Clinical features and diagnosis of

Clinical features and diagnosis of Guillain-Barré syndrome in adults

TOPIC OUTLINE

SUMMARY & RECOMMENDATIONS
INTRODUCTION
EPIDEMIOLOGY
CLINICAL FEATURES
Laboratory features
GBS VARIANTS
Acute inflammatory demyelinating polyneuropathy
Acute motor axonal neuropathy
Acute motor and sensory axonal neuropathy
Miller Fisher syndrome
Bickerstaff encephalitis
Pharyngeal-cervical-brachial weakness
Other variants
DIAGNOSTIC EVALUATION
Cerebrospinal fluid analysis
Clinical neurophysiology studies
Antibodies
Diagnostic criteria
DIFFERENTIAL DIAGNOSIS
Chronic inflammatory demyelinating polyneuropathy
Other polyneuropathies
Spinal cord disorders
Neuromuscular junction disorders
Muscle disorders
Differential diagnosis of Miller Fisher syndrome
INFORMATION FOR PATIENTS
SUMMARY AND RECOMMENDATIONS
REFERENCES
GRAPHICS View All
TABLES
Differential diagnosis of GBS
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Clinical features and diagnosis of Guillain-Barré syndrome in adults
Author
Francine J Vriesendorp, MD
Section Editors
Jeremy M Shefner, MD, PhD
Ira N Targoff, MD
Deputy Editor
John F Dashe, MD, PhD
Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Oct 2013. | This topic last updated: Feb 4, 2013.
INTRODUCTION — The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré syndrome (GBS), after the authors of early descriptions of the disease. Guillain-Barré syndrome is a heterogeneous condition with several variant forms. Most often, GBS presents as an acute monophasic paralyzing illness provoked by a preceding infection.
The clinical features and diagnosis of GBS in adults will be reviewed here. Other aspects of GBS are discussed separately. (See "Treatment and prognosis of Guillain-Barré syndrome in adults" and "Overview of Guillain-Barré syndrome in children" and "Treatment of Guillain-Barré syndrome in children" .)
EPIDEMIOLOGY — GBS occurs world-wide with an overall incidence of 1 to 2 per 100,000 per year [ 1,2 ]. While all age groups are affected, the incidence increases by approximately 20 percent with every 10-year increase in age beyond the first decade of life. In addition, the incidence is greater in males than in females.
CLINICAL FEATURES — The cardinal clinical features of Guillain-Barré syndrome (GBS) are progressive, fairly symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. Patients usually present a few days to a week after onset of symptoms. The weakness can vary from mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory, and bulbar muscles.
Studies from the United States and Europe, reflecting primarily patients with acute inflammatory demyelinating polyneuropathy (AIDP), show that GBS is associated with the following clinical features [ 3 ]:
Although the weakness usually starts in the legs, it begins in the arms or facial muscles in about 10 percent of patients.
Severe respiratory muscle weakness necessitating ventilatory support develops in 10 to 30 percent [ 4 ].
Facial weakness occurs in more than 50 percent and oropharyngeal weakness eventually occurs in 50 percent.
Oculomotor weakness occurs in about 15 percent of patients.
Paresthesias in the hands and feet accompany the weakness in more than 80 percent of patients, but sensory abnormalities on examination are frequently mild.
Pain, typically located in the back and extremities, can be a presenting feature and is reported during the acute phase by 66 percent of patients with all forms of GBS [ 5,6 ].
Dysautonomia occurs in 70 percent of patients and manifests as symptoms that include tachycardia (the most common), urinary retention, hypertension alternating with hypotension, orthostatic hypotension, bradycardia, other arrhythmias, ileus, and loss of sweating. Severe autonomic dysfunction is important to recognize since this is occasionally associated with sudden death [ 7 ].
Unusual features of GBS include papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes [ 8 ]. The syndrome of inappropriate antidiuretic hormone secretion (SIADH) has also been reported in association with GBS [ 9-12 ].
GBS usually progresses over a period of about two weeks. By four weeks after the initial symptoms, 90 percent of GBS patients have reached the nadir of the disease. Disease progression for more than eight weeks is consistent with the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). (See "Treatment and prognosis of Guillain-Barré syndrome in adults" .)
Laboratory features — The typical finding with lumbar puncture in patients with GBS is an elevated cerebrospinal fluid (CSF) protein with a normal white blood cell count. This finding is called albuminocytologic dissociation, and is present in up to 66 percent of patients with GBS at one week after onset of symptoms. (See 'Cerebrospinal fluid analysis' below.)
Clinical neurophysiology studies (ie, electromyography and nerve conduction studies) show evidence of an acute polyneuropathy with predominantly demyelinating features in acute inflammatory demyelinating polyradiculoneuropathy (AIDP), while the features are predominantly axonal in acute motor axonal neuropathy (AMAN) and acute sensorimotor axonal neuropathy (AMSAN).
Glycolipid antibodies may be associated with different forms or aspects of GBS. (See "Pathogenesis of Guillain-Barré syndrome in adults", section on 'Molecular mimicry' .)
Antibodies against GQ1b (a ganglioside component of nerve) are found in 85 to 90 percent of patients with the Miller Fisher syndrome. (See 'Miller Fisher syndrome' below.)
Antibodies to GM1, GD1a, GalNac-GD1a, and GD1b are mostly associated with axonal variants of GBS. (See 'Acute motor axonal neuropathy' below and 'Acute motor and sensory axonal neuropathy' below.)
Antibodies to GT1a are associated with swallowing dysfunction [ 13 ].
Antibodies to GD1b are associated with pure sensory GBS [ 14 ]. (See 'Other variants' below.)
Laboratory testing for patients with GBS is discussed below. (See 'Diagnostic evaluation' below.)
GBS VARIANTS — Historically, the Guillain-Barré syndrome (GBS) was considered a single disorder. It now is recognized as a heterogeneous syndrome with several variant forms. Each form of GBS has distinguishing clinical, pathophysiologic, and pathologic features.
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common form in the United States and Europe, representing approximately 85 to 90 percent of cases. The clinical variant Miller Fisher syndrome (MFS), characterized by ophthalmoplegia, ataxia, and areflexia, occurs in 5 percent of cases in the United States and 25 percent of cases in Japan [ 15 ].
Acute motor axonal neuropathy (AMAN) and acute sensorimotor axonal neuropathy (AMSAN) are primary axonal forms of GBS. These forms are frequently observed in China, Japan, and Mexico, but they also comprise an estimated 5 to 10 percent of GBS cases in the United States [ 16 ].
Other, less frequent, clinical variants are recognized and listed below. (See 'Other variants' below.)
Acute inflammatory demyelinating polyneuropathy — As noted above, AIDP is the most common form in the United States and Europe, representing approximately 85 to 90 percent of cases. The typical clinical features are a progressive, fairly symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. (See 'Clinical features' above.)
Peripheral nerve myelin is the target of immune attack in AIDP. The immunologic basis of peripheral nerve demyelination in AIDP is discussed separately. (See "Pathogenesis of Guillain-Barré syndrome in adults", section on 'Mechanisms' .)
Inflammatory demyelination is thought to start at the level of the nerve roots, leading to electrophysiologic conduction slowing and conduction blocks with resultant muscle weakness. Multifocal, patchy, widespread peripheral nerve demyelination follows, causing increasing paralysis. Peripheral nerve remyelination occurs relatively rapidly over several weeks to months. However, in a small percentage of patients, there is superimposed significant axonal degeneration with markedly delayed and incomplete recovery.
The earliest abnormalities seen on clinical neurophysiology studies in patients with AIDP are prolonged or absent F waves and absent H reflexes, reflecting demyelination at the level of the nerve roots [ 17,18 ]. Increased distal latencies and conduction blocks with temporal dispersion of motor responses follow [ 19,20 ]. Significant slowing of nerve conduction velocities is usually not seen until the third or fourth week [ 20 ]. Sensory nerve conduction studies (NCS) reveal absent responses or slowed conduction velocities. Typically, the sural sensory response is normal, while median and ulnar sensory responses are affected (sural sparing) [ 18,21 ].
Needle examination (electromyography [EMG]) of weak muscles shows reduced recruitment. Ancillary studies, such as facial NCS and reflexes, are occasionally helpful.
An average distal motor response amplitude reduction to less than 20 percent of normal, measured within 30 days after onset of symptoms, has been shown to predict poorer recovery [ 22 ].
A small percentage of AIDP patients develop severe secondary axonal degeneration, and clin