Guillain-Barré Syndrome

Chapter 63 Guillain-Barré Syndrome



Ala Nozari, MD, PhD , Corey R. Fehnel, MD , Lee H. Schwamm, MD, FAHA




1 What is Guillain-Barré syndrome (GBS)?


Also known as acute polyradiculoneuritis, GBS is an acute onset of peripheral neuropathy with progressive muscle weakness and areflexia that reaches maximum over 3 days to 3 weeks. It has a number of variants, including pure motor or motor-sensory variants, Miller Fisher variant, bulbar variant, and primary axonal GBS.



2 Describe the pathophysiology of GBS


GBS is a collection of syndromes with inflammatory demyelinating polyradiculoneuropathy. It is triggered by both humoral and cell-mediated autoimmune response to an immune sensitizing event such as an upper respiratory tract infection or cytomegalovirus, herpes simplex virus, Campylobacter jejuni, or mycoplasma infections. A clear antecedent infection is often difficult to identify. Antibodies to gangliosides and glycolipids trigger myelin disruption in the peripheral nervous system. Axonal antibodies occur in some cases, typically after campylobacter infection, and carry a worse prognosis for complete recovery. An increased incidence has been reported in patients with lymphoma or lupus. In the United States, it is a sporadic disease, but variants in Asia are often epidemic in the summer months, presumably because of increased human exposure to zoonotic infections from domesticated livestock.



3 What is the typical clinical presentation of GBS?


GBS is an ascending paralysis that is typically characterized by symmetric weakness, sensory dysesthesias, and hyporeflexia. Mild cases may present with only mild weakness or as variants (e.g., ataxia, ophthalmoplegia, and hyporeflexia) without significant appendicular weakness. Fulminant cases may cause severe ascending weakness leading to complete tetraplegia and with paralysis of cranial nerves and respiratory muscles (involvement of the phrenic and intercostal nerves).



4 Describe the diagnostic criteria for GBS


Clinical features required for the diagnosis of GBS include areflexia and progressive motor weakness of more than one limb. Features strongly supportive of the diagnosis include progression of the motor weakness, relative symmetry, cranial nerve involvement, mild sensory symptoms, autonomic dysfunction, and recovery within 2 to 4 weeks after progression stops.



5 What is the differential diagnosis of subacutely evolving, generalized motor weakness?


Disorders frequently mimicking GBS include acute intermittent porphyria, transverse myelitis, tick paralysis, myasthenia gravis, hypophosphatemia, and carcinomatous or lymphomatous meningitis. Severe peripheral neuropathies due to vasculitis or toxins such as lead, nitrofurantoin, dapsone, thallium, arsenic, and many others should also be considered.



6 Describe laboratory and radiologic findings for GBS


Cerebrospinal fluid (CSF) analysis shows elevated protein level without pleocytosis (albuminocytologic dissociation). Electromyogram and nerve conduction study results may be normal in the early acute period but after 1 to 2 weeks reveal characteristic segmental demyelination and reduction of conduction velocity. Dispersion or absence of F waves is an important early finding that is indicative of root demyelination. Although magnetic resonance imaging provides no characteristic findings, diffuse enhancement of cauda equina and nerve roots may occur as a result of disruption of the blood-nerve barrier caused by the inflammation. Conspicuous nerve root enhancement correlates with pain, disability grade, and duration of recovery.



7 Describe the initial management of GBS in patients


Patients with GBS are often admitted to an intensive care unit for close observation, as some may have respiratory failure, autonomic instability, or hypotension during plasmapheresis. Patients with early cranial nerve dysfunction are more susceptible to aspiration and dysautonomia. Early tracheostomy should be considered in patients with severe weakness, particularly if it involves the bulbar musculature. Radicular thoracolumbar pain is often prominent and may benefit from treatment with neuropathic pain agents such as gabapentin. Aggressive skin care, physical therapy, and splinting are important to prevent skin breakdown and contractures. Low-molecular-weight heparin and graduated pressure stockings should be used to prevent deep vein thrombosis.

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Jul 6, 2016 | Posted by in CRITICAL CARE | Comments Off on Guillain-Barré Syndrome

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