GuillainBarr Syndrome

GuillainBarr syndrome (GBS) sometimes Landry's paralysis or GuillainBarrStrohl syndrome, is an acute polyneuropathy, a disorder affecting the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system. It can cause life-threatening complications, in particular if the respiratory muscles are affected or if there is autonomic nervous system involvement. The disease is usually triggered by an infection.

Signs and Symptoms

The disorder is characterized by symmetrical weakness that usually affects the lower limbs first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without dysesthesias (numbness or tingling). As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, oropharyngeal dysphagia (drooling, or difficulty swallowing and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance for treatment of Type II respiratory failure.[5] Facial weakness is also common. Eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant. Sensory loss, if present, usually takes the form of loss of proprioception (position sense) and areflexia (complete loss of deep tendon reflexes), an important feature of GBS. Loss of pain and temperature sensation is usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain, usually in the weakened muscles, which patients compare to the pain from overexercising. These pains are self-limited and may be treated with standard analgesics. Bladder dysfunction may occur in severe cases but is usually transient. In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension (a fall in blood pressure on standing, leading to an increased risk of collapse), and cardiac arrhythmias.

Acute paralysis in GuillainBarr syndrome may be related to sodium channel blocking factor in the cerebrospinal fluid (CSF). Significant issues involving intravenous salt and water administration may occur unpredictably in this patient group, resulting in SIADH, a cause of low sodium levels in the blood.

Cause
All forms of GuillainBarr syndrome are autoimmune diseases, due to an immune response to foreign antigens (such as infectious agents) that is mistargeted at host nerve tissues instead, a phenomenon called molecular mimicry.[6] The targets of such immune attack are thought to be gangliosides, compounds naturally present in large quantities in human peripheral nerve tissues. The most common antecedent infection is the bacterium Campylobacter jejuni, followed by cytomegalovirus (CMV).However, 60% of cases do not have a known cause. Some cases may be triggered by the influenza virus, or by an immune reaction to the influenza virus. There was increased incidence of GuillainBarr syndrome following influenza immunization during the 1976-1977 swine flu pandemic; however, epidemiological studies since then have demonstrated either an extremely small increased risk following immunization (under 1 additional case per million vaccinations) or no increased risk. The end result of this autoimmune attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to muscle paralysis that may be accompanied by sensory or autonomic disturbances.

Diagnosis
The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS. >Testing In cerebrospinal fluid, characteristic findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100 1000 mg/dL), without an accompanying increased cell count (absence of pleocytosis). A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

Electromyography (EMG) and nerve conduction studies (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. F waves and Hreflexes may be prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced, neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle EMG if there is some axonal injury after 3 to 4 weeks. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing. >Criteria Features required for diagnosis are progressive weakness in legs and often arms and areflexia (the absence of deep tendon reflexes). Features that strongly support diagnosis are progression of symptoms over days to 4 weeks, relative symmetry of symptoms, mild sensory symptoms or signs, cranial nerve involvement (especially bilateral weakness of facial muscles), autonomic dysfunction, pain (often present), high concentration of protein in CSF, and typical electrodiagnostic features. Features that should raise doubt about the diagnosis include severe pulmonary dysfunction with limited limb weakness at onset, severe sensory signs with limited weakness at onset, bladder or bowel dysfunction at onset, fever at onset, sharp sensory level, slow progression with limited weakness without respiratory involvement (subacute inflammatory demyelinating polyneuropathy or CIDP is more likely), marked persistent asymmetry of weakness, persistent bladder or bowel dysfunction, increased number of mononuclear cells in CSF (>50106/L), and polymorphonuclear cells in CSF.

Treatment
Supportive care is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle most important for breathing. Intubation may be needed when there is evidence of impending failure of the muscles of breathing when the vital capacity (VC) is less than 20 ml/kg, the negative inspiratory force(NIF) is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability. Subsequent treatment consists of attempting to reduce the body's attack on the nervous system, either by plasmapheresis, filtering antibodies out of the blood stream, or by administeringintravenous immunoglobulins (IVIg), to neutralize harmful antibodies and inflammation causing disease. These two treatments are equally effective and a combination of the two is not significantly better than either alone. Glucocorticoids have not been found to be effective in GBS. Treatment is

usually begun as soon as the diagnosis is made. Plasmapheresis hastens recovery when used within 4 weeks of the onset of symptoms. IVIg has equivalent efficacy to plasmapheresis when started within 2 weeks of the onset of symptoms, and has fewer complications. IVIg is usually used first because of its ease of administration and safety profile. The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Following the acute phase, treatment often consists of rehabilitation with the help of a multidisciplinary team to focus on improving activities of daily living (ADLs). Occupational therapists may offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. Physiotherapists assist to correct functional movement, avoiding harmful compensations that might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with GuillainBarr syndrome regain strength, endurance, and gait quality, as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties. Speech and language therapists help regain speaking and swallowing ability, especially if the patient was intubated or received a tracheostomy.

Nursing Interventions
1. Turn and reposition the patient, and encourage coughing and deep breathing. 2. If respiratory failure becomes imminent, establish an emergency airway with an endotracheal tube. 3. Give meticulous skin care to prevent skin-breakdown and contractures. 4. Perform passive range of motion exercises within the patients pain limits. 5. To prevent aspiration, test the gag reflex and elevate the head of the bed before giving the patient anything to eat. 6. To prevent thrombophlebitis, apply anti-embolism stockings or compression boots and give prophylactic anticoagulants as ordered. 7. If the patient has facial paralysis, give eye and mouth care every 4 hours. 8. Offer bed pan every 3 to 4 hours to monitor intake and output regularly. 9. To prevent or relieve constipation, offer prune juice and high bulk diet. 10.Administer medications as ordered. Analgesics may be prescribed to relieve muscle stiffness and spasm. 11.Provide diversions for the patient, such as televisions, family visits, or listening to the radio. 12.Provide emotional support to the patient and his family.