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) a renowned Canadian neurologist, who first described the condition in 1956 2,3,9.
Clinically the differential includes primarily the other anti-GQ1b IgG antibody-associated conditions.
- 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. Tapia J. [Charles Miller Fisher: a giant of neurology]. (2013) Revista medica de Chile. 141 (8): 1076-80. doi:10.4067/S0034-98872013000800017 - Pubmed
- 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
- 9. Louis R. Caplan, Jay P. Mohr, Robert H. Ackerman. In Memoriam: Charles Miller Fisher, MD (1913-2012). (2012) Archives of Neurology. 69 (9): 1208. doi:10.1001/archneurol.2012.1743 - Pubmed
Related Radiopaedia articles
White matter disorders
- white matter
- normal myelination
white matter disorders
- anti-MOG associated encephalomyelitis
- Guillain-Barre Syndrome (GBS)
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- transverse myelitis
- tumefactive demyelinating lesions
- acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic encephalomyelitis (AHEM)
- neuromyelitis optica (NMO) (Devic disease)
multiple sclerosis (MS)
McDonald diagnostic criteria for MS (current 2017 revision)
- previous 2016 MAGNIMS consensus
- McDonald diagnostic criteria for MS (current 2017 revision)
- radiologically isolated syndrome (RIS)
- clinically isolated syndrome (CIS)
- early-onset neuronal degenerative disorders
- GM1 & GM2 gangliosidoses (e.g. Tay Sachs disease)
- giant axonal neuropathy
- hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC)
- hypomyelination with congenital cataract
- leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation
- hypomyelination with brainstem and spinal cord involvement and leg spasticity
- Pol III-related leukodystrophies
- early-onset neuronal degenerative disorders
- myelin disorders
- myelin vacuolisation
- adult-onset autosomal dominant leukodystrophy
- cerebrotendinous xanthomathosis
- cystic leukoencephalopathy without megalencephaly
- L-2-hydroxyglutaric aciduria
- lysosomal storage diseases
- peroxisomal disorders
- Sjögren-Larsson syndrome