This is a presentation of Guillain-Barre Syndrome, commonly abbreviated as GBS. The objectives of this presentation are to understand the etiology and pathogenesis of GBS, to identify its clinical symptoms and signs, to be able to diagnose GBS and note it's differential diagnosis, to learn the management and prognosis of GBS. GBS is also known as Landry-Guillain Barre Strohl syndrome and Acute Inflammatory Demyelinating Polyradiculo-neuropathy, or AIDP. In the year 1916, Guillain and Barre first described the clinical and laboratory features of the disease. Earlier in 1859, Landry had reported that similar clinical findings in his patients. GBS is the most common cause of acute or sub-acute generalized paralysis. Its incidence is about 1 to 2 per 100,000. GBS occurs all over the world, and affects all ages, and both sexes.
GBS is an acute demyelinating polyradiculo-neuropathy, which has an autoimmune basis. It results from immune responses to non-self antigens, such as microbial antigens that are misdirected against host nerve tissue, in particular, myelin in peripheral nerves through molecular mimicry mechanisms. Both cellular and antibody [INAUDIBLE] mechanisms play a role in the pathogenesis of GBS.
Neural targets are the ganglial sites which are complex [INAUDIBLE] lipids, present in large quantity in human nervous tissue, and in key sites like [INAUDIBLE]. Antibodies, or GM1 are common in GBS. There are seen in 20% to 50% of cases. The flaccid paralysis and sensory disturbance, seen in GBS, is due to conduction block, as a result of the demyelination. Axonal connections remain intact, hence rapid and complete recovery is possible when remyelination occurs.
However, in ceemea forms, secondary axonal degeneration can occur. Such cases are associated with slow recovery and more severe residual disability. Approximately 70% of the cases of GBS occur one to three weeks after an acute respiratory or gastrointestinal infection. Campulobacter jejuni, human herpes viruses, such CMV and EBV, as well as mycoplasma pneumoniae have been identified as antecedent infections. Some cases are preceded by recent immunization. GBS also across more frequently than can be attributed to chance alone in patients with lymphoma, HIV, and SLE. GBS evolves as rapidly evolving [INAUDIBLE] motor paralysis, with or without sensory disturbance. Paresthesia and dysesthesia in toes and fingertips are often the initial symptoms.
Weakness typically evolves over a few days. Pain is common in the easy stages of GBS. Sensory deficits are usually mild. All the muscle weakness results dysphagia and dysphonia. Respiration muscle involvement can present a shortness of breath. Transient bladder dysfunction can occur in severe cases. Lower limb weakness occurs and progresses over days to weeks, followed by upper limb weakness, hence the term ascending weakness.
Both proximal and distal muscles can be involved. Intercostal, neck, cranial muscles may be affected later. Total paralysis and respiratory failure occurs in about 5% of cases.
Facial diplegia occurs in more than half of the cases. Paresthesia and slight decreased sensation in toes and fingers is one of the earliest symptoms. Sensory loss is variable during the first few days, and barely detectable clinically. Vibration and joint position sense is usually impact in toes and fingers by the end of the first week. Deep tendon reflexors attenuate and disappear within the first few days of onset. Autonomic dysfunction in GBS is common.
The usual manifestations of autonomic dysfunction include blood pressure fluctuation, postural hypertension, cardiac dysrhythmias, flushing, diaphoresis, or loss of sweating and urinary retention. There are regional variants of GBS. These include Miller Fisher Syndrome, which is characterized by ophthalmoplegia, ataxia, and areflexia. In the pharayngo-cervico-brachial pattern, the pharyngo and upper limb muscles are predominantly affected. In another rare variant, there is severe bulbar and facial paralysis, with variable limb weakness.
A pure sensory variant and an variant with only pandysautonomia has also been described. Differential diagnosis on GBS include other causes of acute neuropathy, such as porphyria, critical illness neuropathy, diphtheria, and other toxic neuropathies. Neuromuscular junction disorders, like myasthenia gravis and botulism can mimic GBS. Other differentials include acute myopathies and certain central nervous system disorders like poliomyelitis, transverse myelitis, and basilar artery thrombosis. Blood tests are performed to exclude conditions that mimic GBS. Nerve conduction studies will show a demyelinating type of neuropathy. This may, however, be normal in early stages.
Cerebral spinal fluid, or CSF examination, usually shows increased protein level after first week of illness. CSF white cell count is, however, normal. This pattern is called cytoalbuminic dissociation. MR imaging of the lumbar [INAUDIBLE] spine may show contrast enhancement of [INAUDIBLE] roots. However, this is not routinely performed for diagnostic purposes. For acute disease modifying treatment, intravenous immunoglobulin, [INAUDIBLE] s IV Ig, and plasma [INAUDIBLE] are equally effective. IV Ig is the preferred treatment, due to ease of administration and good safety record.
It should be given at a total dose of two grams per kilogram body weight, equally divided in five daily doses. Combination of IV Ig and plasma exchanges has not been shown to confer additional benefit. Occasional patients with [INAUDIBLE], and those who seem to have reached the plateau of weakness, may be managed conservatively without any treatment.
Steroids are not useful in the treatment of GBS. Respiratory weakness is an important cause of mortality in GBS. Vital capacity should be screened and monitored for respiratory involvement. PO2 is not a reliable indicator for respiratory involvement, and PCO2 should be monitored on arterial blood gases. Patients with impending respiratory failure, as suggested by clinical symptoms and signs, drop in vital capacity, or rise in PCO2 should be intubated and mechanically ventilated. [INAUDIBLE] may be compromised, and if so, tube feeding is indicated.
Cut out monitoring for autonomic dysfunction, such as [INAUDIBLE] and the [INAUDIBLE], and BP fluctuations should be performed. Thromboembolic prophylaxis with low molecular weight heparin and compression devices should be considered for immobile patients. Neuropathic pain can be treated with medications such as gabapantene gabamezapene, and [INAUDIBLE]. Depression can occur and should be identified and treated. Bowel function should be regulated with laxatives if needed. Physiotherapy is an important and useful component of the supportive treatment of GBS. Mortality in GBS is about 5%.
At one year, 15% of GBS patients are unable to walk unaided. Poor outcome is associated with older age, preceding diarrheal illness, severe and rapid deterioration of the illness, electrically inexcitable nerves, and muscle wasting. A small proportion of GBS patients continue to progress or relapse. They are then classified to have chronic inflammatory, demyelinating [INAUDIBLE] neuropathy, or CIDP. To summarize, GBS is the most common acute to sub-acute neuropathy.
It is due to autoimmune mediated damage to myelinated nerves. It presents as acute areflecix paralysis. The spectrum extends from more often, unrecognized forms, to severe GBS with respiration failure. Acute treatment is with intravenous immunoglobulin or plasmapheresis, and outcome is good for most patients.
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