Guillain–Barré syndrome
[3] During the acute phase, the disorder can be life-threatening, with about 15% of people developing weakness of the breathing muscles requiring mechanical ventilation.
[3] Although the cause is unknown, the underlying mechanism involves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation.
[1][3] The diagnosis is usually based on the signs and symptoms through the exclusion of alternative causes and supported by tests such as nerve conduction studies and examination of the cerebrospinal fluid.
[1] In those with severe weakness, prompt treatment with intravenous immunoglobulins or plasmapheresis, together with supportive care, will lead to good recovery in the majority of cases.
[3][4] The illness that afflicted US president Franklin D. Roosevelt, and left him paralysed from the waist down, which was believed at the time to be polio, may have been Guillain–Barré syndrome, according to more recent research.
[10] In children, particularly those younger than six years old, the diagnosis can be difficult and the condition is often initially mistaken (sometimes for up to two weeks) for other causes of pains and difficulty walking, such as viral infections,[5] or bone and joint problems.
[5] In the Miller Fisher variant of Guillain–Barré syndrome (see below), a triad of weakness of the eye muscles, abnormalities in coordination, as well as absent reflexes can be found.
[10] The level of consciousness is normally unaffected in Guillain–Barré syndrome, but the Bickerstaff brainstem encephalitis subtype may feature drowsiness, sleepiness, or coma.
[5][10][13] This life-threatening scenario is complicated by other medical problems such as pneumonia, severe infections, blood clots in the lungs, and bleeding in the digestive tract in 60% of those who require artificial ventilation.
[10] The autonomic or involuntary nervous system, which is involved in the control of body functions such as heart rate and blood pressure, is affected in two-thirds of people with Guillain–Barré syndrome, but the impact is variable.
[5][8] An increased incidence of Guillain–Barré syndrome followed influenza immunization that followed the 1976 swine flu outbreak (H1N1 A/NJ/76); 8.8 cases per million (0.0088 per 1000) recipients developed it as a complication.
[18] The 1976 swine flu vaccination-induced GBS was an outlier; small increases in incidence have been observed in subsequent vaccination campaigns, but not to the same extent.
[22][23][24][25] GBS has been reported as a very rare side effect of the Janssen and Oxford–AstraZeneca COVID-19 vaccines[26] and the European Medicines Agency issued a warning to the patients and healthcare providers.
[28] More recent studies, however, found no measurable link between COVID-19 infection and GBS, while correlations with a first dose of AstraZeneca or Janssen vaccines were still positive.
Cerebrospinal fluid analysis (through a lumbar spinal puncture) and nerve conduction studies are supportive investigations commonly performed in the diagnosis of GBS.
Characteristic findings in Guillain–Barré syndrome are an elevated protein level, usually greater than 0.55 g/L, and fewer than 10 white blood cells per cubic millimeter of fluid ("albuminocytological dissociation").
[5] Directly assessing nerve conduction of electrical impulses can exclude other causes of acute muscle weakness, as well as distinguish the different types of Guillain–Barré syndrome.
For instance, some people experience only isolated eye movement or coordination problems; these are thought to be a subtype of Miller-Fisher syndrome and have similar antiganglioside antibody patterns.
[41] BBE is characterized by the rapid onset of ophthalmoplegia, ataxia, and disturbance of consciousness, and may be associated with absent or decreased tendon reflexes and as well as Babinski's sign.
[46] IVIG is usually used first because of its ease of administration and safety; the risks include occasionally causing liver inflammation, or in rare cases, kidney failure.
[48] Respiratory failure may require intubation of the trachea and breathing support through mechanical ventilation, generally on an intensive care unit.
[50] Following the acute phase, around 40% of people require intensive rehabilitation with the help of a multidisciplinary team to focus on improving activities of daily living (ADLs).
[51] Physiotherapy interventions include strength, endurance, and gait training with graduated increases in mobility, maintenance of posture and alignment as well as joint function.
[51] Ongoing specialist community support, information, advice, and guidance is available from a range of Charities, Non-Government Organisations (NGOs), and Patient Advisory Groups around the world.
[55] Guillain–Barré syndrome can lead to death as a result of many complications: severe infections, blood clots, and cardiac arrest likely due to autonomic neuropathy.
About a fifth are unable to walk unaided after six months, and many experience chronic pain, fatigue, and difficulty with work, education, hobbies, and social activities.
[59] In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality—albuminocytological dissociation—of increased spinal fluid protein concentration but a normal cell count.
[39][67] Current research is aimed at demonstrating whether some people who have received IVIg might benefit from a second course if the antibody levels measured in blood after treatment have shown only a small increase.
[13][67] Studies of the immunosuppressive drugs mycophenolate mofetil, brain-derived neurotrophic factor and interferon beta (IFN-β) have not demonstrated benefit to support their widespread use.
[67] An animal model (experimental autoimmune neuritis in rats) is often used for studies, and some agents have shown promise: glatiramer acetate, quinupramine, fasudil (an inhibitor of the Rho-kinase enzyme),[39] and the heart drug flecainide.
