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Guillian

Miller Fisher syndrome

The GBS variant that starts with double vision and unsteadiness rather than leg weakness. What it is, how it is identified, and why it is often missed first time.

By Marrallisa, patient, not a clinician

Most descriptions of Guillain-Barré start with weakness in the legs that climbs upwards. Miller Fisher syndrome does not follow that script, which is exactly why it is so often missed at the first appointment.

The three things that define it

Miller Fisher syndrome is a variant of Guillain-Barré, first described in 1956. It is recognised by a triad:

Ophthalmoplegia. The muscles moving your eyes stop working properly. In practice: double vision, drooping eyelids, difficulty tracking something across the room. This is usually the first thing people notice.

Ataxia. Unsteadiness. Your limbs do not go quite where you send them and your walking becomes wide-based and uncertain. It can feel like being drunk while completely sober.

Areflexia. Your tendon reflexes disappear. You will not notice this yourself, but it is one of the first things a neurologist checks.

Not everyone has all three, and the picture can overlap with ordinary GBS, including limb weakness or swallowing problems.

How it is identified

Anti-GQ1b antibodies. This is the specific one. These antibodies are found in the large majority of people with Miller Fisher syndrome, and they are strongly associated with the eye movement problems. A positive result is a strong pointer.

Lumbar puncture. As in GBS, raised protein with a normal cell count. And as in GBS, often still normal in the first week, which does not rule anything out.

Nerve conduction studies. Can be subtle in Miller Fisher, particularly early. Findings often centre on the sensory nerves.

Like GBS, the diagnosis is primarily clinical. The tests support the picture and rule out other causes, they do not by themselves prove it.

Triggers

The same territory as GBS: an infection one to four weeks earlier. Campylobacter jejuni and Haemophilus influenzae are recognised triggers.

What to expect

Miller Fisher syndrome generally has a better outlook than ordinary GBS. Most people recover well, and the eye movement problems typically settle over weeks to months.

That said, two honest caveats:

  • A minority progress to include limb weakness or breathing problems. That means the same monitoring rules apply: rapidly increasing weakness, trouble breathing or swallowing is an emergency.
  • “Better outlook” is a statement about groups. Fatigue and unsteadiness can outlast the headline recovery by a long way, and that experience is real even when the examination looks normal.

Treatment

The same tools as GBS: IVIg or plasma exchange. Because the outlook is generally good, milder cases are sometimes monitored rather than treated immediately. That is a legitimate approach, and it is also a decision you are entitled to understand rather than simply receive.

Ask: what would have to change for you to start treatment? Getting that threshold named, and written down, is worth more than any reassurance.

Full detail on treatment.

If it comes back

The same rule applies as for GBS. Repeated deterioration, or deterioration beyond eight weeks from your first symptoms, is a reason to ask whether this is still a one-off event or something chronic that needs maintenance treatment.

See relapse and CIDP.

Sources

  1. Fisher M. An unusual variant of acute idiopathic polyneuritis. New England Journal of Medicine, 1956;255:57-65
  2. Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome. Neurology, 1993;43:1911-1917
  3. Wakerley BR, Uncini A, Yuki N. Guillain-Barré and Miller Fisher syndromes, new diagnostic classification. Nature Reviews Neurology, 2014;10:537-544
  4. Leonhard SE et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nature Reviews Neurology, 2019;15:671-683

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