Myasthenia Gravis

Myasthenia Gravis

Chronic autoimmune disease of the neuromotor junction presents as muscle weakness and fatigue

Clinical manifestations
* Weakness
* Fatigue
* Ptosis
* Diplopia
* Facial muscles weakness
* Dysphagia
* Nasal quality to speech
* Respiratory distress
* Muscles involved – eyes, eyelids, chewing, swallowing
* Speech affected
* Muscles of the trunk and limbs less affected
* Proximal muscles of the neck, shoulder and hips are affected
* No sensory loss
* Reflexes normal
* Muscle atrophy rare
* Pt may have exacerbation and remission

Exacerbation of MG
* Emotional stress
* Pregnancy
* Menses
* Secondary illness
* Trauma
* Temperature extremes
* Hypokalemia
* Drugs – aminoglycosides antibiotics, beta blockers, procainamide, quinidine, phentoin, and some psychotropic drugs

Diagnostic
* History and physical
* Antibodies to ACH receptors
* Upward gaze
* EMG
* Tensilon test

Management
* Anticholinesterase drugs – mestinon, prostigmin
* Corticosteriods
* Immunosuppressant drugs – imuran, cytoxan
* Must check for other drug interactions antibiotic, antiarrhythmics, diuretics etc.
* Surgery – removal of thymus gland
* plasmapheresis

Nursing care
* Admin. anticholinesterase drugs
* Respiratory assessment – suction
* Elevate HOB when eating
* Check swallow reflex – oral motor strength
* Plan activities – muscles strongest in morning
* Assess muscle strength before and after activity

Myasthenic crisis
* Due to exacerbation of myasthenia or failure to take drug
* S/S – improved strength with anticholinesterase drugs, inc. weakness of skeletal muscles, ptosis, difficulty on swallowing, articulating words, dyspnea

Cholinergic crisis
* Due to overdose
* S/S – weakness within 1 hour of taking anticholinesterase drug, ptosis, dyspnea, blurred vision, salivations, diarrhea, N/V, abd. cramps, inc. bronchial secretions, sweating, lacrimation
* Due to overdose
* S/S – weakness within 1 hour of taking anticholinesterase drug, ptosis, dyspnea, blurred vision, salivations, diarrhea, N/V, abd. cramps, inc. bronchial secretions, sweating, lacrimation, difficulty swallowing, dyspnea

Nursing DX
Discharge teaching
* Instruct on disease process
* Importance of drug regime – sch drugs at peak action at mealtime, other drug interactions
* Suction equipment at home
* S/S of underdose and overdose of meds
* Instruct on precipitating factors
* Diet – semisolid food
* Rest, Plan activities
* Use of adaptive devices – OT, home care
* MG support group, Community resources

Amyotrophic Lateral Sclerosis
* Known as Lou Gehrig’s disease
* Cause unknown
* Motor neurons in the brainstem and spinal cord gradually degenerate
* Electrical and chemical messages originating in the brain do not reach the muscles to activate them
* Death within 2-6 years after diagnosis

ALS
* S/S – weakness of upper extremities
* Dysarthria
* Dysphagia
* Weakness may begin in legs
* Muscle wasting, fasciculations
* Sensory intact
* Death usually results from respiratory infection

Dx
* Difficult to dx- rule out other diseases
* EMG
* MRI
ALS
* Treatment – Riluzole to slow progression
* No cure
* Cognition is intact

Management
* Supportive therapy – OT, PT, RT
* Assess client’s ability to do ADL
* Conserve energy
* Encourage small freq meals
* Suction equipment
* Soft collar to stabilize head
* Adaptive equipment
* Allow time to complete activities
* Avoid exposure to anyone with respiratory infection
* Good posture and swallowing techniques
* Diaphragmatic breathing
* Follow up pulmonary tests
* Home care
* ALS support group

Multiple Sclerosis
* A chronic progressive degenerative disease that affects the myelin sheath of neurons in the CNS
* Cause unknown – genetic, virus, autoimmune response, inherited, antigen-antibody reaction

Clinical Course
* Relapsing – remitting: relapses with full recovery and residual deficit with recovery
* Primary – progressive: dx progression from onset with occ plateaus and temp. minor improvements
* Secondary – progressive: relapsing-remitting course followed by progression with or without relapses, minor remission and plateaus
* Progressive – relapsing: Progressive dx, with acute relapses with or without full recovery, periods between relapses cont. progression

Dx
* History and Physical
* CSF analysis
* CT scan
* MRI

Clinical manifestations
* S/S may vary
* Motor- weakness or paralysis,of the limbs, trunk, or head, speech problems, spasticity of the muscles
* Sensory – numbness, tingling, paresthesia, visual changes, vertigo, tinnitus, decrease hearing, chronic neuropathic pain, radicular pain (pain in thoracic area and abdominal region), lhermitte’s phenomenon – electric shock radiating down the spine, into the limbs with the flexion of the neck

Clinical manifestations
* Cerebellar signs – nystagmus, ataxia, dysarthria, dysphagia
* B/B function can be affected
* Constipation a problem
* Spastic bladder – incont.
* Flaccid bladder – no sensation of voiding
* Mood swings, Intellect intact
* S/S may be triggered by physical, emotional trauma, fatigue and infection

Medical Management
* Corticosteriods
* Immunomodulators – B-interferon
* Immunosuppressants
* Cholinergics - flaccid bladder
* Anticholinergic – spastic bladder
* Muscle relaxants
* Surgery – control tremors

Nursing Dx
Guillain Barre
* An acute form of polyneuritis
* Etiology unknown
* A cell mediated immunologic reaction directed at the peripheral nerves
* Involves degeneration of the myelin sheath of the peripheral nerves
* In half of cases, an upper respiratory or GI infection precedes the onset of the syndrome by 1-4 weeks
* Antecedent illness-cytomegalovirus, Epstein Barr virus, mycoplasma pneumonia, salmonella typhosa, campylobacter jejuni, HIV
* A chronic form of GB paralysis evolves more slowly with no involvement of respiratory of cranial nerves
* With support, pt will recover

DX
* History and physical exam
* Electrophysiological studies
* Cerebrospinal fluid with elevated protein levels
* EMG

Characteristics of GB
* Ascending weakness usually beginning in the lower extremities and spreading to trunk, upper extremities and face
* Improvement and recovery occur with remyelination; if nerve axons are damaged
* Some residual deficit may remain
* Recovery is usually 6 months with 85%-90% of clients recovering completely
* 10% have recurrence and 20% have long term disabilities/emotional trauma

Guillian Barre
* Complication- is respiratory failure
* Impt to monitor respiratory rate, depth, vital capacity
* Client may be intubated with mechanical ventilation
* Complications can occur due to immobility

Clinical Manifestations
* Flaccid quadraplegia
* Facial weakness, dysphagia, diplopia, hypotonia
* Autonomic dysfunction found in severe muscle involvement and respiratory muscle paralysis – orthostatic hypotension, hypertension, pupillary disturbances, sweating dysfunction, bradycardia, paralytic ileus, urinary retention
* Weakness
* Paresthesia of the limbs
* Loss of deep tendon reflexes
* Deep, aching muscle pain in shoulder and thighs
* Respiratory compromise or failure-dyspnea, dec. breath sounds, dec. tidal volume (air in & out)

Medical/Nursing Management
* Supportive care
* Immunoglobulin therapy
* Pain control worse at night due to paresthesia, muscle aches and cramps
* Problems - airway, aspiration, communication problems, orthostatic hypotension, nutritional intake
* Plasmaphoresis
* ABGs
* Assist ability to perform self care
* Set communication system
* Work closely with PT, OT
* Monitor for complications of immobility
* Safety measures provided

Myasthenia Gravis.ppt