Name: Cherry Nolle Sanchez

Guillain-Barr Syndrome is 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. 1. GBS may rarely develop within a day or two, or after 4-6 weeks, of an acute illness. 2. Most antecedent illnesses associated with GBS affect the upper respiratory or GI tracts. 3. Cytomegalovirus (CMV) is the most common viral antecedent infection with serologic evidence in up to 15% of cases 4. Epstein Barr (EBV) infection may precede GBS in about 10% of cases; preceding clinical signs include mononucleosis, hepatitis, or pharyngitis. 5. GBS may occur with HIV seroconversion. 6. Camplylobacter jejuni (C. jejuni) is, overall, the most common antecedent infection and has been reported in up to 32% of cases. 7. GBS may possibly occur after surgery, trauma, and in the post-partum period. Pathogenesis: Guillain-Barr syndrome is an autoimmune disorder that affects the nerves. Autoimmune disorders occur when the immune system malfunctions and attacks the body's own tissues and organs. In Guillain-Barr 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. 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-Barr syndrome can affect the neurons that control muscle movement (motor neurons); the neurons that transmit sensory signals such as pain, temperature, and touch (sensory neurons); or both. As a result, affected individuals can experience muscle weakness or lose the ability to feel certain sensations. 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. 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. 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).

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.

Clinical Manifestations: Muscle weakness or paralysis. The weakness often begins in the legs and spreads to the arms, torso, and face and is commonly accompanied by numbness, tingling, or pain. Dysphagia and Dyspnea. Occasionally, the nerves that control involuntary functions of the body such as blood pressure and heart rate are affected, which can lead to fluctuating blood pressure or an abnormal heartbeat (cardiac arrhythmia).

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. 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 Ventilatory Support Nutritional support: via nasogastric tube

WEB LINKS: http://www.ipin.edu.pl/kiz/neurologia2/Ganglio/uk-guillain.pdf http://www.nhs.uk/conditions/guillain-barre-syndrome/Pages/Introduction.aspx http://ghr.nlm.nih.gov/condition/guillain-barre-syndrome http://emedicine.medscape.com/article/315632-clinical#a0256