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Miller-Fisher syndrome

Orpha number ORPHA98919
Synonym(s) Cranial variant of GBS
Cranial variant of Guillain-Barré syndrome
Fisher syndrome
Prevalence 1-9 / 1 000 000
Inheritance
  • Sporadic
  • Multigenic/multifactorial
Age of onset Variable
ICD-10
  • G61.0
OMIM -
UMLS
  • C0393799
MeSH
  • D019846
MedDRA
  • 10049567
SNOMED CT
  • 1767005

Summary

Miller-Fisher syndrome (MFS) is a rare cranial nerve variant of Guillain-Barré syndrome (GBS; see this term). Annual incidence is estimated at 1/1,000,000. MFS is characterized by the clinical triad of acute onset of gait ataxia, areflexia, and ophthalmoplegia. Acute onset of external ophthalmoplegia is a cardinal feature of MFS. Facial and bulbar nerve palsies have also been reported. 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). The clinical picture in MFS shows clinical overlap with Bickerstaff brainstem encephalitis (BBE; see this term), which is also associated with raised titers of anti-GQ1b antibodies, leading to the suggestion that BBE and MFS represent variable manifestations of the same clinical spectrum. However, patients with BBE also have central nervous system involvement leading to disturbed consciousness and in some cases tetraparalysis with involvement of cranial nerves (including the intraocular nerves leading to fixed pupils). MFS is generally self-limiting and patients recover within a few months, however, cases of progression to respiratory failure have been reported in MFS patients with features of both MFS and other forms of GBS (MFS/GBS overlap syndrome).

Expert reviewer(s)

  • Pr Pieter VAN DOORN

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