Good afternoon, everyone.

Today, I’ll be talking about my diagnostic algorithm for Guillain-

Barré syndrome. GBS consists of a group of neuropathic conditions characterized by progressive
weakness and diminished reflexes that is autoimmune in nature.

The mechanism behind GBS is thought to be an inflammatory neuropathy caused by a cross-

reaction between antibodies, induced by specific infections, and host antigens. These infectious
agents, for example Campylobacter jejuni, express lipooligosaccharides on their cell wall that
are similar to gangliosides on host nerve tissue. This molecular mimicry creates antiganglioside
antibodies that attack host nerves. The specific antibodies that are stimulated and their target
areas differentiate the subtypes of GBS.

All of this results in inflammation in the nerves. Complement deposition results in a

characteristic disintegration of the myelin sheath. This demyelination causes a block in nerve
conduction and it’s what causes the flaccid paralysis and sensory disturbances found in patients
with GBS.

In more severe cases, secondary axonal degeneration usually occurs.

These are the types of GBS as mentioned in the Harrison’s, and Acute Inflammatory
Demyelinating Polyneuropathy is considered the most common subtype. This occurs more
often in adults. This subtype is characterized by its initial attack on Schwann cells and is
associated with widespread damage and inflammation, with variable secondary axonal damage.

Acute Motor and Acute Motor-Sensory Axonal Neuropathies are axonal variants and are often
more clinically severe.

The last type which was mentioned is the Miller Fisher syndrome, which accounts for 5% of
cases and is notable for its presentation, which I will be discussing later.

In assessing a patient with suspected GBS, it’s important to identify the triggering conditions
that have led to its development. Most commonly, patients will report of respiratory or
gastrointestinal symptoms around 1-3 weeks before the onset of neurologic symptoms that
coincide with an antecedent infection. GI symptoms have been more likely to be associated
with slower recovery from GBS.

It’s also important to ask patients regarding recent immunizations. Although there have been
reports that GBS occurs after receiving tetanus, hepatitis, and influenza shots, these
immunizations lead to very little increased risk. The most recent study with attributable risk for
GBS is the 2009 A(H1N1) vaccine which caused up to two cases of GBS per 1 million doses.

GBS manifests as a rapidly evolving, areflexic motor paralysis with or without sensory
disturbances. Initial symptoms typically include weakness, tingling, numbness, or pain. The
usual pattern for the paralysis is an ascending paralysis that may first be noticed as rubbery
legs. The advancing weakness may compromise respiratory muscles and up to 30% may require
mechanical ventilation at some time during their illness. Sensory symptoms are generally mild.

For the following features, although these are not required for the diagnosis of GBS, these
findings further support in its diagnosis. Patients may complain of autonomic involvement,
wherein cardiac dysfunction, in the form of BP fluctuations, postural hypotension, and
dysrhythmias, are the most common manifestations.

Lower cranial nerve involvement may also be noted, causing bulbar weakness.

Pain, especially with movement, is reported in more than 50% of patients, and it’s usually
described as severe, deep, aching, or cramping in the affected muscles or back, and is often
worse at night.

Generally, the course of GBS is as follows, wherein clinical worsening occurs and then peaks
within 4 weeks, and then proceeds into a plateau phase which is identified when there are
persistent, unchanging symptoms, followed days later by gradual improvement.

Again, just to emphasize the differences in the subtypes apart from their common
presentations, the most prominent feature of AIDP is a bilateral, relatively symmetrical
ascending weakness of the limbs that progress rapidly.

In the acute motor axonal subtype, most cases exhibit only motor symptoms, and the deep
tendon reflexes may be preserved.

Acute motor-sensory axonal neuropathy is identifiable because there is a predominance of

sensory involvement.

And as I mentioned, Miller Fisher Syndrome may be identified as patients also present with
ophthalmoplegia, ataxia and areflexia of the limbs without weakness.

Apart from clinical findings, the diagnosis of GBS also depends on repeated neurologic
examinations. Lumbar puncture and neurophysiology testing should be done in all patients with
suspected GBS, and specific findings strongly support GBS diagnosis.

Although results for LP may be normal within 48 hours of symptom onset, GBS classically
presents with increased CSF protein and a normal WBC count. This cyto-albuminologic
dissociation is characteristic but not diagnostic of GBS. The normal WBC usually helps
differentiate GBS from other infectious, inflammatory, and malignant diseases.

In conducting electrodiagnostic studies, it’s important to test at least 3 sensory and 3 motor
nerves to get adequate data, and the sural nerve should be avoided as it often remains normal
in GBS.
In AIDP, the earliest features include the following. Sequential studies will show slowing of
nerve conduction and conduction block, which are present in up to 80% of patients with GBS.
For primary axonal pathologies, the primary feature is a reduction in the amplitude of CMAPs
without conduction slowing.

GBS is a descriptive entity, and the diagnosis is made by recognizing the clinical pattern of
symmetrical paralysis with areflexia, absence of fever or other systemic symptoms, and the
presence of a characteristic antecedent event. There are no pathognomonic clinical features
and no biomarkers for GBS.

In 2011, the Brighton Criteria was developed for the case definition of GBS, taking into account
the clinical, electrophysiological and laboratory features of the disease. The criteria also
account for the level of diagnostic certainty, with level 1 as the level with the highest diagnostic
certainty for GBS, and level 4, which is considered as “a case that is reported as GBS, possibly
due to insufficient data for further classification”.

For the differentials, it’s important to consider other causes of peripheral neuropathies and
paralysis. Recognizing specific clinical patterns is important to proceed with diagnostic workup
in patients who present with signs and symptoms of neuropathy.

Chronic inflammatory demyelinating polyneuropathy or CIPD, although they share several

features with GBS, is distinguished by its chronic course, and should be considered especially if
patient continues to deteriorate after 9 weeks.

Acute myelopathies may be considered in patients with prolonged back pain and sphincter
disturbances.

GBS and vasculitis similarly present similarly; however, systemic vasculitis should be considered
if patients also present with constitutional symptoms. Nerve and muscle biopsies may be taken
to rule out this differential.

Myasthenia gravis is an NMJ disorder characterized by weakness and fatigability, however, it’s
not usually associated with areflexia or impairment of sensation or other neurologic function.

Lastly, myopathies and metabolic derangements such as severe hypophosphatemia should also
be considered in patients presenting with muscle weakness.

Main predictors of poorer prognosis in GBM are listed in this table. Recovery period may range
from a few weeks to a few years. Majority patients are able to walk independently at 6 months
but up to 30% of patients still have residual weakness after 3 years.