Miller Fisher syndrome: symptoms, diagnosis, treatment and recovery

Guillain-Barre syndrome (GBS) is a rare condition in which the immune system, which normally fights infection, mistakenly attacks the nerves. The NHS describes it as thought to be caused by problems with the immune system and says it usually starts within a few weeks of an infection. NINDS says Miller Fisher syndrome is another type of GBS involving the cranial nerves, which run from the brain to areas of the head and neck.

A case-based literature review explains that MFS was first recognised by James Collier in 1932 as a separate clinical triad of ophthalmoplegia, ataxia and areflexia, and was later described by Charles Miller Fisher in 1956 as a limited variant of GBS. It accounts for one to five per cent of GBS cases in Western countries, according to that review, and is more common in men than women, with a median age of onset in the fifth decade.

The NHS page on GBS does not discuss MFS by name, so details specific to MFS here draw on NINDS and a published clinical review, both US sources.

According to the case-based review, MFS usually begins over days with the rapid development of three problems: ophthalmoplegia, weakness of the muscles within or around the eye; ataxia, uncoordinated movements and poor balance with clumsy walking; and areflexia, loss of deep tendon reflexes. The commonest presentations are double vision, unsteady walking or both.

NINDS notes that people with MFS may also have other common GBS symptoms such as muscle weakness. The NHS lists tingling, numbness or pins and needles in the feet and hands, followed by muscle weakness, and problems such as drooping face muscles, trouble swallowing or speaking, and double vision as symptoms of GBS, and says that symptoms usually get worse over the first 2 to 4 weeks. Many people with MFS go on to develop more widespread weakness of GBS, according to the review, so it is monitored closely.

• Double vision or difficulty moving the eyes.

• Unsteadiness, clumsiness and difficulty walking.

• Loss of reflexes, found on examination.

• Tingling or pins and needles in the hands and feet.

• Drooping eyelids or weakness of the face in some people.

• Spreading weakness or breathing difficulty, which is an emergency.

The NHS says to ask for an urgent GP appointment or get help from NHS 111 if you think you have Guillain-Barre syndrome, and to call 999 or go to A&E if you have problems with your face muscles, such as one side drooping or trouble swallowing, you are not able to lift one or both arms, or you have trouble speaking such as slurring your words, which could be signs of a stroke.

Sudden double vision with unsteadiness can have several causes, including stroke, so it should be assessed promptly rather than watched at home. Difficulty breathing or swallowing is an emergency.

The exact cause is unknown. NINDS describes GBS as an autoimmune disease in which the immune system mistakenly attacks healthy nerves, often after a respiratory or gastrointestinal infection. The review says the most common pathogens associated with MFS are Campylobacter jejuni and Haemophilus influenzae, with Mycoplasma pneumoniae and cytomegalovirus also associated, and onset typically 8 to 10 days after the preceding illness.

It is not contagious or inherited, according to NINDS, which describes GBS this way. Most people have no way of having prevented it, so there is no cause for self-blame.

The review says MFS is primarily a clinical diagnosis, based on the combination of ataxia, areflexia and ophthalmoplegia, together with normal findings on CT or MRI and, typically, raised protein with a normal cell count in the cerebrospinal fluid. Anti-GQ1b antibodies, which are directed against a component of nerves, relate to disease activity and can help confirm the diagnosis, although they are not unique to MFS.

The NHS says that for GBS the GP will check reflexes and look for numbness or weakness, then refer to a neurologist for tests such as an electrical test of the nerves and muscles, a breathing test, blood tests and a lumbar puncture. Imaging may be used to exclude other causes such as stroke, and the review lists conditions that can mimic MFS, including myasthenia gravis and thyroid eye disease.

The NHS says that if you have been diagnosed with GBS you will be treated in hospital straight away, usually for several weeks, with immunotherapy to stop your immune system attacking the nerves, either IVIG or plasma exchange, alongside treatment to manage symptoms and monitor your condition. This may include medicines and compression stockings to reduce the risk of blood clots in people who cannot walk, medicines for nerve pain, and, if breathing is severely affected, a ventilator in intensive care.

The review notes that although MFS follows a self-limiting course, immunomodulatory therapies including IVIG and plasmapheresis have been used to hasten recovery, and in the case it reports the patient improved with IVIG. NINDS adds that IVIG and plasma exchange are equally effective if started within two weeks of symptoms, and that corticosteroids have been tried but trials show they are not effective. Because MFS may progress to wider weakness, people are monitored closely, particularly breathing and swallowing.

The review says ataxia and ophthalmoplegia typically resolve within one to three months after onset and that near-complete recovery is expected within six months. It notes that areflexia may persist but is not associated with functional disability, and that serious complications such as cardiac arrhythmia or respiratory failure are rare.

For GBS more broadly, the NHS says most people will be able to walk within 6 months and recover within a year, although some have longer-term problems such as tingling, nerve pain, fatigue, muscle weakness, anxiety and depression. It says the doctor will check you every few months after recovery and then once a year, because some people can become unwell again, and that you should contact your specialist straight away if symptoms return. Physiotherapy and occupational therapy may be offered.

MFS and GBS are assessed and treated through NHS emergency and neurology services because they need hospital admission, specialist tests and sometimes intensive care. Rehabilitation is usually arranged by the hospital team and community therapists.

The NHS suggests free NHS talking therapies for the emotional impact of GBS and points to Inflammatory Neuropathies UK for support. Private neurology opinions are available for a second view, but acute treatment is delivered in hospital.