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Miller-Fisher Syndrome


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]
  • Very rarely, MFS can present with a recurrent course.[ncbi.nlm.nih.gov]
  • His presentation is discussed in the context of the animal and human literature on neuromuscular junction abnormalities in MFS.[ncbi.nlm.nih.gov]
  • Her presentation and natural history of disease were similar to the 2 previously published cases. We present the third case of postvaccination Miller Fisher syndrome in the literature and the first associated with the novel influenza A(H1N1) vaccine.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Sneezing
  • In the month preceding admission, he had had low grade, intermittent fevers, followed by a nonproductive cough and sneezing. During hospital admission he lost deep tendon reflexes and was unable to walk.[ncbi.nlm.nih.gov]
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]
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]
Arthralgia
  • 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]
Positive Romberg Sign
  • She had positive Romberg sign and failed the right heel-knee-tibia tests. Her brain diffusion-weighted magnetic resonance imaging (DWI) showed an abnormally high circular signal in the brainstem surrounding the fourth ventricle.[ncbi.nlm.nih.gov]
Excitement
  • The compound muscle action potential (CMAP) scan is a recently developed non-invasive, painless, and reproducible method for detecting early changes in motor nerve excitability.[ncbi.nlm.nih.gov]

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.[ncbi.nlm.nih.gov]
Hyperviscosity
  • Cerebral infarction following intravenous immunoglobulin is thought to be secondary to hyperviscosity, thromboemboli, vasculitis, or cerebral vasospasm and reported to occur after a short latency when the immunoglobulin load is highest.[ncbi.nlm.nih.gov]

Treatment

  • 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 treatment of Takotsubo cardiomyopathy is supportive with beta-blockers and angiotensin-converting enzyme inhibitors are recommended until left ventricle ejection fraction improvement.[ncbi.nlm.nih.gov]
  • 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.[ninds.nih.gov]
  • We report a patient with diffuse large B-cell lymphoma (DLBCL) who initially presented as Miller Fisher syndrome (MFS) responsive to high-dose immunoglobulin treatment.[ncbi.nlm.nih.gov]

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]
  • Serial electrophysiological studies allowed not only to understand the underlying pathophysiology and formulate a more correct prognosis but also to guide the treatment.[ncbi.nlm.nih.gov]
  • Emphasis is placed on the mode of presentation and management issues, as early diagnosis is crucial and confers a favourable prognosis. In that respect, we consider this case noteworthy and instructive.[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 MFS is an autoimmune-mediated peripheral neuropathy associated with anti-GQ1b neuronal antibodies (up to 80% of cases).1 ,2 As with Guillain-Barré syndrome, MFS can develop after infection by various pathogens.[eyewiki.org]
  • Although the etiology behind this disorder is not yet clear, it has been associated with certain viral and bacterial infections. Certain microbes use molecular mimicry to escape the immune system of the body.[buzzle.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]
  • The results of biochemistry tests, including electrolytes, liver and kidney function tests, hemoglobin A1c, coagulation function, thyroid function, cobalamine, folic acid, tumor marker screen test, test for infectious etiologies, and C-reactive protein[alliedacademies.org]

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]
  • 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]
  • Epidemiology The incidence of typical GBS in Europe is 1.2-1.9 per 100,000 [ 3 ] . There is an increased incidence in males. Peak ages are 15-35 years and 50-75 years.[patient.info]
  • The Epidemiology of Guillain-Barré Syndrome Worldwide. A Systematic Literature Review. Neuroepidemiology . 2008 Dec 17. 32(2):150-163. [Medline] . McKhann GM, Cornblath DR, Griffin JW.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • 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]
  • These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers.[ncbi.nlm.nih.gov]
  • The electrophysiological findings in PCB/MFS are of great interest and may provide insight into the pathophysiology of the disorder.[ncbi.nlm.nih.gov]
  • 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]

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]
  • 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]
  • 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]
  • 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]

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