare and vertigo, a symptom of vestibulocerebellar dysfunction, has not been reported yet. A 56-year-old woman visited our hospital due to sudden vertigo. [jkna.org]

  Several reports document nystagmus, including gaze-evoked (6, 10, 14) dissociated abducting (6, 10, 13), convergence-retraction (10), rebound (13) and upbeat lid nystagmus (15). [neuroophthalmology.ca]

  Physical examination showed restricted eye movement without nystagmus, bilateral flattening of forehead and nasolabial folds, and positive eyelash sign. Her tongue deviated to the left when protruded. [ncbi.nlm.nih.gov]

  However, the above definition has now been expanded to include the combination of upgaze palsy, convergence retraction nystagmus and pupillary light-near dissociation. This classic triad is seen in about 65% of patients [2]. [ejnpn.springeropen.com]

  On examination, there was ophthalmoplegia, nystagmus, areflexia and lower limb and gait ataxia. Serological testing was positive for GQ1b antibody in keeping with the diagnosis of Miller Fisher Syndrome (MFS). [eprints.whiterose.ac.uk]

The patient exhibited the classic triad of ataxia, areflexia, and opthalmoplegia characteristic of MFS, but also had less typical signs and symptoms making for a more challenging diagnostic workup. [ncbi.nlm.nih.gov]

Past medical and family history were negative for thromboembolic disease, and subsequent hypercoagulability workup was unremarkable. During the course of hospitalization, the patient also developed angioedema. [amjcaserep.com]

Guillain-Barré Syndrome Workup. Medscape. Reviewed May 4, 2018. Available at: https://emedicine.medscape.com/article/315632-workup Accessed Jan 9, 2020. Guillain-Barré Syndrome. National Organization for Rare Disorders. Reviewed 2017. [rarediseases.org]

• Helicobacter Pylori

  An antibody to VacA of Helicobacter pylori in the CSF of patients with Miller-Fisher syndrome. Neurology 2004;63:2184–2186. ↑ Yuki N, Hirata K. Fisher’s syndrome and group A streptococcal infection. [eyewiki.org]

  Cytomegalovirus, Mycoplasma pneumoniae, Epstein-Barr virus, Group A streptococcus, Coxiella burnetii, Pasturella multocida, Helicobacter pylori, Aspergillus, and varicella zoster have been more rarely identified preceding the onset of MFS [5]. [eyerounds.org]

  The following infective agents that have been associated with MFS include mycoplasma pneumonia, HIV, Campylobacter jejuni, Hemophilus influenza, Helicobacter pylori and Epstein-Barr virus. 4,16 In one report that looked at 466 MFS patients, 90% had an [reviewofoptometry.com]

People with more severe MFS who develop swallowing difficulties or progression to GBS may benefit from specific treatment, although no randomized controlled trials have been performed to demonstrate treatment efficacy. [disabled-world.com]

Table 1 Literature cases of MFS in association with anti-TNFα agents Reference Patient Anti TNF agent Underlying condition Duration of treatment Symptoms at onset GQ1b titre Treatment Outcomes Kurmann et al. [ 9 ] 77 years/female Adalimumab Rheumatoid [nnjournal.net]

Table 1 Literature cases of MFS in association with anti-TNFα agents Reference Patient Anti TNF agent Underlying condition Duration of treatment Symptoms at onset GQ1b titre Treatment Outcomes Kurmann et al.[9] 77 years/female Adalimumab Rheumatoid arthritis [oaepublish.com]

We here report a case of MFS/Bickerstaff brain stem encephalitis (BBE)-overlap syndrome and nonhypertensive PRES that occurred in close temporal association with IVIG treatment and caused stroke. Immunoadsorption ameliorated the disease course. [ncbi.nlm.nih.gov]

The prognosis of Miller Fisher syndrome was good--recovery occurred after a mean time period of 10.1 weeks. Residual symptoms were present in 74 cases (33.2%), and a recidivism of the Miller Fisher syndrome was reported in seven patients. [collections.lib.utah.edu]

Clinical spectrum, natural history, prognosis, and pathogenesis of MFS are not fully documented. OBJECTIVE: Probe the clinical features of MFS in a tertiary center in Thailand and compare its pattern with other Asian countries. [ncbi.nlm.nih.gov]

Relapses may occur rarely (in less than 3 percent of cases). x Prognosis The prognosis for most individuals with Miller Fisher syndrome is good. [ninds.nih.gov]

Campylobacter jejuni and Haemophilus influenzae are frequently reported etiological agents. We describe a boy with Miller Fisher syndrome following Epstein-002DBarr virus primary infectious mononucleosis. [ncbi.nlm.nih.gov]

While ophthalmoparesis could be compatible with MG, other clinical features suggested a different etiology. [frontiersin.org]

Etiology In the majority of cases, MFS occurs following Campylobacter jejuni infection. Although the exact pathological mechanism is not fully understood, MFS is associated with the presence of antiganglioside antibodies (primarily, anti-GQ1b). [orpha.net]

Probably a viral etiology. Precipitating factors of the Miller Fisher syndrome are an upper respiratory tract infection in the majority of cases but also include infection, surgery, vaccination, or insect bite. [accessanesthesiology.mhmedical.com]

Etiology MFS is an autoimmune-mediated peripheral neuropathy associated with anti-GQ1b neuronal antibodies (in up to 80% of cases).[1][2] As with Guillain-Barré syndrome, MFS can develop after infection by various pathogens. [eyewiki.org]

The epidemiology of Guillain-Barré syndrome worldwide. A systematic literature review. Neuroepidemiology. 2009;32(2):150-163. 7. Hughes RA, Rees JH. Clinical and epidemiologic features of Guillain-Barré syndrome. [he01.tci-thaijo.org]

Summary Epidemiology Annual incidence is estimated at 1/1,000,000. Clinical description MFS is characterized by the clinical triad of acute onset of gait ataxia, areflexia, and ophthalmoplegia. [orpha.net]

Clinical and epidemiological features of Guillain–Barre syndrome. J Infect Dis 1997; 176 (Suppl. 2): S92–S98. 2. Fisher CM. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia, areflexia). [nature.com]

MFS Epidemiology and Differential Diagnosis Miller Fisher syndrome is a rare disease. The incidence is not precisely known and might vary between geographical areas. [disabled-world.com]

Disponible en:https://repositorio.uta.edu.ec/bitstream/123456789/29789/1/CASO%20CLINICO%20GUILLAIN%20BARRE%20VARIANTE%20ATIPICA.pdf Ansari B, Basari K, Derakhshan Y, Kadkhodaei F, Okhovat A, Epidemiology and Clinical Features of Guillain-Barre Syndrome [revistas.juanncorpas.edu.co]

Fisher syndrome (MFS) is the result of a predominantly axonal or demyelinating polyneuropathy and whether the Guillain-Barré syndrome variant of acute ataxia and areflexia without ophthalmoplegia, ataxic Guillain-Barré syndrome (atxGBS), has a distinct pathophysiology [einstein.pure.elsevier.com]

Immunoglobulins inhibit pathophysiological effects of anti-GQ1b-positive sera at motor nerve terminals through inhibition of antibody binding. [pedneur.com]

Immunohistochemical studies suggest a pathophysiological role for anti-GQ1b antibodies at the paranodal regions of the oculomotor nerves, at some neurons of the dorsal root ganglia (DRG) and at motor nerve terminals. [ncbi.nlm.nih.gov]

Although it may present as benign as an acute change in voice, early diagnosis and prompt management may prevent further complications. [ncbi.nlm.nih.gov]

Prevention How can I prevent Miler Fisher syndrome? There’s no way to prevent Miller Fisher syndrome. But you can take steps to reduce your risk of getting a viral or bacterial infection. Be sure to: Avoid contact with people who are sick. [my.clevelandclinic.org]

Guillain-Barré syndrome after vaccination in United States: data from the Centers for Disease Control and Prevention/Food and Drug Administration Vaccine Adverse Event Reporting System (1990-2005). [jamanetwork.com]

Willison, Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model, Brain, 131, 5, (1197), (2008). [dx.doi.org]

How can Guillain-Barré syndrome be prevented? There are no known preventative measures for GBS. What is the evidence? Hughes, RA, Cornblath, DR. “Guillan-Barré syndrome”. Lancet. vol. 366. 2005. pp. 1653-66. [cancertherapyadvisor.com]