‘nit5r2020 Guillain-Barre Syndrome Workup: Approach Considerations, Peripheral Neuropathy Workup, Biochemical Screening
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Guillain-Barre Syndrome Workup
Updated: Jun 24, 2020
Author: Michael T Andary, MD, MS; Chief Editor: Milton J Klein, DO, MBA more...
WORKUP
Approach Considerations
Guillain-Barré syndrome (GBS) is generally diagnosed on clinical grounds. Basic laboratory studies,
such as complete blood counts (CBCs) and metabolic panels, are normal and of limited value in the
workup. They are often ordered, however, to exclude other diagnoses and to better assess functional
status and prognosis. The ordering of specific tests should be guided by the patient's history and
presentation
Electromyography (EMG) and nerve conduction studies (NCS) can be very helpful in the diagnosis.
Abnormalities in NCS that are consistent with demyelination are sensitive and represent specific
findings for classic GBS. Delayed distal latencies, slowed nerve conduction velocities, temporal
dispersion of waveforms, conduction block, prolonged or absent F waves, and prolonged or absent H-
reflexes are all findings that support demyelination. Needle EMG may be normal in acute nerve
lesions, and it may take 3-4 weeks for fibrillation to develop. In the acute phase, the only needle EMG.
abnormality may be abnormal motor recruitment, with decreased recruitment and rapid firing motor
units in weak muscles. Unfortunately, electrodiagnostic studies can be completely normal in acute
GBS and a normal study does not rule GBS, !104, 105]
Frequent evaluations of pulmonary function parameters should be performed at bedside to monitor
respiratory status and the need for ventilatory assistance.
Lumbar puncture for cerebrospinal fluid (CSF) studies is recommended. During the acute phase of
GBS, characteristic findings on CSF analysis include albuminocytologic dissociation, which is an
elevation in CSF protein (>0.55 g/L) without an elevation in white blood cells. The increase in CSF
protein is thought to reflect the widespread inflammation of the nerve roots
Imaging studies, such as magnetic resonance imaging (MRI) and computed tomography (CT)
scanning of the spine, may be more helpful in excluding other diagnoses, such as mechanical causes
of myelopathy, than in assisting in the diagnosis of GBS
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Peripheral Neuropathy Workup
A basic peripheral neuropathy workup is recommended in cases in which the diagnosis is uncertain
These studies may include the following
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+ Thyroid panel
+ Rheumatology profiles
+ Vitamin B-12
+ Folic acid
+ Hemoglobin A1C
+ Erythrocyte sedimentation rate (ESR)
+ Rapid protein reagent
+ Immunoelectrophoresis of serum protein
+ Tests for heavy metals
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Biochemical Screening
Biochemical screening includes the following studies:
* Electrolyte levels
+ Liver function tests (LFTs)
* Creatine phosphokinase (CPK) level
» ESR
The following should be considered:
» LFT results are elevated in as many as one third of patients
+ CPK and ESR may be elevated with myopathies or systemic inflammatory conditions
* Astool culture for C jejuni and a pregnancy test are also indicated
+ The syndrome of inappropriate antidiuretic hormone (SIADH) may occur
@medicine
Serologic Studies
Serologic studies are of limited value in the diagnosis of GBS. Assays for antibodies to the following
infectious agents may be considered:
+ C jejuni
+ Cytomegalovirus (CMV)
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+ Epstein-Barr virus (EBV)
+ Herpes simplex virus (HSV)
* HIV
+ Mycoplasma pneumoniae
An increase in titers for infectious agents, such as CMV, EBV, or Mycoplasma, may help in
establishing etiology for epidemiologic purposes. HIV has been reported to precede GBS, and
serology should be tested in high-risk patients to establish possible infection with this agent.
Serum autoan'
10
S
Serum autoantibodies are not measured routinely in the workup of GBS, but results may be helpful in
patients with a questionable diagnosis or a variant of GBS. Antibodies to glycolipids are observed in
the sera of 60-70% of patients with GBS during the acute phase, with gangliosides being the major
target antigens. [198]
Specific antibodies found in association with GBS include the following:
‘+ Antibodies to GM1: Frequently found in the sera of patients with the acute motor axonal
neuropathy (AMAN) or acute demyelinating polyradiculoneuropathy (AIDP) variants of GBS
+ Anti-GM1 antibodies: Elevated titers are closely associated antecedent C jejuni infections
+ Anti-GQ1b antibodies: Found in patients with GBS with ophthalmoplegia, including patients with
the Miller-Fisher variant
Other antibodies to different major and minor gangliosides also have been found in GBS patients.
medicine
Nerve Conduction Studies
Nerve conduction studies (NCS) can be very helpful in the diagnostic workup and prognostic
evaluation of patients with suspected GBS. Abnormalities in NCS that are consistent with
demyelination are sensitive and represent specific findings for classic GBS. {941
Signs of demyelination can include the following:
+ Nerve conduction slowing
+ Prolongation of the distal latencies
* Prolongation or absence of the F-waves |". 2)
+ Conduction block or dispersion of responses: Evidence frequently demonstrated at sites of
natural nerve compression
Changes on NCS should be present in at least 2 nerves in regions that are not typical for those
associated with compressive mononeuropathies (preferentially in anatomically distinct areas, such as
an arm and a leg or a limb and the face).
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Although NCS results classically show a picture of demyelinating neuropathy in most patients, axonal
neuropathy and inexcitable results are found in certain subgroups. The inexcitable studies may
represent either axonopathy or severe demyelination with distal conduction block
Other characteristics of GBS include the following:
* Nerve motor action potentials: May be decreased, but this is technically difficult to determine
until the abnormality is severe; the extent of decreased action potentials correlates with
prognosis
+ Compound muscle action potential (CMAP): Amplitude may be decreased
+ Sensory abnormalities: Exhibited by most patients, but these findings are much less marked
than they are in motor nerves; sural sparing is a common finding in patients with clinical sensory
deficits
+ Abnormal (delayed, small, or absent) H-reflex: May be noted
The needle examination is of limited value in GBS. Reduced motor unit recruitment and absent
denervation help to support the suggestion of a demyelinating mechanism, although the same
changes can be observed in early axonal damage with pending wallerian degeneration. In severe
cases, denervation changes may be observed later in the disease course.
In the axonal variant of the disease, absent or markedly reduced distal CMAP is observed on NCS.
On needle examination, profuse and early denervation potentials (fibrillations) also support the
conclusion that there has been axonal injury.
In some cases, neurophysiologic testing is normal in patients with GBS, especially in the first 1-2
weeks of the disease. This is believed to be due to the location of demyelinating lesions in proximal
sites not amenable to study. !'°5] For example, a retrospective, single-center study by Luigetti et al
found that in 37% of patients with GBS who underwent an early nerve conduction study (ie, 4 days or
less after disease onset), neurophysiologic results were normal. As a result, the investigators, whose
study involved 71 patients with GBS, suggested that extensive neurophysiologic assessment should
be performed in patients who are in the early phases of GBS, [107]
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Pulmonary Function Tests
Maximal inspiratory pressures and vital capacities are measurements of neuromuscular respiratory
function and predict diaphragmatic strength. Maximal expiratory pressures also reflect abdominal
muscle strength. Frequent evaluations of these parameters should be performed at bedside to monitor
respiratory status and the need for ventilatory assistance.
Forced vital capacity (FVC) is very helpful in guiding disposition and therapy. '82] Patients with an FVC.
of less than 15-20 mL/kg, maximum inspiratory pressure of less than 30 cm water, or a maximum
expiratory pressure of less than 40 cm water generally progress to require prophylactic intubation and
mechanical ventilation. Respiratory assistance should also be considered when there is a decrease in
oxygen saturation (arterial partial pressure of oxygen [POz] < 70 mm Hg).
Negative inspiratory force (NIF) is a relatively easy bedside test to measure respiratory muscle
function and can easily be performed every half hour to hour in difficult cases. Normal is usually
tps
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greater than 60 cm water. If the NIF is dropping or nears 20 cm water, respiratory support needs to be
available.
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Lumbar Puncture
Most, but not all, patients with GBS have an elevated CSF protein level (>400 mg/L), with normal CSF
cell counts. Elevated or rising protein levels on serial lumbar punctures and 10 or fewer mononuclear
cells/mm? strongly support the diagnosis.
A normal CSF protein level does not rule out GBS, however, as the level may remain normal in 10% of
patients. CSF protein may not rise until 1-2 weeks after the onset of weakness.
Normal CSF cell counts may not be a feature of GBS in HIV-infected patients. CSF pleocytosis is well
recognized in HIV-associated GBS.
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Magnetic Resonance Imaging
MRI is sensitive, but nonspecific, for diagnosis. However, it can reveal nerve root enhancement and
may be an effective diagnostic adjunct, [18. 109]
Spinal nerve root enhancement with gadolinium is a nonspecific feature seen in inflammatory
conditions and is caused by disruption of the blood-nerve barrier. Selective anterior nerve root
enhancement appears to be strongly suggestive of GBS, !1"°] with the cauda equina nerve roots being
enhanced in 83% of patients.
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Other Studies
Muscle biopsy may help to distinguish GBS from a primary myopathy in unclear cases. Many different
abnormalities may be seen on electrocardiography, including second- and third-degree atrioventricular
(AV) block, T-wave abnormalities, ST depression, QRS widening, and various rhythm disturbances.
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Histology
Lymphocyte and macrophage infiltration is observed on microscopic examination of peripheral nerves,
with macrophage influx believed to be responsible for the multifocal demyelination seen in GBS. A
variable degree of wallerian degeneration also can be observed with severe inflammatory changes.
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Cellular infiltrates are scattered throughout the cranial nerves, nerve roots, dorsal root ganglions, and
peripheral nerves.
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Treatment & Management
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