Heltty,M.Kep.,Ns.Sp.

MB

Is an acute, rapidly progressing, and potentially fatal from of polyneuritis It afffects the peripheral nervous system and result in loss of myelin, edema and inflammation of the affected nerves, causing a loss of neorotransmission to the periphery With adequate supportive care, 85% of the patients recover completely from this disorder

Etiology
Unknown Believed to be a cell-mediated immunologic reaction directed at the peripheral nerves The syndrome is frequently preceded by immune system stimulation from a viral infection, trauma surgery, viral immunizations, human immunodeficiency virus (HIV) or lymphoproliferative neoplasms

The stimuli are thougth to cause an alteration in the immune system, resulting in sensitization of T lymphocytes to the patients myelin, causing myelin damage Demyelination occurs, and the transmission of nerve impulses is stopped or slowed down The muscles innervated by the damaged peripheral nerves undergo denervation and atrophy

In the recovery phase, remyelination occurs slowly and returns in a proximal to distal pattern. The lymphocytes are basically normal and return to complete functioning after the illness

The syndrome affects both genders equally and is more commonly seen in adults, althougth it is observed in all age groups Worldwide the incidence has varied from 0,4 to 1,7 cases per 100.000 persons per year

Clinnical Manifestations
Symptoms of GBS usually develop 1 to 3 weeks after an upper respiratory or GI infection Weakness of the lower extremities (evolving more or less symmetrically) occurs over hours to days to weeks, usually peaking about the fourteenth day Distal muscles are more severely affected Paresthesia (numbness and tingling) is frequent, and paralysis usually follows in the extremities.

Hypotonia and areflexia are common, persistent symptoms Objective sensory loss is variable, with deep sensitivity more affected than superficial sensations Autonomic nervous system dysfunction results from alterations in both the symphatetic and parasymphatetic nervous systems

Autonomic disturbances are usually seen in patients with severe muscle involvement and respiratory muscle paralysis The most dangerous autonomic dysfunctions include orthostatic hypotension, hypertension, and abnormal vagal responses (bradycardia, heart block, asystole)

Other autonomic dysfunctions include bowel and bladder dysfunctions, facial flushing, and diapohoresis Patients may also have syndrome of inappropriate antidiuretic hormon (SIADH) secretion. Progression of GBS to include the lower brainstem involves facial, abducens, oculomotor, hypoglossal, trigeminal, and vagus nerves (CN VII, VI, III, XII, V and X, respectively)

This involvement manifests itself through facial weakness, extraocular eye movement difficulties, dysphagia, and paresthesia of the face Pain is a common symptom in the patient with GBS The pain can be catagorized as paresthesias, muscular aches and cramps, and hyperesthesias Pain appears to be worse at night Narcotics may be indicated for those experiencing severe pain Pain may lead to a decreased in appetite and may interfere with sleep

Complications

The most serious complications : respiratory failure, which occurs as the paralysis progresses to the nerves that innervate the thoracic area Respiratory or urinary tract infections (UTI) may occur Fever is generally the first sign of infection, and treatment is directed at the infecting organism Immobility from the paralysis can cause problems such as paralytic ileus, muscle atrophy, deep vein thrombosis, pulmonary emboli, skin breakdown, orthostatic hypotension, and nutritional deficiencies

Diagnostic Studies
Diagnosis is based primarily on the patient;s history and clinical signs Cerebrospinal fluid (CSF) is normal or has a low protein content initially, but after 7 to 10 days it shows an elevated protein level to 700 mg/dl (7 gr/L) (normal protein is 15 to 45 mg/dl ; 0,15 to 0,45 g/L) with a normal cell count Electromyographic (EMG) and nerve conduction studies are markedly abnormal (reduced nerve conduction velocity) in the affected extremities

Collaborative Care
Management is aimedat supportive care, particulary ventilatory support, during the acute phase Plasmapheresis is used in the first 2 weeks of GBS In patients with severe disease treathed wihin 2 weeks of onset, there is a distinct reduction in the length of stay, length of time on ventilator, and some required to resume walking

Cont..
After 3 weeks of onset little value has been seen in plasmapheresis IV administration of high-dose immunoglobulin has also show n to be as effective as plasmapheresis and has the advantage of immediate availability and greater safety Because of the ease of administering immunoglobulin, it is now being used more frequently than plasmapheresis The cost of the two treatments are comparable

Cont..
Recovery is accelerated by early institution of plasmapheresis and Iv therapy Corticosteroid and ACTH are used to suppress the immune response but appear to have little effect on the prognosis or duration of the disease

Nutritional Therapy
Nutritional intake is compromised in the patient with GBS During the acute phase, the patient may experience difficulty swallowing because of cranial nerve involment Mild dysphagia can be managed by placing the patient in an upright position and flexing the head forward during feeding

Cont..
For more severe dysphagia, tube feedings may be required Patient who experience paralytic ileus or intestinal obstruction may require total parenteral nutrition Later in the course of the disease, motor paralysis or weakness continue to affect the ability to selffeed The patients nutritional satus, including body weight, serum albumin levels, and calorie counts, must be evaluated at regular intervals

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