2 Guillain Barre Syndrome (GBS) is a serious disorder that occurs when the body’s defense (immune) system mistakenly attacks part of the nervous system. This leads to nerve inflammation that causes muscle weakness.There are 6 different subtypes of GBS and they are:Acute Inflammatory demyelinating polyneuropathyMiller Fisher syndromeAcute motor axonal neuropathyAcute motor sensory axonal neuropathyAcute panautonomic neuropathyBickerstaff’s brainstem encephalitis
3 PathophysiologyGuillain Barre Syndrome (GBS) is a rare and severe disease. It occurs after an acute infectious procedure. GBS initially affects the peripheral nervous system. Normally it is acute form of paralysis in lower body area that moves towards upper limb and face. Gradually patient loses all his/her reflexes and goes through a complete body paralysis. GBS is a life threatening disorder and needs timely treatment and supportive care with intravenous immunoglobulins or plasmapheresis.
4 Pathophysiology cont… GBS is considered to be an autoimmune disease triggered by a preceding bacterial or viral infection. Campylobacter jejuni, cytomegalovirus, Epstein-Barr virus and Mycoplasma pneumoniae are commonly identified antecedent pathogens. They have specific antigens in their capsule that they share with nerves. The immune system usually then response to these components in the capsule by producing antibodies that cross-react wit the myelin in the peripheral nervous system, causing demyelination and then damage to the peripheral nervous system. In GBS there is infiltration of the spinal roots and peripheral nerves via the lymphatic system, causing stripping of the myelin. This will lead to a defect in the transmission of electrical nerve impulses which will then lead to flaccid paralysis.
5 CausesNobody knows precisely what the exact cause is. We know it is due to an immune response to foreign antigens, such as infectious agents (bacteria, etc.) that the body's immune system mistargets - attacks good tissue by mistake. Experts believe our immune system mistakenly attacks gangliosides - compounds which are naturally present in nerve tissues.The most common infection which precedes the development of Guillain-Barre syndrome is Campylobacter jejuni - one of the most widespread causes of human gastroenteritis. Even so, in over half of all cases no previous infection was present; in other words, there was nothing to which the doctor could link the syndrome.It is believed the influenza virus may also trigger an autoimmune response which causes the syndrome.Experts believe the foreign agent (bacterium/virus) causes the body's immune system to attack the myelin sheath of the peripheral nerves. The myelin sheath is a cover that protects the nerve, a bit like the plastic that covers electrical wires. The sheath becomes damaged, causing nerve damage, resulting in faulty sending of signals between nerves and muscles. This faulty wiring causes muscle weakness, numbness and tingling, and eventually paralysis.
6 Risk factors Age (15-35) and (60-75) In men more likely than woman Recent gastrointerstinal or respiratory infection by viruses or bacteriaRecent vaccination (especially influenza and meningococcal)Recent surgeryHistory of lymphoma, Systemic lupus erythromatosus, or HIV and AIDS
7 Signs and Symptoms Muscle weakness Numbness Loss of reflexes in arms and legsLow blood pressureUncoordinated movementFacial weaknessClumsiness and fallingSevere pain in the lower backSensation changesTenderness or muscle painBlurred visionRespiratory problems
8 Medical managementPatients with Guillain-Barre syndrome will be hospitalized initially (medical emergency). It is important to monitor the individual's respiration carefully. If breathing problems are severe he/she may be placed in an ICU (intensive care unit) and put on a ventilator.According to the National Health Service, the two main initial treatment options for Guillain-Barre syndrome are intravenous immunoglobulin, which is safer and easier to give, or plasmapheres (plasma exchange).
9 Medical managementIntravenous immunoglobulin - concentrated antibodies is injected straight into a vein. The antibodies are extracted from healthy donors.Plasmapheresis - the aim here is to deplete the body of blood plasma without depleting it of its blood cells. Plasma is the liquid part of blood. In the case of Guillain-Barre syndrome, the aim is to remove the cells that are attacking the nerves.
10 After the acute phase of the syndrome, the patient may need rehabilitation to regain functions that were lost. Treatment concentrates on improving activities for daily living, such as brushing teeth, washing, getting dressed and performing some other everyday tasks.
11 Physiotherapeutic problems AcuteRespiratory failure due to paralysis of diaphram and secretionsDecreased A/E due to paralysis of diaphram and secretionsDecreased chest expansion due to decreased A/EIncreased secretions due to pneumonia, poor cough attempt and weakness of respiratory musclesPost acuteDecreased Fx w.r.t. ADL due to muscle weaknessPoor proximal and distal control due to weaknessDecreased Fx due to immobility
12 Physiotherapeutic problems cont… Associated problemsMuscle atrophy due to weakness and inactivityPoor sensation due to nerve damageContractures due to immobilityDecreased exercise tolerance due to inactivityDecreased circulation due to inactivitySecondary lung infection due to secretionsPressure sores and DVT due to immobilityWeakness due to inactivity
13 Short term Aims Improve/maintain respiratory function Prevention of pressure sores and DVTPrevention of muscle atrophyImprove/maintain muscle strengthImprove/maintain function
14 Long term Aims Maintain exercise tolerance Maintain function Wheelchair handlingSupport groupsRetrain proximal controlMaintain muscle strengthRetraining of gait
15 Evidence-based article Recognising and managing guillain barre syndromeJason Lugg (emergency care practitioner) explores the difficulties of recognising the viral-like symptoms of this rare condition and offers diagnostic advice to emergency practitioners.
16 ReferencesHughes RA, Wijdicks EF, Baohn R, et al. Practice parameter: immunotherapy for Guillain-Barre syndrome: Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology ;61(6): [PubMed: ].Jason L. Recognising and managing Guillain-Barre Syndrome. Emergency nursing: June 2010; 18(3): 27-30Paediatric PowerPoint's presentations (2012), Mrs R. Smith