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Miller Fisher Syndrome

Franklyn Rocha Cabrero; Elizabeth H. Morrison.

Author Information

Last Update: October 27, 2018.

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Introduction
Polyneuropathies are a dysfunction in multiple nerves and divided into 2 broad categories:
axonal or demyelinating. Axonal neuropathies cause symptoms related to axon damage and loss
and are caused by a broad number of systemic illnesses. Demyelinating neuropathies produce
abnormalities because Schwann cells do not interact appropriately with axons. Schwann cells are
glial cells that play an important role in the peripheral nervous system including saltatory
conduction of nerve impulses along axons, nerve development and regeneration, modulation of
neuromuscular synaptic activity, and presentation of antigens to T lymphocytes. Demyelinating
neuropathies include toxic, hereditary, and immune-mediated etiologies; the latter can be further
be classified in acute and chronic depending on the onset. Acute, immune-mediated
demyelinating polyneuropathies (AIDP) are classified within the spectrum of Guillain-Barre
syndrome (GBS), named after the French physicians who discovered it. The focus of our review
will be the Miller Fisher syndrome (MFS), a rare variant of GBS. James Collier first discovered
the variant in 1932 and described it as a triad of symptoms including ophthalmoplegia,
ataxia, and areflexia. Miller Fisher later characterized it in 1956, classifying it as a unique entity
within the GBS spectrum.

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Etiology
MFS and GBS are thought to result from an aberrant acute autoimmune response to a preceding
infection (e.g., Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, or human
immunodeficiency virus (HIV). A cross-reaction between peripheral nerve antigens and
microbial/viral components through molecular mimicry is thought to drive the inflammatory
process of this illness. Approximately two-thirds of cases are preceded by symptoms of upper
respiratory tract infection or diarrhea, and about 50% develop following an infection.
Researchers do not fully understand the precise mechanism of pathogenesis. The immune
response can be directed toward the myelin or the axon of the peripheral nerve.

MFS is associated mainly with dysfunction of the third, fourth, and sixth cranial nerves.
Documented cases have been reported, however, for most of the other cranial nerves. Antibodies
against the GQ1b ganglioside are a typical serological finding as described in the
pathophysiology section of this article, but the absence of antibodies does not rule out the disease
completely. Other risk factors associated with the disease include the use of certain drugs (heroin,
suramin, streptokinase, and isotretinoin), use of TNF-alpha antagonist therapy, other concurrent
autoimmune diseases (systemic lupus, Hodgkin disease, and sarcoidosis), surgery, epidural
anesthesia, bone marrow transplant, and immunizations.

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Epidemiology
The worldwide incidence of GBS is approximately 1 to 2 in 100,000, with MFS variant
representing a tiny subset of the cases (1 to 2 in 1,000,000). It affects more men than women
with an approximate gender ratio of 2:1 and a mean age of 43.6 years at the onset of disease. It
has a higher incidence in Asians, estimated between 15% to 25% of GBS in this population
compared to 5% in the Western populations. A viral infection precedes the neurological
symptoms in 72% of cases, with an average latent asymptomatic and incubation period of 10
days. A recent study that looked at patients admitted with GBS, MFS, and Bickerstaff brainstem
encephalitis (BBE) in a tertiary hospital showed that recurrence of symptoms occurs at a higher
rate in patients with MFS, and BBE, than GBS.

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Pathophysiology
Molecular mimicry between peripheral nerve and microbial/viral antigens is thought to occur
through the activation of the adaptive immune system. Humoral and cell-mediated lymphocyte
mobilization is thought to play a major role. Gangliosides are important carbohydrate
determinants for autoimmune activity. Several studies have suggested that antibodies against
gangliosides, the IgG anti-GQ1b antibody, are a specific feature of MFS. The presence of
ophthalmoparesis in MFS is thought to result from a direct action of anti-GQ1b antibodies on the
neuromuscular junction between the cranial nerves and ocular muscles. Other disorders including
post-infectious acute ophthalmoplegia (AO), also named incomplete MFS lacking ataxia,
Bickerstaff brainstem encephalitis (BBE, also named atypical MFS with central nervous system
signs), and Guillain-Barre syndrome with ophthalmoplegia (GBS-OP) present with a positive
GQ1b antibody. About 70% to 90% of patients will have positive result through an enzyme-
linked immunosorbent assay (ELISA). A minority of patients (10% to 30%) of patients are still
negative (GQ1b-seronegative), possibly related to the requirement of calcium-dependent ligands
to be present for binding of the antibody, as shown in a study by Uchibori et al., 2016 in the
Journal of Neuroimmunology.

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Histopathology
The first neuropathological description of MFS was described in a case report by Phillips and
Anderson in 1984. In this case, the brain of the patient, who died unexpectedly from
bronchopneumonia, was prepared for histopathological analysis. Microscopic examination using
solochrome cyanine staining (for myelin) showed patchy and extensive segmental demyelination
associated with invasion of foamy macrophages and lymphocytes. These changes affected both
motor and sensory roots in the peripheral nervous system, as well as cranial nerves. The brain
stem and spinal cord, among other regions of the central nervous system, are relatively spared.
Electron microscopy confirmed the above findings and exquisitely showed complete
demyelination of axons in Schwann cell cytoplasm with surrounding macrophages and
lymphocytes.

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History and Physical


The clinical hallmark of MFS is a triad presentation of acute ophthalmoplegia, areflexia, and
ataxia in the setting of a preceding bacterial or viral illness. Distal paresthesia with or without
weakness is also present. Symptoms on an average peak in four weeks or less, with supporting
ancillary criteria as described by Brighton for GBS by Fokke C et al. in 2014. Other associated
symptoms include diplopia or blurred vision, dysarthria, dizziness and extremity tingling. Cranial
nerve involvement is typical, resulting in facial, oculomotor, or bulbar weakness, which may
extend to the limbs. Physical examination findings include typical findings for GBS like facial
paresis, distal hyporeflexia without signs of upper motor neuron dysfunction, and loss of light
and vibratory sensation in the distal extremities. Autonomic dysfunction such as hypertension,
hypotension or cardiac arrhythmia presents in advanced untreated GBS/MFS. Interestingly, the
corneal reflex can be impaired.

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Evaluation
If there is clinical suspicion for MFS and/or GBS, a lumbar puncture with appropriate
cerebrospinal fluid (CSF) studies are warranted to narrow the differential diagnosis further. A
hallmark of GBS and MFS, if present, is an albuminocytologic dissociation, or a combination of
normal cell count and raised protein level in the CSF found in approximately 90% of patients at
peak disease. There are, however, certain caveats: only half of the patients have
albuminocytologic dissociation on initial analysis, and a normal protein level, especially early in
the disease, does not exclude the diagnosis. Approximately 10% of patients with GBS have
normal CSF studies. Approximately 15% to 20% have a mild increase in cerebrospinal fluid cell
count (5 to 50 cells/microliter). Furthermore, nerve conduction studies can support the diagnosis
and provide prognostic information.

For MFS, electrodiagnostic studies may show reduced or absent sensory responses without
slowing of sensory conduction studies. CT/MRI scans of the spine may show thickening and
enhancement of the intrathecal spinal nerve roots and cauda equina, along with some spinal
nerve roots enhancement. The literature has described abnormalities of the spinal cord posterior
columns, and brain oculomotor, abducens, and facial nerves.

The Brighton criteria are a validated, quantitative tool that use clinical history, physical exam,
laboratory, and imaging findings to diagnose GBS and its variants, including MFS. The scoring
system is based on the following features:

 Bilateral and flaccid weakness of limbs (100% of patients)


 Decreased or absence of deep tendon reflex in distal extremities
 Monophasic course and time between onset and nadir is 12 hours to 28 days (97% of
patients)
 Decreased or absent deep tendon reflexes in weak limbs (91% of patients)
 Cerebrospinal fluid cell count fewer than 50/microliter (100% of patients)
 Cerebrospinal fluid protein concentration greater than reference range (49% of patients
initially, 88% at 3 weeks
 Nerve conduction study results consistent with 1 of the Guillain-Barré syndrome
subtypes (99% of patients)
 Lack of an alternative diagnosis for weakness

The Brighton criteria range from level 1 (highest level of diagnostic certainty) to level 4
(Guillain-Barre syndrome diagnosis of exclusion, alternative possible).

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Treatment / Management
MFS is mainly treated with adequate supportive care, pain control, respiratory support as needed,
and immunotherapy. Although used in the past, oral or intravenous (IV) steroids are no longer
recommended in the treatment of GBS or MFS because they are ineffective. Corticosteroids may
slow recovery from GBS they are recommended only in the setting of neuropathic or radicular
pain. IV immunoglobulin (IVIg) and plasma exchange are both effective treatments for GBS and
severe cases of MFS. No difference exists between the primary outcomes of mortality, disability,
and length of intubation between IVIG and plasmapheresis. Patients with MFS usually do not
require immunotherapy, presumably because they have a good prognosis and spontaneous
recovery. IVIG should be considered in patients with severe Miller Fisher syndrome who have
swallowing and respiratory difficulties, despite lack of supporting evidence of benefit. Overall,
IVIG is preferred over exchange due to convenience, availability, and minimal adverse effects,
however; the cost can be prohibitive for some low income or underinsured patients.

Before IV immunoglobulin therapy, clinicians should check serum IgA levels because patients
with IgA deficiency are at higher risk of anaphylaxis. The usual IVIg dose is 2 g/kg divided over
2 to 5 days. A second treatment course may be necessary for some patients. In children and
adolescents, a dose of 1 g/kg per dose IV daily for 2 days is given. Alternatively, 400 mg/kg per
dose IV daily for 5 days has been used. For patients with renal impairment, clinicians should use
approximately 50% of the usual dose. Plasma exchange is effective when given within 2 weeks
of illness onset in patients who are unable to walk, reaching highest effectiveness within seven
days of weakness onset. Plasma exchange sessions (2 to 3 L of plasma/body weight) over 2
weeks is the standard course for patients who are unable to walk without assistance. Mildly
affected patients with low disability score still benefit from 2 plasma exchanges of 1.5 plasma
volumes. Contraindications for plasma exchange include the recent prior use of IV
immunoglobulin infusion therapy, hemodynamic instability, pregnancy, sepsis, and
hypocalcemia.

Pain relief is an important consideration and a barrier to rehabilitation in the hospital. In a study
of pain intervention in patients with GBS, 75% of patients required oral or parenteral opioids for
pain management and 30% of patients required IV opiates. Therefore, an optimal pain regimen
early in the course of the disease is important to accelerate recovery. A combination of
medications is often needed as because of the mixed nature of the pain. Indicated medications
include gabapentin, pregabalin, carbamazepine, and amitriptyline. Corticosteroids can be used to
address neuropathic or radicular pain. Oral or intravenous opioids, for example, IV morphine 1
to 7 mg per hour, should be used with extreme care due to the suppressing effect on respiratory
drive, and autonomic system side effects like urinary retention.

Deep vein thrombosis (DVT) prophylactic therapy should be started promptly to reduce the risk
of pulmonary embolism. Administration of prophylactic doses of subcutaneous heparin or
enoxaparin is appropiate. Alternatively, mechanical compression stockings can be used in adult
patients unable to walk. If autonomic dysfunction is present, additional supportive treatment may
be necessary. If the patient is moderate to severely bradycardic and at risk of asystole, the patient
may require a cardiac pacemaker. If dysphagia is present, a nasogastric tube may be necessary
for feeding and nutrition. Bladder catheterization can relieve patients with urinary retention. A
bowel regimen will be indicated to help with constipation. Early physical therapy during
the illness and early rehabilitation is pivotal once patient clinically improves.

Inpatient and intensive care unit (ICU) disposition is an important consideration for a patient
with acute GBS and its variant MFS. This is based on symptoms severity, and, most importantly,
respiratory status. Mechanical ventilation is required for the 20% to 30% of patients who develop
respiratory failure; endotracheal intubation and even a tracheostomy may be necessary. Signs of
respiratory muscle fatigue include tachycardia, tachypnea, asynchronous chest/abdomen
movement, and evident use of accessory muscles. All patients with acute debilitating symptoms,
as mentioned, are admitted inpatient for supportive care. ICU admission and mechanical
ventilation are recommended in patients with at least 1 major criterion or 2 minor criteria. Major
criteria include hypercapnia PaCO2 greater than 48 mm Hg, hypoxemia PaO2 less than 56 mm
Hg at room air, vital capacity less than 15 mL/kg of body weight, and negative inspiratory force
less than -30 cm H2O. Minor criteria include an inefficient/weak cough, dysphagia, and
atelectasis as evidenced in a chest x-ray.

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Differential Diagnosis
MFS can be mistaken with similar disorders with overlapping clinical features. The most
common differential is brainstem (Bickerstaff) encephalitis and a pharyngeal-cervical-brachial
weakness GBS variant. Distinguishing features of brainstem encephalitis is the presence of
hyperreflexia and encephalopathy. The pharyngeal-cervical-brachial variant of GBS is clinically
characterized by acute weakness of the oropharyngeal, neck, and shoulder muscles, dysphagia,
and facial paresis with preserved reflexes and strength in the lower extremities. These 3 disorders
have in common the presence of anti-ganglioside antibodies in CSF. The gradual onset of MFS is
a key factor in distinguishing it from acute brainstem stroke. Other differential diagnoses to
consider include Wernicke encephalopathy, ocular myasthenia gravis, Lambert Eaton syndrome,
multiple sclerosis, and sarcoidosis, among other autoimmune disorders. Neuroimaging (CT/MRI
or MRA) and electrodiagnostic (EMG, nerve conduction, or evoked potential), among other
ancillary tests in the central and peripheral nervous system can be helpful to exclude these
alternative diagnoses. Of note, under an uncertain GBS variant besides MFS and/or suspicion of
brainstem encephalitis that is severely debilitating, the treatment regimen remains unchanged:
IVIG or plasma exchange.

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Prognosis
The outcome of MFS is usually good with case fatality of less than 5%. The mean recovery times
range between 8 to 12 weeks. Residual symptoms may be present in some patients, and
recurrence has been reported in the literature. In GBS, however, hyponatremia is predictive of
poor outcome with the development of syndrome of inappropriate antidiuretic hormone secretion
(SIADH). About 21% to 48% of GBS patients can suffer from hyponatremia. Hyponatremia as
an independent predictive factor for mortality has been called into question in newer studies,
making respiratory status and complications in the ICU as the top predictors of mortality and
morbidity.

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Complications
The most common complication is generalized fatigue, reported in three-fourths of patients with
GBS and MFS. Recovery tends to be better in patients with MFS. About one-third of all patients
still experience pain one year after onset. Severe complications are more likely in patients with
an extended ICU stay. These include sepsis, pneumonia, pulmonary embolism from a deep vein
thrombosis (e.g., immobilization), and gastrointestinal bleeding. Other complications may arise
from patients suffering from autonomic dysfunction including arrhythmias, and ileus.
Respiratory muscle fatigue is a feared complication in ICU patients older than 50 years, due to
the risk of mortality from respiratory failure. Among severely affected patients, 20% to 33% may
be unable to walk for more than 6 months after symptom onset, especially if infected with C.
jejuni. Patients may also suffer from chronic psychiatric illness due to persistent pain and
disability.

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Postoperative and Rehabilitation Care
A patient discharged with GBS or MFS may need a lengthy and intense program of
physiotherapy to recover function. Complete recovery is dependent on many factors, including
severity of neurological deficits at onset, the age of patient, complications, motivation, and goals
of the patient, among others. A thorough physical and occupational therapy assessment in the
hospital is essential to identify the patient’s needs and goals of therapy. Patients with GBS and
MFS frequently begin acute care and PT/OT therapy in the intensive care unit, then progress to a
sub-acute setting in a rehabilitation department or outside nursing/rehab facility and eventually
translate to home-based or outpatient therapy. The assessment includes a patient/caregiver
interview, sensory function, skin inspection, testing joint range motion, manual muscle testing,
functional testing (e.g., ADL/IADL pre and post-illness), mobility, respiration (e.g., vital
capacity and inspiratory force), autonomic dysfunction, and endurance.

The principal goals of therapy includes achieve optimal muscle use as tolerated by pain, and use
supportive equipment to help patient resume functional activity as close to baseline as possible.
Activities are increased gradually as tolerated, with increased muscle repetitions at low
resistance to avoid injuries, teaching energy conservation, and training caregivers in transferring
technique and mobilization of the patient at home. In summary, PT/OT are integral parts of the
recovery and management of MFS. An appropriate plan of care can help a patient minimize pain,
increase strength and endurance, and prevent secondary complications including overuse damage
to muscles and joints while improving balance, mobility, and restoring functional activity.

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Consultations
Neurology and possibly neuromuscular subspecialty consultations, if possible, should be
considered for GBS or MFS cases. If needed, a critical care intensivist may need to be consulted
for ICU admission and management.

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Deterrence and Patient Education


What are Miller Fisher and Guillain-Barre syndrome?

Miller Fisher Syndrome (MFS) is one of the rare forms of a spectrum of Guillain-Barré
syndrome (GBS). It is a neurological condition that causes mild to severe muscle weakness. It is
caused by an immune system reaction against certain proteins in our nerves important for
movement, sensation, and function. This syndrome can begin after certain bacterial or viral
infections found in our food, or the environment infects us. The body confuses the nerves with
the bacterial or viral proteins leading to nerve damage, resulting in symptoms observed. The
most common bacterial trigger for GBS and MFS is Campylobacter jejuni which can cause
abdominal pain and diarrhea. Viruses that may cause MFS and GBS include HIV infection,
Epstein-Barr (mononucleosis), and Zika virus.

What are the symptoms of Miller Fisher syndrome?

Miller Fisher syndrome causes eye and muscle weakness on both sides of the body. It may cause
trouble walking and balance problems. It can start in the legs and slowly spread to the arms and
face. Some people lose the ability to move their legs, arms, or face. Some people may have
trouble breathing because it affects their respiratory muscles. Other symptoms of MFS can
include:

1. Tingling or numbness in the hands or feet


2. Pain in the back, legs, or arms
3. Problems with eye movement and/or blurry vision
4. Loss of coordination in the arms and legs

What are the tests for Miller Fisher syndrome?

The doctor or nurse will ask about symptoms and do an exam. He or she will also do tests to be
sure you have MFS and differentiate from other possible illnesses. Tests can include:

1. A lumbar puncture (spinal tap): A procedure where the doctor puts a thin needle in the
lower back and removes a small amount of spinal fluid. Spinal fluid is the fluid that
surrounds the brain and spinal cord. That fluid is sent for further testing.
2. Antibody testing: The doctor may send a blood test to determine whether the patient has
MFS. There are specific sugar chains in the nerve called a ganglioside that the body
attacks. This antibody is present in most, but not all, patients.
3. Nerve conduction studies: This test can show whether the nerves are carrying electrical
signals the correct way.
4. Electromyography (EMG): This test shows whether the muscles are responding to the
electrical signals from the nerves in the correct way.
5. Neuroimaging CT/MRI of the spine: This test can show whether the body is attacking the
nerves cells in the spinal cord. It would light up if it is positive.
6. Other blood tests to rule out other illnesses

How is Miller Fisher syndrome treated?

Treatment for Miller Fisher syndrome is the same as GBS and involves different components
depending on severity:

1. Treatments for problems caused by the MFS: People with MFS are usually treated in the
hospital to rule out a more life-threatening version of GBS. In the hospital, the doctor will
monitor a heartbeat, breathing, and general health. If breathing problems are present and
begin to get worse, you may need a breathing tube. A breathing tube goes down the throat
and into the lungs and provides oxygen to your body.
2. Treatment for pain: Doctors can use different medicines to treat pain, always watching
that they do not use medication that could make breathing harder
3. Treatment for the condition itself: There is no immediate cure for MFS and GBS.
However, there safe and effective treatments that can help the body improve in a shorter
time. These treatments are immunoglobulins (IVIG) and plasma exchange. IVIG is a
medicine that helps the body rid itself from the proteins (antibodies) that damage the
nerves. Plasma exchange is an alternative procedure where a machine pumps blood from
the body and removes the proteins attacking the nervous system. Then the machine
returns the blood to the body clean.
4. Physical rehabilitation of affected muscles: People whose muscles get very weak may
need to go to a facility for physical and neurological rehabilitation. It usually consists of 1
to 2 weeks of intense rehabilitation with a comprehensive team of health professionals.
Their goal is to strengthen the body’s muscles as close to the previous function as
possible.

How long does Miller Fisher syndrome last?

Acute MFS usually lasts a few weeks and can be shortened with appropriate treatment. Complete
resolution of symptoms last several weeks to few months depending on the severity of
presentation. The good news is that MFS tends to have a more benign course compared to other
forms of GBS. Therefore, most people will recover completely and have no long-term muscle
weakness. Very few people have muscle weakness that lasts years.

What can I do to prevent Miller Fisher Syndrome?

Certain bacterial and viral infections can cause these disorders. Some measures can be followed
to reduce contact with eliciting agents including:

1. Improve sanitation: Washing hands thoroughly with soap and water before and after
handling poultry and generally when touching raw meat
2. Cooking poultry products thoroughly
3. Disinfecting water supply
4. Avoiding raw milk consumption
5. Safe sex practices
6. Immunocompromised patients must adhere to medication regimen to avoid certain viral
infections

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Pearls and Other Issues


 MFS and GBS are thought to result from an aberrant acute autoimmune response to a
preceding infection (e.g. Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, or
Human immunodeficiency virus (HIV) through a molecular mimicry mechanism that
leads to the destruction of myelinating components of the central and peripheral nervous
system.
 MFS variant represents 1 to 2 in 1,000,000, with average ocurrence during mid-adulthood,
affecting more men than women 2:1, and disproportionate incidence in Asians
communities.
 The clinical hallmark of MFS is a triad presentation of acute ophthalmoplegia, areflexia,
and ataxia in the setting of a preceding bacterial or viral illness. Associated symptoms
also include diplopia or blurred vision, dysarthria, dizziness, and extremity tingling with
or without cranial nerve involvement.
 Physical exam findings include facial paresis, distal hyporeflexia without signs of upper
motor neuron dysfunction, and loss of light and vibratory sensation in the distal
extremities. Autonomic dysfunction although possible, is less likely to occur in MFS.
 Although diagnosis of MFS is mostly clinical, supportive testing can help differentiate
from other disease or variants of GBS, including a lumbar puncture with CSF studies,
anti-ganglioside antibody testing, nerve conduction studies, electromyography, CT/MRI
imaging, among other ancillary testing.
 Differential diagnosis include brainstem (Bickerstaff) encephalitis, pharyngeal-cervical-
brachial weakness GBS variant, and other neuromuscular autoimmune disorders. MFS
and these 2 variants have in common the presence of anti-ganglioside antibodies in CSF.
 MFS is mainly treated with adequate supportive care, pain control, and respiratory
support as needed, and immunotherapy. IVIG or plasmapharesis are the mainstays of
therapy. Corticosteroids are no longer recommended and are ineffective in the majority of
cases.
 The prognosis of MFS is usually good with case fatality of less than 5%. The mean
recovery times range between 8 to 12 weeks. Although recurrence may occur, it is
uncommon.
 The most common complication is generalized fatigue. About one-third of all patients
still experience pain one year after onset. Severe complications are more likely in patients
with an extended ICU course.
 ICU transfer from the emergency department is reserved for the sickest patients who
meet certain criteria including impending respiratory failure. Major criteria includes
hypercapnia PaCO2 greater than 48 mm Hg, hypoxemia PaO2 less than 56 mm Hg at
room air, vital capacity less than 15 mL/kg of body weight, and negative inspiratory force
less than -30 cm H2O. Minor criteria include an inefficient/weak cough, dysphagia, and
atelectasis as evidenced in a chest x-ray.
 Early physical and occupational therapy can lead to a faster recovery in patients with any
form of GBS, including MFS. Goals of therapy should be individualized according to the
patient's wishes, it includes achieving optimal muscle use as tolerated by pain, and use
supportive equipment to help patient resume functional activity as close to pre-
hospitalization baseline as possible.

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Questions
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