(Acute idiopathic polyneuritis, acute inflammatory demyelinating polyradiculoneuropathy, Guillain-Barre’s syndrome)
Guillain-Barre syndrome is an acute, usually very rapidly progressive, however self-limiting inflammatory polyneuropathy and characterized by muscle weakness and mild distal sensory loss. As a cause of an autoimmune process is adopted. The diagnosis is made clinically. The treatment includes iv immunoglobulins, plasma exchange and, in severe cases, one of apparatus ventilation.
(See also summary of disorders of the peripheral nervous system.)
Guillain-Barre syndrome is an acute, usually very rapidly progressive, however self-limiting inflammatory polyneuropathy and characterized by muscle weakness and mild distal sensory loss. As a cause of an autoimmune process is adopted. The diagnosis is made clinically. The treatment includes iv immunoglobulins, plasma exchange and, in severe cases, one of apparatus ventilation. (See also overview of disorders of the peripheral nervous system.) The Guillain-Barre syndrome is the most common acquired inflammatory neuropathy. There are several variants. If the cause is not fully understood, it is nevertheless probably autoimmune-related. In some variations, the demyelination dominated, in other variants of the axon is affected. About two thirds of patients Guillain-Barre syndrome begins 5 days to 3 weeks after a banal infection, surgery or vaccination. Infection is 50% of the patients the trigger at>. Common pathogens are Campylobacter jejuni, Entereroviren, herpes viruses (incl. Cytomegalovirus and Epstein-Barr virus) and Mycoplasma spp. A cluster of cases followed the swine flu vaccination program in 1976, but the relationship turned out to be due to a bias in the survey to be wrong. Symptoms and signs A flaccid paralysis dominates in most patients; it is the sensory disturbances against always on top and may be proximal most pronounced. A relatively symmetrical weakness with paresthesias usually begins in the legs and spreads to the arms, but it can sometimes begin in the arms or the head. In 90% of patients the weakness reaches its maximum in the 3rd to 4th week of illness in general. The tendon reflexes fail. The sphincters are spared in general. Weakness remains the same for a variable period of time, usually for about a week and then goes back. The facial and oropharyngeal muscles are weak at> 50% of patients with severe cases. This dehydration and hyperosmolarity may result. Respiratory paralysis, which is so heavy that endotracheal intubation and mechanical ventilation are required, occurs in 5-10% of cases. Few patients (possibly with a variant of the disease) have significant, life-threatening autonomic dysfunction, blood pressure fluctuations, inappropriate antidiuretic hormone secretion, cardiac arrhythmias, gastrointestinal paralysis, urinary retention and pupil changes cause. An unusual variant (Fisher variant or Miller Fisher syndrome) can cause a combination of only Ophthalmoparese, ataxia and areflexia. Diagnosis Clinical assessment Electro-diagnostic testing CSF analysis The diagnosis of Guillain-Barre syndrome is made primarily clinically. Differential Diagnosis A similar acute weakness can be caused by myasthenia gravis, botulism, poliomyelitis (especially outside the US), tick paralysis, West Nile virus infection and metabolic neuropathy, but these faults of Guillain-Barre syndrome usually distinguished as follows be: myasthenia gravis is intermittent and is aggravated by physical exertion. Botulism can cause rigid dilated pupils (in 50% of cases) as well as prominent cranial nerve disorders in normal sensors. Poliomyelitis is commonly found epidemic. Tick ??paralysis caused ascending paralysis, but does not affect the sensor. West Nile virus infection causes headache, fever and asymmetric flaccid paralysis, but does not affect the sensor. Metabolic neuropathies occur in the context of chronic metabolic disorders. Testing will be carried out tests on infectious diseases and immune disorders, incl. Tests for hepatitis and HIV, and serum protein electrophoresis. In V. a. Guillain-Barre syndrome should be hospitalized every 6-8 h patients for electrochemical diagnostic testing (testing of nerve conduction and electromyography) to cerebrospinal fluid, and monitoring of the repeated measurement of the forced vital capacity. A survey conducted at the beginning of electrodiagnostic testing reveals slow nerve conduction velocities and signs of segmental demyelination in two thirds of patients; but normal findings do not exclude the diagnosis and should not delay treatment. In the cerebrospinal fluid zytoalbuminäre a dissociation can be detected (increased protein in normal leukocyte); However, this can sometimes only after to show to a week, and in 10% of patients they do not develop. Rarely cervical Rückenmarkkompression- particularly when coexisted polyneuropathy (to hyporeflexia contributing or causing these) and bulbar involvement is not prominent-can mimic Guillain-Barre syndrome; In such cases, MRI should be performed. Forecast In <2% of patients runs Guillain-Barré Syndrome fatal. Most patients show a significant improvement over a period of months, but about 30% of adults and more children after 3 years still have a residual paralysis. Patients with Residualdefekten may need exercise treatments, orthopedic aids or surgery. After initial improvement 3-10% of patients develop chronic inflammatory demyelinating polyneuropathy (CIDP). Medical treatment intensive supportive treatment i.v. immunoglobulins (IVIG), or plasma exchange, the Guillain-Barre syndrome is a medical emergency. It requires constant monitoring and support of vital functions, typically in an ICU. The forced vital capacity should be measured frequently, so that the breathing may be necessary, supported. If the vital capacity is <15 ml / kg, endotracheal intubation is indicated. The inability to lift the head by neck flexion from the pillow, is another danger sign; it often develops simultaneously with a weakness of the phrenic nerve (diaphragmatic paralysis). In case of difficulty with the oral fluid intake i.v. liquids are given as required to maintain a urine volume of at least 1-1.5 l / day. The limb should be protected from injuries and storage damage in bedridden. Heat treatment helps to relieve pain and makes an early physical therapy possible. Immobilization should be avoided as they can lead to ankylosis and contractures. A passive By moving all the joints in the entire range of motion should be started immediately, and an active exercise treatment should follow as soon as possible in regression of acute symptoms. 5000 I.U. Heparin s.c. 2 times / day help prevent deep vein thrombosis in bedridden patients. Early doses of IVIG 400 mg / kg i.v. once daily for 5 consecutive days is the treatment of choice; it has up to 1 month from the onset some benefit. A plasma exchange helps if it occurs early; it is used in invalidity of IVIG. Plasma exchange is relatively safe, it shortens the course of disease and duration of hospital stay, reduces the risk of mortality and reduces the incidence of permanent paralysis. In plasma exchange all IVIG previously administered be removed and thus made their benefits to naught, and therefore it should never be done during or shortly after the use of IVIG. It is recommended to wait until at least 2-3 days after discontinuation of IVIG. Tips and risks Do not give corticosteroids in Guillain-Barre syndrome, because these can worsen the outcome. Corticosteroids do not contribute to improve and may worsen the outcome. Conclusion The Guillain-Barre syndrome typically begins with an ascending, relatively symmetrical flaccid weakness. initially on the basis of medical history and examination results from other diseases that cause similar symptoms Narrow (eg, myasthenia gravis, botulism, tick paralysis, West Nile virus infection, metabolic neuropathies; outside the US. poliomyelitis). Perform electrodiagnostic testing and CSF analysis, although the diagnosis in the first place is clinical. About 70% of patients recover completely, but 3-10% develop chronic inflammatory demyelinating Polyneuropathy. Intensive supportive care is the key to recovery. First try IVIG and then, they should be invalid, a plasma exchange.