From the Guidelines
Miller Fisher syndrome is a rare variant of Guillain-Barré syndrome (GBS) characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, with a relatively mild disease course and favorable prognosis, especially with prompt treatment and supportive care. The condition typically begins with abnormal eye movements and difficulty walking, often following a viral or bacterial infection by 1-4 weeks 1. Unlike classic GBS, which primarily affects the limbs, Miller Fisher syndrome predominantly impacts the cranial nerves, particularly those controlling eye movement. Most patients have anti-GQ1b antibodies in their serum, which target components of peripheral nerve cells 1. The condition is caused by an autoimmune response where the body's immune system mistakenly attacks the peripheral nervous system.
Some key features of Miller Fisher syndrome include:
- Ophthalmoplegia (eye muscle weakness)
- Ataxia (poor coordination)
- Areflexia (absent reflexes)
- Incomplete forms with isolated ataxia or ophthalmoplegia can occur
- Overlaps with classical sensorimotor GBS in an estimated 15% of patients 1
Treatment approaches for Miller Fisher syndrome mirror those for typical GBS, including intravenous immunoglobulin (IVIG) at 2g/kg divided over 5 days or plasma exchange, although the efficacy of these therapies might differ in certain populations, such as those in low-income and middle-income countries (LMIC) 1. Intravenous immunoglobulin is considered the first choice of treatment as it is easy to administer, widely available, and associated with a reduced frequency of adverse effects compared with plasma exchange. Supportive care is essential, with close monitoring for respiratory compromise. Most patients with Miller Fisher syndrome have a favorable prognosis, with symptoms typically resolving within 2-3 months, though recovery time varies 1. Early recognition and prompt treatment are important for optimal outcomes.
From the Research
Miller Fisher Variant of GBS
The Miller Fisher variant of Guillain-Barré Syndrome (GBS) is a rare condition characterized by a triad of symptoms, including:
- Ophthalmoplegia (weakness or paralysis of the eye muscles)
- Areflexia (absence of reflexes)
- Ataxia (loss of coordination and balance) 2, 3, 4, 5, 6
Clinical Presentation
The clinical presentation of Miller Fisher variant of GBS can vary, but common symptoms include:
- Rapidly ascending paresthesias (numbness or tingling sensations) in the limbs
- Urinary incontinence
- Slurred speech
- Dysphagia (difficulty swallowing)
- Gait disturbance
- Diplopia (double vision) 2, 3
Diagnostic Testing
Diagnostic testing for Miller Fisher variant of GBS may include:
- Cerebrospinal fluid (CSF) analysis to check for albuminocytological dissociation (ACD)
- Nerve conduction studies
- Imaging studies such as MRI or ultrasound
- Serologic testing for anti-ganglioside antibodies, particularly anti-GQ1b IgG antibodies 5, 6
Treatment
Treatment for Miller Fisher variant of GBS typically involves immunotherapy with:
- Intravenous immunoglobulin (IVIG)
- Plasma exchange
- Complement inhibition combined with IVIG (although further studies are needed to prove efficacy) 5, 6
Outcome
The outcome of Miller Fisher variant of GBS is usually good, with a case fatality rate of less than 5% 5. Most patients can expect to make a full recovery with treatment, although some may experience residual symptoms.