part 3 huntingtons to epilepsy

7/27/2019 Part 3 Huntingtons to Epilepsy

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DISTURBANCES OF THE NERVOUS SYSTEM (Series 3)By Franklin C. Barberan R.N. 2

ndPlace Board Topnotcher

Michelle A. Barberan R.N. 3rd

Place Board Topnotcher

DISTURBANCE #9: HUNTINGTONS DISEASE

DESCRIPTION:

Chronic, progressive hereditary disease of the nervous system. An autosomal dominant genetic disorder Characterized by:

involuntary choreiform (dancelike) movements. dementia

K! each child of a parent with the disease has 50 % chance of developing the disease.

ETIOLOGY:

Involves premature death of cells in the basal ganglia (involve in control of motormovement)

There is also loss of cells in the cortex (leads to impaired thinking, memory, perception, andjudgment).

Lack of important brain chemicals like the gamma-aminobutyric acid and acetylcholine

INCIDENCE: Onset usually 35-45 years old

MANIFESTATIONS:

Chorea constant twisting, writhing movement of the body facial tics and grimaces speech impairment

n! There is constant danger of aspiration ambulation is impaired eventually

Cognitive changes: dementia

Intellectual decline Emotional disturbances irritability impatience profound suicidal depression apathy or euphoria

K! Despite strong appetite for sweets, the patient is EMACIATEDMANAGEMENT:

K! NO TREATMENT stops or reverses the underlying process.

Phenothiazines, Butyrophenones blocks dopamine receptors thereby decreases chorea Thioxanthines

RESERPINES: depletes presynaptic dopamine.

PATIENT EDUCATION:

Consider genetic counseling


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Long term counseling Regular check ups Emphasize diet high in calories to provide adequate source of energy.

DISTURBANCE #10: ALZHEIMERS DISEASE

Also called: Primary Degenerative Dementia Senile dementia of Alzheimers type (SDAT)

DEFINITION: Progressive IRREVERSIBLE degenerative neurologic diseaseK! Onset is insidious characterized by:

Gradual loss of cognitive function Disturbance in behavior and affect

ETIOLOGY AND INCIDENCE: Age are established risk factors Family historyK! Genetic predisposition involves:

chromosome 14 (early onset AD).chromosome 19 (late onset)chromosome 21

K! Onset is common at age 65 and above.

PATHOPHYSIOLOGY:

K! There is DEPOSITION of : neurofibrillary tangles & at the cerebral cortex that leads senile/ neuritic plaques to decreased brain size

DIAGNOSTIC EVALUATION: EEG To exclude other diseases but are not reliable to CT- scan diagnose AD. MRI

K! Diagnosis can only be confirmed by cerebral biopsy /autopsy

MANIFESTATIONS:K! During the early stage,

Forgetfulness and subtle memory loss Depression may occur Inability to recognize faces, places, and common objects Conversation may become difficult (Patient forgets what to say) Inability to formulate concepts and abstraction disappears. Agitation and physical activity increase

K! During the terminal stage;

Patient becomes immobile Death occurs from complications

MANAGEMENT:

Tacrine HCl the first drug approved for alzheimers disease


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N! Adverse effects: Toxic to the liver.NURSING INTERVENTIONS:

Support cognitive functions. K! provide calm and predictable environment provide clear and simple instructions Use memory aids and cues Frequent reality orientation Color coding the doorway may be helpful. prominent clock and calendar

Promote physical safety Remove all potential hazards provide night lights low bed smoking may be allowed only with supervision wandering behavior can be reduced by gently persuading or by

distracting the person Use bracelet identification

Reduce anxiety and agitation Provide recreational activities set realistic goals provide constant emotional support that will enhance positive self image.

Improve communication; Reduce noises and distractions use clear, easy to understand sentences List simple written instructions

Provide for socialization and intimacy needs Promote adequate nutrition Promote balanced activity and rest Support and educate caregivers.

DISTURBANCE #11: MYASTHENIA GRAVIS

DEFINITION: An autoimmune disease wherein antibodies destroy acetylcholine receptors impairing the

neuromuscular transmission of impulses.

Loss of acetylcholine receptors at neuromuscular junctions

Impaired nerve transmission

MANIFESTATIONS: Extreme muscular weakness and easy fatigability. (N! Worse after exertion and is relieved

by rest) N! Patient tires easily even on slightest effort like chewing. There is diplopia and ptosis due to involvement of ocular muscles. Patient has sleepy, masklike expression due to involvement of facial muscles. K! Weakness of bulbar muscles results problems with chewing and swallowing. N!Potential for choking!!! K! There is weakness of diaphragm and intercostal muscles. (N! Results to respiratory

distress!!!!!)

DIAGNOSTIC EVALUATION:


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1. Edrophonium (Tensilon test) a medication that facilitates transmission of impulses atthe myeoneural junction.

How to do test? Ans: Within 30 seconds after IV injection oftensilon, A TEMPORARY IMPROVEMENT OF

MUSCULAR STRENGTH confirms the diagnosis of myasthenia gravis.

2. Demonstration of Anti-AchR antibodies. (Antiacetylcholine receptor antibodies)3. Electromyography measures the electrical potential of muscle cells. (But is NOT

CONSIDERED specific for myasthenia gravis).

MANAGEMENT:

1. To improve muscular functioning:K! Anticholinesterase medications to increase relative

concentration of available acetylcholine at neuromuscularjunction.

Pyridostigmine bromide (Mestinon) Ambenonium Cl (Mytelase) Neostigmine bromide (Prostigmin) N! give anticholinesterase drugs together with milk, crackers, or

other buffering foods.

N! SIDE EFFECTS!!!!GIT

abdominal cramps nausea and vomiting diarrheamuscular system

fasciculations spasms weaknessCNS

irritability, anxiety, dysarthria seizures, coma

N! Give the medications on time (RATIONALE: Any delay mayresult to patient inability to swallow, making oral

administration difficult)

2. Immunosupressive therapyK! Goal:

to reduce production of antireceptor antibody remove it by plasma exchange

Ex. * Corticosteroids - prednisone* cytotoxic drugs azathioprine (Imuran),

- cyclophosphamide (Cytoxan)

reduces the circulating antiacetylcholine receptor titers

* plasmapheresis

* thymectomy

COMPLICATIONS:

1. Myasthenic crisis: Sudden onset of muscular weakness in patients with myasthenia. Usually due to

under medication.


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K! May also result from: progression of the disease itself emotional upset systemic infections certain medications trauma.Signs and symptoms:

acute respiratory distress inability to speak or swallow

2. Cholinergic crisis

caused by overmedication with cholinergic or anticholinesterase agents NURSING ALERT! Medications that can aggravate myasthenia gravis

o morphineo quinineo betablockerso certain antibioticso cardiovascular drugso anticonvulsants and antipsychotic drugs

NURSING DIAGNOSIS

ineffective breathing pattern R/T weakness of respiratory muscles impaired physical mobility R/T voluntary muscle weakness risk for aspiration R/T weakness of bulbar muscles

NURSING INTERVENTIONS:

Assess respiratory status Promote physical mobility without exhausting the patient Provide health teaching covering the following:

taking medications on time factors that could trigger myasthenic crisis

Provide eye care: For PTOSIS, vision can be enhanced by taping the eyes open for short intervals. Use artificial tears if necessary. Place patch on one eye if there is double vision

Prevent aspiration: N! watch for drooling, regurgitation through the nose, and choking when attempting

to swallow. ALERT!!!! Always keep SUCTION machine readily available rest before meals Keep patient on upright position with neck slightly flexed to facilitate

swallowing. K! soft foods in gravy or sauces appear to be easily swallowed than liquids.

ALERT!!!! Mealtime should coincide with the PEAK EFFECTS ofanticholinesterase if patient has difficulty of swallowing.

HEALTH TEACHING: teach patient and family how to use suction.

DISTURBANCE #12: AMYOTROPHIC LATERAL SCLEROSIS(LOU GEHRIGS DISEASE)

DESCRIPTION:

Loss of motor neurons in the anterior horns of the spinal cords that leads atrophy of themuscles being supplied.


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May occur both in upper and lower neuronsETIOLOGY: Unknown

INCIDENCE: Common in men, age 50, 60s

MANIFESTATIONS: K! Signs and symptoms depends on the location of the affected motor neurons. K! The chief signs and symptoms are:

Muscle weakness Atrophy Fasciculations

DIAGNOSIS:

K! No specific clinical or laboratory test. Diagnosis is based on symptomatology.

MANAGEMENT: No specific treatment for ALS is available. Symptomatic Rehabilitation of affected body part

POSSIBLE NURSING DIAGNOSIS:

Alteration in body image. Impaired mobility. High risk for injury. Self esteem disturbance. Ineffective coping mechanisms. Alterations in ADLs

CONVULSIVE DISORDERS

DISTURBANCE #13: SEIZURES (Convulsions)

DESCRIPTION: Episodes of abnormal motor sensory, autonomic, or psychic activity

ETIOLOGY:A. Idiopathic Genetic Developmental defects

B. Acquired Hypoxemia Fever Head injury CNS infections Metabolic and toxic conditions. Ex. Renal failure, hypocalcemia, hypoglycemia. Brain tumor Drug withdrawal

MANAGEMENT:

K! Assess the following.1. The circumstances before the seizure (stimuli, emotional, or psychological

disturbances).


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2. The first thing the patient does in a seizure. (ex. movement, start of stiffness)3. Types of movement in the body part involved.4. Size of pupils. Are the eyes open? Does head turn to one side?5. Involuntary motor activity (ex. Automatism like lip smacking).6. Incontinence of urine / feces.7. Duration of seizure.8. Presence and duration of unconsciousness if present.

AFTER THE SEIZURE, ASSESS FOR THE FOLLOWING;9. Any paralysis or weakness of arms/ legs10.Inability to speak11.Movement12.Whether or not the patient sleeps13.Presence of confusionCARE OF THE PATIENT DURING SEIZURE

Nursing Goal: Prevent injury to the patient:

During seizure:

Provide privacy. Protect patient from curious onlookers. Ease the patient to the floor if possible. Protect head from striking a hard surface. Loosen constrictive clothing Clear area of anything that may injure patient during the seizure. Remove the pillows and raise side rails. If an aura precedes the seizure, insert a padded tongue blade between

the teeth to reduce risk of biting the tongue or cheek. Do not attempt to pry open the jaws that are clenched to insert

anything .

Do not attempt to restraint patient during the seizure. Whenever possible, position patient on one side with head flexed

forward to facilitate drainage of saliva and mucus. If suction is available, suction if necessary.

After seizure

Maintain a patent airway Keep patient on a side lying position to prevent aspiration. Upon awakening, reorient the patient as there is usually disorientationafter a grand mal seizure. If patient is experiencing severe excitement after the seizure, handle

the situation with calm persuasion and gentle restraint.

DISTURBANCE #14: EPILEPSY

DEFINITION:

A symptom complex which is thought to be due to an electrical disturbance in the nervecells causing them to emit abnormal recurring, uncontrolled electrical discharges.

ETIOLOGY:K! In many cases the cause is UNKNOWN

K! Epilepsies often follow:

birth trauma asphyxia neonatorum head injuries some infectious diseases


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toxicity (carbon monoxide and lead poisoning fever nutritional deficiencies drug/ alcohol intoxication

K! There is evidence that susceptibility to some type can be inherited.

PREVENTION:

1. Infants of mothers taking anticonvulsants are at risk. Monitor blood levelsduring pregnancy

2. Assess mother about: Histories of difficult delivery R! A brain lesion that can cause Drug abuse epilepsy may develop. Diabetes Hypertension

3. Control childhood infections with appropriate vaccinations and therapy4. Instruct mothers how to manage febrile seizures.5. Avoid head injuries (safety awareness).

MANIFESTATIONS:

SEIZURES ranges from a simple staring spell to prolonged convulsive movements.AURA - a premonitory symptom like seeing a flashing light.

TYPES OF SEIZURES:

1. Partial seizure: Focal in origin Affect only part of the brain Generally without impairment of consciousness

Types of Partial seizuresa. Simple partial seizure complex

only a finger or hand may shake mouth may jerk uncontrollably may be dizzy may experience unusual or unpleasant sights, sounds, odors, or tastes but

without loss of consciousness.b. Partial seizure:

person may become motionless may move automatically but inappropriately may experience excessive emotion

K! Whatever episodes occur, the patient does NOT remember

afterwards.

2. Generalized seizures (Grand mal seizures) Involves both hemispheres of the brain (both sides of the body react) There is intense rigidity Jerky alternations of muscle contraction and relaxation (tonic-clonic) Epileptic cry may be produced by the simultaneous contraction of the diaphragm

and chest muscles.K! During the postictal state, patient is often confused , hard to arouse,

and may sleep for several hours.

DIAGNOSIS:

K! Goals of diagnostic assessment:


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1. Determine the: Type of seizure Frequency Severity Factors that precipitate an attack.

2. CT- scan to determine/ rule out presence of a brain lesion3. EEG - provides information about the electrical activity of the brain.

MANAGEMENT:

K! To control seizure:

1. Phenytoin (Dilantin)N! Provide thorough;

Oral hygiene after each meal Regular dental care Regular gum massage

R! To prevent gingival hyperplasia

K! SIDE EFFECTS of phenytoin!!!! Visual problems Hirsutism Gingival hyperplasia Dysrhythmias

N! TOXIC EFFECTS!!!! Severe skin reaction Peripheral neuropathy Ataxia and drowsiness Blood dyscrasias

2. PhenobarbitalSIDE EFFECTS!!! sedation, irritability diplopia ataxia

TOXIC EFFECT: skin rashes3. Carbamazepine:

SIDE EFFECTS: dizziness, drowsiness unsteadiness nausea and vomiting diplopia; mild leukopeniaTOXIC EFFECTS: severe skin rash blood dyscrasias hepatitis

4. Other medications include: Primidone Ethosuximide Valproate

NURSING INTERVENTIONS:

1. Emphasize that anticonvulsant is NOT a habit forming drug and must be takencontinuously

2. Avoid alcohol3. Some patients may need to avoid photic stimulation. Ex. Bright flickering lights,

television viewing. (Advice to wear dark glasses or cover one eye may be helpful).

4. Instruct patient to look for a safe place at first sign of impending seizure.5. Have a balance between rest and exercise. Avoid over expenditure of energy.


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6. Assist patient through psychological aspects of adaptation to the disease7. Watch for potential complications.

DISTURBANCE #15: STATUS EPILEPTICUS

DESCRIPTION: Prolonged generalized seizure that occur without full recovery of consciousness

between attacks

Continuous electrical or clinical seizures at least 30 minutes even withoutimpairment of consciousness.

MANIFESTATIONS:

Vigorous muscular contractions that impose heavy metabolic demands Respiratory arrest possible at the height of each seizure. This leads to venous

congestion and cerebral hypoxia and eventually fatal brain injury.

MANAGEMENT: Goal:

To stop the seizure as soon as possible. Ensure adequate cerebral oxygenation Maintain a seizure free state

Medications: Diazepam to stop the seizure immediately Phenytoin and Phenobarbital given after diazepam because the effect of

diazepam is short lived. Intravenous dextrose to manage hypoglycemia if it has caused the seizure General anesthesia if the above medications fail.

NURSING INTERVENTIONS: Monitor respiratory and cardiac functions. Monitor vital signs and neurologic status. Position patient on his side if possible. Protect patient from injury Implement seizure precautions. (Refer to fundamentals of Nursing for details)

DISCLAIMER: This handout is not complete by itself. Some contents are best

understood if are related to other systems. For example, we presented herethe GBS. Although it is a neurologic disorder, it has important INTEGRATION

to Respiratory System Interventions. The most important is realize every

disease in a very holistic manner in terms of nursing care needs- very

challenging! Need assistance? Come to Health Ambassadors Nursing Review.

You will be guided by Topnotchers in the Board Exams.

Contact us via facebook (NURSE AMBASSADOR)... our page (HEALTH

AMBASSADORS UNIVERSITY) our email: [email protected] . We will call

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part 3 huntingtons to epilepsy

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