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Miller-Fisher Syndrome
Cranial Variant of GBS

Presentation

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

Skin

  • Anhidrosis

    Patients have severe postural hypotension, bowel and bladder retention, anhidrosis, decreased salivation and lacrimation, and pupillary abnormalities. Cardiovascular involvement is common, and dysrhythmias are a significant source of mortality. [emedicine.medscape.com]

Neurologic

  • 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]

    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]

    Clinical examination showed normal eye movements with no residual limb ataxia. He still had some problems with mild gait ataxia as evident by mild difficulty in tandem walking. [cerebellumandataxias.biomedcentral.com]

  • 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]

  • Paresthesia

    We report a 63-year old patient who presented to our care initially with a hypernasal voice followed by ataxia, ptosis, dysphonia, and paresthesias. [ncbi.nlm.nih.gov]

    Despite the absent ACD, areflexia, and no third, fourth, and sixth cranial nerve deficits, there was high suspicion for GBS due to acutely rapid ascending paresthesia, mild dysarthria, and mild ataxia. [cureus.com]

  • Hyporeflexia

    He presented with bilateral dysfunction of several cranial nerves and hyporeflexia of the limbs but without ataxia. Miller Fisher syndrome was confirmed by the presence of anti-GQ1b antibodies in a blood sample. [ncbi.nlm.nih.gov]

    Initial neurological examination demonstrated dysarthria, bilateral positive cerebellar signs, weak proximal muscle groups, hyporeflexia, and hypertonia, with reduced sensation to light touch, vibration, and joint position. [nature.com]

    Further, progressive descending weakness of the platysmal muscles and tongue, upper extremities paresis (Grade 3/5) with hyporeflexia, and dysmetria in both hands and feet are added. [scielo.org.mx]

    Bilateral hyporeflexia was detected throughout the patient's body. Some terminal dysmetria was observed during finger-to-nose testing. [jaoa.org]

  • 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]

    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]

    Negative Steroids/ azathioprine Gradual recovery over 1 year Shin et al. [ 8 ] 56 years/male Infliximab Rheumatoid arthritis 10 infusions Ataxia, nystagmus < 1:100 Steroids/ IVIG Independent ambulation at 6 months Ratnarajan et al. [ 10 ] 43 years/female [nnjournal.net]

Workup

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]

Bulbar involvement is uncommon but can lead to extensive workup to rule out stroke, myasthenia gravis (MG), and other neuromuscular disorders. [cureus.com]

Treatment

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]

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]

Read More Read Less Treatment Treatment Treatment for Miller Fisher syndrome is identical to treatment for Guillain-Barré syndrome: intravenous immunoglobulin (IVIg) or plasmapheresis (a procedure in which antibodies are removed from the blood) and supportive [ninds.nih.gov]

Treatment does not cure the condition but instead may shorten the time to recovery. Supportive treatment can also prevent severe complications of GBS. [medicalnewstoday.com]

Prognosis

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]

Etiology

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]

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]

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

Its etiology is post-infectious and immune mediated, causing it to increase research in areas such as neurology, psychiatry, immunology, infectology, and genetics2. [scielo.org.mx]

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]

Epidemiology

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]

Pathophysiology

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]

Prevention

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]

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]

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]

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]

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

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