Presentation
Miller Fisher syndrome classically presents with ophthalmoplegia, ataxia and areflexia. The syndrome may present rarely with atypical clinical features. Whether the central or peripheral nervous system is primarily involved remains controversial. [ncbi.nlm.nih.gov]
Musculoskeletal
- Acroparesthesia
The authors describe a 38-year-old man who presented with hypernasality, perioral and acroparesthesia, dyspnea, and dysphagia. Further evaluation revealed a diagnosis of Miller-Fisher syndrome (MFS). [ncbi.nlm.nih.gov]
Ears
- Otalgia
In a 48-year-old male tiredness, tinnitus, otalgia, parietal hyperaesthesia, coughing, plugged nose, hypoacusis, globus sensation, epipharyngeal pain, dysarthria, hypogeusia, arthralgia, lid cloni, facial hypaesthesia and tooth ache consecutively developed [ncbi.nlm.nih.gov]
- Tinnitus
In a 48-year-old male tiredness, tinnitus, otalgia, parietal hyperaesthesia, coughing, plugged nose, hypoacusis, globus sensation, epipharyngeal pain, dysarthria, hypogeusia, arthralgia, lid cloni, facial hypaesthesia and tooth ache consecutively developed [ncbi.nlm.nih.gov]
Neurologic
- Ataxia
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]
- 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]
- 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]
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]
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]
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 Infliximab [nnjournal.net]
- Cerebellar Ataxia
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]
The first report of anti-ganglioside auto-antibodies was against cerebellar neurons (Gregson et al., 1977), so it is not surprising that patients present with cerebellar ataxia. [docneuro.com]
Rarely is the cerebellar ataxia or areflexia severe enough to cause major problems; the major impediment to these patients is their vision. [accessanesthesiology.mhmedical.com]
GD1b IgG antibody may be associated with acute motor and sensory axonal neuropathy with cerebellar ataxia or ataxic presentations of Guillain-Barré Syndrome. GQ1b IgG antibody is associated with the Miller-Fisher Syndrome. [questdiagnostics.com]
Causes of progressive cerebellar ataxia: prospective evaluation of 1500 patients. J Neurol Neurosurg Psychiatry. 2017;88(4):301-309. Tanaka Y, Satomi K. Overlap of Myasthenia Gravis and Miller Fisher Syndrome Intern Med. 2016;55(14):1917-1918. [revistas.juanncorpas.edu.co]
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]
However, other neurological signs and symptoms may also be present leading to a more challenging diagnostic workup. Bilateral ophthalmoplegia is the first presenting feature of MFS. [cureus.com]
The workup suggested a diagnosis of acute polyradiculoneuropathy; thus, the patient was treated with five sessions of plasmapheresis, leading to stabilization of the clinical features and subsequent slow mild clinical improvement, especially of limb ataxia [frontiersin.org]
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 care. × Treatment Treatment [ninds.nih.gov]
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]
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]
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
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]
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]
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]
While ophthalmoparesis could be compatible with MG, other clinical features suggested a different etiology. [frontiersin.org]
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]
The etiology of this condition was speculated to be similar to that of Guillain-Barré syndrome because of evidence of prodromal upper respiratory infection, areflexia, and CSF albuminocytological dissociation ( Bickerstaff and Cloake 1951 ). [medlink.com]
Epidemiology
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]
Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology: Guillain-Barré syndrome variants in Emilia-Romagna, Italy, 1992–3: incidence, clinical features, and prognosis. J Neurol Neurosurg Psychiatry 1998;65:218–224. [karger.com]
[…] study reported a decreased GBS incidence following administration of tetanus toxoid containing vaccinations when compared with the baseline population [54] An epidemiologic study failed to show any conclusive epidemiologic association between GBS and [emedicine.medscape.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]
An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody. [ncbi.nlm.nih.gov]
Prevention
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]
Plasmapheresis and/or administration of intravenous immunoglobulin (IVIg) are the main treatment options, and they aim to reduce the immune reaction and prevent further damage. » Plasmapheresis is a technique where blood is collected; the plasma is isolated [buzzle.com]
Deep vein thrombosis (DVT) prophylaxis : DVT due to immobility should be prevented with gradient compression stockings and subcutaneous low molecular weight heparin. See separate Prevention of Venous Thromboembolism article. [patient.info]
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]