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Guillain-Barré syndrome is a rare autoimmune disorder that affects the peripheral nervous system, causing the immune system to damage nerve cells and their connections. It is usually preceded by a viral or bacterial infection 1-4 weeks prior. The most common types are acute inflammatory demyelinating polyneuropathy which damages the myelin sheath of nerves, and acute motor axonal neuropathy which damages motor neuron axons. Treatment involves plasma exchange or intravenous immunoglobulin to stop the immune response. Supportive care focuses on managing respiratory function, autonomic dysfunction, and preventing complications.
Guillain-Barré syndrome is a rare autoimmune disorder that affects the peripheral nervous system, causing the immune system to damage nerve cells and their connections. It is usually preceded by a viral or bacterial infection 1-4 weeks prior. The most common types are acute inflammatory demyelinating polyneuropathy which damages the myelin sheath of nerves, and acute motor axonal neuropathy which damages motor neuron axons. Treatment involves plasma exchange or intravenous immunoglobulin to stop the immune response. Supportive care focuses on managing respiratory function, autonomic dysfunction, and preventing complications.
Guillain-Barré syndrome is a rare autoimmune disorder that affects the peripheral nervous system, causing the immune system to damage nerve cells and their connections. It is usually preceded by a viral or bacterial infection 1-4 weeks prior. The most common types are acute inflammatory demyelinating polyneuropathy which damages the myelin sheath of nerves, and acute motor axonal neuropathy which damages motor neuron axons. Treatment involves plasma exchange or intravenous immunoglobulin to stop the immune response. Supportive care focuses on managing respiratory function, autonomic dysfunction, and preventing complications.
a rare and serious condition of the peripheral nervous
system. It occurs when the body's immune system attacks part of the nervous system. ETIOLOGY • GBS is common to all races and ages; mild increase in frequency in patients between ages 30-50; GBS is less common in infants or the elderly. • GBS has a yearly incidence of 0.6-1.9 cases/100,000 population. • Prior infection is well established as a precipitating event in the development of GBS. GBS preceded by an acute illness, 1-4 weeks before, in about 75% of cases. ETIOLOGY • GBS may rarely develop within a day or two, or after 4-6 weeks, of an acute illness. • Most antecedent illnesses associated with GBS affect the upper respiratory or GI tracts. • Cytomegalovirus (CMV) is the most common viral antecedent infection with serologic evidence in up to 15% of cases PATHOGENESIS • Guillain-Barré syndrome is an autoimmune disorder that affects the nerves. • In Guillain-Barré syndrome, the immune response damages peripheral nerves, which are the nerves that connect the central nervous system (the brain and spinal cord) to the limbs and organs. PATHOGENESIS • Specifically, the immune response affects a particular part of peripheral nerves called axons, which are the extensions of nerve cells (neurons) that transmit nerve impulses. • Guillain-Barré syndrome can affect the neurons that control muscle movement (motor neurons); TYPES Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) • In AIDP, the immune response damages myelin, which is the covering that protects axons and promotes the efficient transmission of nerve impulses. TYPES Acute motor axonal neuropathy (AMAN) • The axons of motor neurons are damaged. Acute motor-sensory axonal neuropathy (AMSAN). • The axons of motor and sensory neurons are also damaged. TYPES • Because of sensory nerve damage, affected individuals can lose the ability to sense the position of their limbs and can have abnormal or absent reflexes (areflexia). TYPES Miller Fisher Syndrome • Involves cranial nerves, which extend from the brain to various areas of the head and neck. Miller Fisher syndrome is characterized by three features: weakness or paralysis of the muscles that move the eyes (ophthalmoplegia), problems with balance and coordination (ataxia), and areflexia. DIFFERENTIAL DIAGNOSIS • History taking • Standard blood tests • Cerebrospinal fluid examination • Electromyography – may be helpful to establish the diagnosis. NEUROLOGIC EXAMINATION • Facial weakness (cranial nerve VII) is observed most frequently, followed by symptoms associated with cranial nerves VI, III, XII, V, IX, and X. • Upper extremity, trunk, facial, and oropharyngeal weakness is observed to a variable extent. • Reflexes are absent or reduced early in the disease course. TREATMENT Plasma Exchange (PE) or Plasmapheresis • a process in which some of the patient's blood is removed, the liquid part separated, and the blood cells returned to the body, has been used for severe cases. TREATMENT Intravenous Immunoglobulin (IVIg) • healthy immunoglobulin is taken from blood donors and given to intravenously (directly into a vein). The healthy antibodies block and destroy the harmful antibodies that are attacking the nerves. IVIg is given usually every day for five days. Each infusion takes about two hours. MANAGEMENT Supportive Care: ICU monitoring & basic medical management Maintaining Respiratory Function: • Monitoring for changes in vital capacity and negative inspiratory force • Mechanical ventilation is required if the vital capacity falls, making spontaneous breathing impossible and tissue oxygenation inadequate. • Suctioning may be needed to maintain a clear airway. MANAGEMENT For autonomic dysfunction: • Assessment of BP and HR frequently. • Sustained hypertension managed by ACE inhibitor or beta blocking agent. • Postural hypotension treated with fluid bolus or positioning. • Urinary difficulties may require intermittent catheterization. MANAGEMENT For nosocomial infections: • Antibiotic therapy should be reserved.
For Deep Vein Thrombosis:
• Range-of-motion exercises, position changes, anticoagulation, the use of anti- embolism stockings or sequential compression boots, and adequate hydration decrease the risk of DVT. MANAGEMENT Nutritional Support: • Administer IV fluids and parenteral nutrition as a supplement and monitor for the return of bowel sounds. • Nasogastric tube needed in patients who are intubated or have significant oropharyngeal weakness.