Miller Fisher syndrome
Miller Fisher syndrome is a regional variant of Guillain–Barré syndrome and characterized by cranial nerve involvement and the triad of ataxia, areflexia, and ophthalmoplegia. They are now believed to represent, along with a number of other entities, different clinical manifestations of a similar underlying autoimmune disorder, anti-GQ1b IgG antibody syndrome.
Patients who develop Miller Fisher syndrome usually have a rapid onset of ataxia, areflexia and ophthalmoplegia, which usually follows a viral illness in the preceding 5-10 days 1,5. Ophthalmoplegia (initially upgaze, then lateral gaze and then downgaze) and ataxia usually precede areflexia 5.
Areflexia of the limbs only occasionally is associated with weakness 5.
Electrophysiological findings most commonly include reduced sensory nerve action potentials and absent H reflexes 7.
Miller Fisher syndrome is considered to be a peripheral neuropathy, however involvement of the central nervous system has also been reported (somewhat controversially), including lesions within the posterior columns of the spinal cord and in the brainstem 1,4.
Anti-GQ1b antibodies are present in most (~80-90%) of cases 6-7.
Although MRI is usually used to investigate patients with these symptoms, there can be no abnormalities 7.
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.
Generally, recovery begins a few weeks after symptom onset and is usually complete by 6 months 5.
Treatment with immunomodulatory therapies is controversial 6.
History and etymology
It is named after Charles Miller Fisher (1913-2012) Canadian neurologist, first described the condition in 1956 2-3.
Clinically the differential includes primarily the other anti-GQ1b IgG antibody associated conditions.
- primary demyelinating disorders
- clinically isolated syndrome (CIS)
- radiologically isolated syndrome (RIS)
- multiple sclerosis (MS)
- neuromyelitis optica (NMO) (Devic disease)
- acute disseminated encephalomyelitis (ADEM) and acute haemorrhagic encephalomyelitis (AHEM)
- tumefactive demyelinating lesions
- transverse myelitis
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- Guillain-Barre Syndrome (GBS)
- primary demyelinating disorders
- 1. Inoue N, Ichimura H, Goto S et-al. MR imaging findings of spinal posterior column involvement in a case of Miller Fisher syndrome. AJNR Am J Neuroradiol. 2004;25 (4): 645-8. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Charles Miller Fisher biography - Whonamedit.com
- 3. Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N. Engl. J. Med. 1956;255 (2): 57-65. doi:10.1056/NEJM195607122550201 - Pubmed citation
- 4. Giroud M, Mousson C, Chalopin JM et-al. Miller-Fisher syndrome and pontine abnormalities on MRI: a case report. J. Neurol. 1991;237 (8): 489-90. Pubmed citation
- 5. Fenichel GM. Clinical Pediatric Neurology. Saunders. (2009) ISBN:1416061851. Read it at Google Books - Find it at Amazon
- 6. Roos KL. Emergency Neurology. Springer. (2012) ISBN:0387885854. Read it at Google Books - Find it at Amazon
- 7. Arányi Z, Kovács T, Sipos I et-al. Miller Fisher syndrome: brief overview and update with a focus on electrophysiological findings. Eur. J. Neurol. 2012;19 (1): 15-20, e1-3. doi:10.1111/j.1468-1331.2011.03445.x - Pubmed citation
- 8. Saremi F, Helmy M, Farzin S et-al. MRI of cranial nerve enhancement. AJR Am J Roentgenol. 2005;185 (6): 1487-97. doi:10.2214/AJR.04.1518 - Pubmed citation