myasthenia gravis and GBS

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MYASTHENIA GRAVIS & GUILLIAN BARRE SYNDROME:

MYASTHENIA GRAVIS & GUILLIAN BARRE SYNDROME PRESENTED BY: KIRAN RANDHAWA ARMY COLLEGE OF NURSING

INTRODUCTION:

INTRODUCTION MYASTHENIA GRAVIS – M.G. Literally means “GRAVE MUSCLE WEAKNESS” hallmarks of M.G. is muscle weakness that increases during the periods of activity and improves after periods of rest.M.G is purely a motor disorder with no effect on sensation or coordination.

DEFINITION :

DEFINITION M.G. is a chronic autoimmune neuromuscular disorder characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body.

EPIDEMIOLOGY:

EPIDEMIOLOGY Occurs in all ethnic groups Male=Female Sudden onset Commonly affects young adults women(under 40s) &older men (over 60s) Can occur at any age Neither inherited nor contagious Juvenile

ETIOLOGY:

ETIOLOGY Defective neuromuscular transmission. Autoimmune destruction. Thymus gland abnormality. Defective gene that produces protein in Acetylcholine receptor or Acetylcholinestrase { 80%- 90%}

PATHOPHYSIOLOGY :

PATHOPHYSIOLOGY In M.G, autoantibodies directed at the Acetylcholine receptor sites impair transmission of impulses across the myoneural junction Therefore, fewer receptors are available for stimulation

CONTD……….:

CONTD………. Resulting in voluntary muscle weakness that escalates with continued activity That result in development of M.G.

CLINICAL MANIFESTATION:

CLINICAL MANIFESTATION Occular involvement - - diplopia - ptosis Generalized weakness - balbar symptoms

CONTD………:

CONTD……… Laryngeal involvement - dysphonia

DIAGNOSTIC EVALUATION:

DIAGNOSTIC EVALUATION History Physical Examination Neurological Examination

CONTD…….:

CONTD……. CONFIRMATRY DIAGNOSIS - Special blood test ANOTHER TEST - Edrophonium test - Version of nerve conduction - EMG {Electromyography with single fibre}

CONTD…….:

CONTD……. - CT Scan - PFT - MRI

MEDICAL MANAGEMENT:

MEDICAL MANAGEMENT PHARMACOTHERAPY - Anticholinestrase agent { Pyrostigmine} - Immunosuppressive agent { Prednisone, Azathioprine } - Cyclophosphamide & Cyclosporin PLASMAPHARESIS

CONTD……….:

CONTD………. REHABILITATION PROGRAMME: Physical therapy Occupational therapy Speech therapy Recreational therapy

POTENTIAL SIDE EFFECTS OF ANTICHOLINESTRASE AGENTS:

POTENTIAL SIDE EFFECTS OF ANTICHOLINESTRASE AGENTS CNS: Irritability Headache Dysarthria Syncope Anxiety

CONTD………:

CONTD……… Seizures Diaphoresis Coma

CONTD…..:

CONTD….. RESPIRATORY SYSTEM: Bronchial relaxation Increased bronchial secretions. CVS: Tachycardia Hypotension

CONTD…….:

CONTD……. GIT: Abdominal cramps Nausea Vomiting Diarrhea Anorexia Increased salivation

CONTD…….:

CONTD……. MUSCULOSKELETAL SYSTEM: Fasciculations Spasm Weakness

CONTD……:

CONTD…… GENITOURINARY SYSTEM: Frequency Urgency

CONTD…..:

CONTD….. INTEGUMENTARY SYSTEM : Rash Flushing

SURGICAL MANAGEMENT:

SURGICAL MANAGEMENT THYMECTOMY

DRUGS TO BE AVOIDED:

DRUGS TO BE AVOIDED Muscle relaxant Barbiturates Morphine sulphate Tranquilizers Neomycin ( potentiate muscle weakness because it effect on myoneural junction)

COMPLICATIONS:

COMPLICATIONS Myasthenic Crisis Cholinergic Crisis Respiratory Failure

PROGNOSIS:

PROGNOSIS With Rx outcome is bright Significant improvement can expect to lead normal lives

RESEARCH NOTE:

RESEARCH NOTE GOAL: To increase scientific understanding of the disorder. NEW STUDY: Testing the efficacy of IV ADMINISTRATION of IMMUNOGLOBINS in patients with M.G to determine improvement in muscle strength or not.

CONTD…….:

CONTD……. ANOTHER STUDY Regarding basis of synaptic transmission in nervous system.

NURSING MANAGEMENT:

NURSING MANAGEMENT ASSESSMENT: -History -Physical examination -Neurological examination

CONTD…..:

CONTD….. NURSING DIAGNOSIS: Risk for injury to self & others R/T disturbances in vision & drooping of eyelid. GOAL: To prevent from injury. INTERVENTIONS: Instruct the patient not to close eye completely

CONTD….:

CONTD…. Instruct the patient to tape the eyes closed for short intervals & regularly instill artificial tears. Instruct the patient to use crutches if he wear glasses, help to lift the eyelids. Patching one eye can help with double- vision.

CONTD…….:

CONTD……. NURSING DIAGNOSIS : Dysphagia R/T weakness of muscles of face & throat. GOAL: To overcome dysphagia. INTERVENTIONS: Rest before meals is encouraged to reduce muscle fatigue.

CONTD……..:

CONTD…….. Advise the patient to sit upright during meals & neck slightly flexed to facilitate swallowing. Soft foods in gravy or sauces can be given. Do suctioning if needed. provide GASTROSTOMY FEEDINGS if indicated.

CONTD……..:

CONTD…….. NURSING DIAGNOSIS: Easily fatigue R/T generalized weakness of the body. GOAL: To prevent from fatigueness. INTERVENTIONS: Patient is taught to conserve energy. Help the patient to identify the best time for rest period throught the day.

CONTD……..:

CONTD…….. Advise the patient to keep all needed items like hygiene products, cleaning products, snacks on each floor, if he lives in two story house. Encourage the patient to apply for a handicapped license plate.

CONTD……:

CONTD…… NURSING DIAGNOSIS: Knowledge deficit R/T disease, prognosis, Rx regimen. GOAL: To provide knowledge to patient & family members.

CONTD….. :

CONTD….. INTERVENTIONS : Educate the patient & family about medication, action, side effects, prognosis. Teach the patient to keep diary to determine fluctuation of symptoms. Teach the patient not to dismiss the Rx. Make the patient aware that disease can be cure if follow certain measures.

CONTD….:

CONTD…. NURSING DIAGNOSIS : Impaired verbal communication R/T weakness of the larynx ,lips ,mouth ,pharynx & jaw . GOAL : To improve the verbal communication

CONTD……:

CONTD…… INTERVENTIONS: Assess the level of communication . Allow sufficient time for communication to reduce patient’s frustration. Encourage deep breaths before speaking. Consult speech therapist to provide specialized guidance in care of patient .

CONTD…..:

CONTD….. Provide alternative communication methods such as picture books or flash cards . Massage patient’s facial & neck muscles relaxations that can facilitate speech

Slide 41:

GUILLIAN BARRE SYNDROME

INTRODUCTION:

INTRODUCTION GUILLIAN-BARRE SYNDROME [ POLYRADICULONEURITIS ] may be described as a collection of clinical syndromes manifested by an acute inflammatory polyradiculoneuropathy, with resultant weakness & diminished reflexes with poliomyelitis under control in developed countries, GBS is now the most common cause of ACUTE FLACCID PARALYSIS.

DEFINITION:

DEFINITION GBS is an inflammation of peripheral nerves result in acute, rapid segmental demyelination of peripheral nerves & some cranial nerves, producing Ascending weakness with Dyskinesia, Hyporeflexia, Paraesthesias.

EPIDEMIOLOGY:

EPIDEMIOLOGY IN US 3.0 cases/100,000 population annually. Mean rate of hospitalization R/T GBS, increases with advancing age such as 1.5 cases/100,000 population under 15yrs than peak of 8.6 cases/100,000 population up to age 70-79yrs.

CONTD…….:

CONTD……. INTERNATIONALLY GBS is widespread & reported throughout the world. MORTALITY/MORBIDITY Mortality rate –2-12%, increases with age. Death rate 1.3 times > in men than females after age 40s.

CONTD….. :

CONTD….. Length of hospitalization is increases with age. RACE GBS reported throughout international community. SEX Slight male preponderance is seen, especially in older people. AGE Peak age { older}

ETIOLOGY:

ETIOLOGY PATHOLOGICAL FACTORS Campylobacter jejuni most common. CMV{Cytomegalovirus} Others EBV {Epstein-Barr Virus} Mycoplasma Pneumoniae Varicella ZosteerVirus HIV, Haemophillus Influenza,Parainfluenza1 Virus, Herpes Zoster.

PATHOPHYSIOLOGY:

PATHOPHYSIOLOGY Pathophysiologic mechanism of an anticedent illness, GBS can be typified by C.jejuni infection The virulence of C.jejuni thought to be based on the presence of specific Ags in its capsule that are shared with nerves

CONTD……..:

CONTD…….. Immune response directed against the capsular components produce Abs that cross react with myelin to cause demyelination Ganglioside {GM1}appears to cross-react with C.jejuni lipopolysaccharide Ags Resulting in immunologic damage to PNS

CONTD……:

CONTD…… This process termed as MOLECULAR MIMICRY Pathogenic findings in GBS include lymphocytic infiltration of spinal roots & peripheral nerves Followed by macrophage- mediated multifocal stripping of myelin

CONTD ……:

CONTD …… Results in defects in propagation of electrical nerve impulse with eventual conduction block & flaccid paralysis.

CLINICAL MANIFESTATION:

CLINICAL MANIFESTATION GENERAL WEAKNESS ascending & symmetrical in nature, lower limbs usually involve before upper limbs & weakness develops acutely & progressive over days to week SENSORY CHANGES On nerve conduction studies {NCS}, 58%-76% exhibit sensory abnormality.

CONTD…:

CONTD… CRANIAL NERVE INVOLVEMENT {45%-75%} C/O Facial droop Diplopia Dysarthria dysphagia

CONTD…….:

CONTD……. PAIN {89%} Pts C/O pain during illness Mainly at back & legs with uncertain cause Throbbing/ aching in nature AUTONOMIC CHANGES Dysfunction in SNS & PSNS include Tachycardia, Bradycardia Facial flushing, Paroxysmal & Orthostatic HTN Anhidrosis & Diaphoresis

CONTD…….:

CONTD……. Urinary retention & Paralytic ileus Bowel/Bladder dysfunction Dysautonomia with severe weakness & Respiratory failure RESPIRATORY INVOLVEMENT {40%}C/O Dyspnoea on exertion

CONTD……:

CONTD…… Shortness of breath Difficulty in swallowing Slurred sppech Ventilatory failure PHYSICAL CHANGES Vital changes Cranial nerves dysfunction

CONTD…..:

CONTD….. Motor changes Reflex changes { hyporeflexic/absent reflexes }

DIAGNOSTIC EVALUATION:

DIAGNOSTIC EVALUATION LAB STUDIES CSF studies { elevated CSF protein, > 0.5gm/L } Reflect widespread inflammatory disease of nerve roots Basic LAB studies: CBC [ Limited values ] Metabolic panels

CONTD…….:

CONTD……. Basic PERIPHERAL NEUROPATHY RA factor VIT B12, folic acid Hb A1C, ESR Rapid protein reagent Immunoelectrophoresis of SERUM PROTEIN

CONTD……:

CONTD…… Test for HEAVY METALS SEROLOGIC STUDIES: Increase in titers for infectious agent { CMV, EBV, MYCOPLASMA, HIV } Serum autoantibodies [ 60%-70% ] IMAGING STUDIES: MRI EMG NCS

CONTD…….:

CONTD……. PULMONARY FUNCTION TESTS: Maximal Inspiratory/Expiratory pressures & Vital Capacities Lumbar Puncture HISTOLOGICAL STUDIES: Lymphocyte & Macrophage infiltration in peripheral nerves & Wall-erian degeneration.

MEDICAL MANAGEMENT:

MEDICAL MANAGEMENT REHABILITATION PROGRAMME: Physical therapy Occupational therapy Speech therapy Recreational therapy

CONTD…..:

CONTD….. PHARMACOTHERAPY: Antibiotic agents Anticoagulant agents Plasmapharesis

SURGICAL MANAGEMENT:

SURGICAL MANAGEMENT Tracheostomy

COMPLICATIONS OF GBS:

COMPLICATIONS OF GBS Respiratory failure Cardiac dysrhythmias Complications of immobility & paralysis Anxiety & depression

NURSING MANAGEMENT:

NURSING MANAGEMENT ASSESSMENT: History Physical Examination Neurological Examination

CONTD…….:

CONTD……. NURSING DIAGNOSIS: Ineffective breathing pattern & impaired gas exchange R/T rapidly progressive weakness & impending respiratory failure. GOAL: To improve respiratory function.

CONTD…….:

CONTD……. INTERVENTIONS: Assess the respiratory status frequently. Provide chest physiotherapy. Monitor for vital capacity & -ve inspiratory force.

CONTD………:

CONTD……… NURSING DIAGNOSIS: Impaired physical mobility R/T paralysis. GOAL:- to increase physical mobility. INTERVENTIONS:- Support the paralyzed extreemitiees in functional positions & passive ROM exercises at least twice daily

CONTD..:

CONTD.. Administer anticoagulant therapy, adequate hydration to prevent from DVT . Padding may be placed over bony prominences to prevent from pressure ulcers. revaluate the lab test for malnutrition & dehydration.

CONTD…..:

CONTD….. NURSING DIAGNOSIS:- Imbalanced nutrition less than body requirement R/T inability to swallow GOAL: To improve nutritional status

CONTD…:

CONTD… INTERVENTIONS: Administer IV fluids/ parenteral nutrition Monitor for return of bowel sounds Insert the gastrostomy tube if patient can’t swallow Monitor for gag reflex

CONTD…:

CONTD… NURSING DIAGNOSIS: Impaired verbal communication R/T cranial nerve dysfunction. GOAL: To promote effective communication.

CONTD….:

CONTD…. INTERVENTIOS: Establish form of communication with picture cards, or an eye blink system.

CONTD…:

CONTD… NURSING DIAGNOSIS: Fear & anxiety R/T loss of control & paralysis GOAL: To decrease the fear & anxiety

CONTD….:

CONTD…. INTERVENTIONS: Communicate with patient Educate patient & family members about disease , prognosis & treatment Do not make the patient alone at any time Teach relaxation techniques Modify the environment.

BIBLIOGRAPHY:

BIBLIOGRAPHY Mosby, “Comprehensive review of nursing for NCLEX-RN ”17 th ed. , published by St. Louis London Philadelphia. Sydney ,Pp-895-900. Suddharth & Brunner, “Medical Surgical nursing” 10 th ed. Lippincott publishers, Pp-1956-1962.

CONTD….:

CONTD…. Black M. Joyecee, “Medical Surgical Nursing” 6 th ed. Vol. 2 , Sounder’s publishers, Pp-800-807 WWW.Goggle. com

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