POLICIOS, SHARMAINE ANNE M.

BSN 3Y2 – 3A AUTOIMMUNE DISEASES

DEGENERATIVE DISEASES plasmapheresis
HUNTINGTON’S
AMYOTROPHIC GUILLAIN BARRE
PARKINSON’S DISEASE (HD) ; MYASTHENIA
LATERAL SCLEROSIS MULTIPLE SCLEROSIS (MS) SYNDROME (GBS) /
DISEASE (PD) HUNTINGTON’S GRAVIS (MG)
(ALS) Polyradiculoneuritis
CHOREA (dance)
Disorder causes There is destruction of As motor neuron cells die, Sensitized T cells that would disturbance in the GBS results from an
degeneration of cells in the caudate the muscle fibers that they typically cross the blood-brain transmission of autoimmune (cell-mediated and
the dopamine- nucleus and putamen areas supply undergo atrophic barrier to check for antigens in the impuled from nerve to humoral) attack on peripheral
producing neurons of the basal ganglia and changes. CNS and then leave; in MS would mucle cells @ the nerve myelin proteins
( in the substantia extrapyramidal motor remain in the CNS neuromuscular ( substances speeding
nigra in the system. junction= PNS conduction of nerve impulses) .
midbrain ) Neuronal degeneration
may occur in both the
The neurotransmitters, upper and lower motor Promote infiltration of other
Dopamine gamma-aminobutyric acid neuron systems. agents that damage the immune causing extensive The Schwann cell( which
influences -(GABA) and Ach are system muscle weakness produces myelin in the
PATHOPHYSIOLOG purposeful decreased. -----Dopamine peripheral nervous system) is
Y movement is not affected, but the paired in GBS,
decrease of Ach cause The leading theory held by
relative increase of researchers is that over Immune system attack leads to
Depletion of dopamine in the basal excitation of nerve cells inflamm that destroys myelin and
dopamine ganglia. by the neurotransmitter oligodenroglial cells allowing for remyelination in the
glutamate leads to cell recovery phase of the disorder.
injury and neuronal
degeneration.
results in The excess dopamine
degeneration of causes uncontrolled
the basal ganglia. movement in Huntington’s
chorea.
MEANING Parkinson's  Huntington’s disease  Amyotrophic lateral  Multiple sclerosis is a chronic,  Myasthenia Gravis  Also known as
symptoms usually (also called sclerosis (ALS) is a progressive, non- contagious, is a neuromuscular polyradiculoneuritis.
begin gradually Huntington’s chorea) disease of unknown degenerative disease of the disease  It is an acute inflammatory
and get worse over  A rare abnormal cause in which there is CNS characterized by characterized by polyneuropathy of the
time. hereditary disorder of a loss of motor demyelinization of the considerable peripheral sensory and
 difficulty the CNS. neurons (nerve cells neurons. weakness and motor and nerve roots.
 walking and  It is characterized by controlling muscles)  Multiple sclerosis usually abnormal fatigue of  Affected nerves are
talking. chronic progressive in the anterior horns of occurs between the ages of 20 the voluntary demyelinated with possible
 behavioral chorea (involuntary the spinal cord and the and 40 and consists of periods muscles. axonal degeneration.
changes, purposeless, rapid motor nuclei of the of remissions and  A defect in the  It’s exact cause is unknown,
 sleep movements) and lower brain stem. exacerbations. transmission of Guillain-Barré Syndrome is
problems, mental deterioration  It is often referred to  The causes are unknown, but nerve impulses at believed to be an
 depression, that results in as Lou Gehrig’s the disease is thought to be a the myoneural autoimmune disorder that
 memory dementia. disease. result of an autoimmune junction occurs. may be triggered by viral
difficulties,  Chorea, the Greek response or viral infection.  Causes include infection, Campylobacter
 fatigue. word meaning  Precipitating factors include insufficient diarrheal illness,
“dance”. pregnancy, fatigue, stress, secretion of immunization, or other
infection, and trauma. acetylcholine, precipitating event.
 Electroencephalogram excessive secretion  The syndrome is marked by
findings are abnormal of cholinesterase, acute onset of symmetric
 A lumbar puncture indicates and progressive muscle
increased gamma globulin, unresponsiveness weakness, most often
but the serum globulin level is of the muscle fibers beginning in the legs and
normal. to acetylcholine. ascending to involve the
trunk, upper extremities, and
facial muscles. Paralysis
may develop.
 Complications may include
respiratory failure, cardiac
arrhythmias, and
complications of immobility.

GENDER men > women men=women men>women women>men women>men men=women

 age 60  onset 35-45 y.o. 10% onset 50-60 y.o. 20-40 y.o. W: 15-35 y.o. occurs at any age
 "early-onset" are children M: 40 y.o.
AGE
before the age
of 50
CAUSE UNKNOWN UNKNOWN  The cause of MS is unknown.  MG, thought to be  Although the exact cause of
Any one or more of the
The majority of all Some evidence suggests that an autoimmune GBS is unknown, two-thirds
following factors may be
cases of classic an infective agent causes a disorder, is caused of patients who develop it
responsible for the
Parkinson’s predisposition to MS, by a loss of have had a viral or bacterial
disease:
disease are although that agent has not acetylcholine infection 1 to 3 weeks before
 primary, or  Defective been identified. (ACh) receptors in the development of
idiopathic, glutamate  The risk of developing MS is the postsynaptic symptoms.
Parkinson’s metabolism 15 times higher when the neurons at the  The most typical site and
disease (IPD).  Free radical injury disease is present in the neuromuscular cause of infections are a
 a hereditary  Mitochondrial patient’s immediate family. junction. lung or intestinal infection
pattern. dysfunction  Conditions such as pregnancy,  About 80% of all caused by Campylobacter
 Secondary, or  Gene defects infection, and trauma seem to MG patients have jejuni(C. jejuni) or
 iatrogenic,  Programmed cell precipitate the onset of MS or elevated titers for cytomegalovirus (CMV).
 A drug- or death or apoptosis cause relapses ACh receptor Infections with Epstein-Barr
chemical-  Cytoskeletal antibodies, which virus and Mycoplasma
related. protein defects can prevent the pneumoniae are also
 Dopamine-  Autoimmune and ACh molecule associated with GBS.
depleting inflammatory from binding to  Another 10% of patients
drugs such as mechanisms these receptor sites have had recent surgical
reserpine,  Accumulation of or can cause procedures during the 4
phenothiazine, protein aggregates damage to them. weeks before GBS
metocloprami (clumps)  MG is often developed.
de,  Viral infections associated with  Other diseases that have
tetrabenazine, thymic tumors. been linked to the
and the also a theory: development of GBS are
butyrophenon overexcitation of nerve lymphoma,human
es (droperidol cells by glutamate immunodeficiency virus
and  (HIV)
haloperidol) Cell injury disease,gastroenteritis,Hodg
can lead to kin’s disease, and lupus
secondary erythematosus. In some
Parkinson’s cases,GBS develops after
disease. immunization for influenza.
DXTIC TEST  Positron X  EMG studies of X Tensilon test 1. Lumbar puncture
emission affected muscles EMG obtains cerebrospinal fluid
tomography indicate reduction in Blood test samples, which reveal low
(PET) the number of -w/ anti-acetylcholine cell count and high protein
 Single photon functioning motor receptor antibodies levels.
emission units 2. Nerve conduction
computed  MRI may show studies, which allow
tomography high signal intensity decreased conduction
 (SPECT) in the corticospinal velocity of peripheral
shows tracts nerves due to
decrease demyelination.
dopamine 3. Abnormal laboratory
uptake in the studies may point to prior
basal ganglia. infection or illness.

CSF studies: ↑CHON

EMG: slow nerve
conduction
S/SXS  Tremor  Intellectual decline The signs and  1st sx: visual disturbances:  Ptosis, diplopia ,
(trembling) in  Abnormal movements symptoms presented blurred vision, scotomas dysphagia
hands, arms,  Restlessness, depend on the location of (patchy blindness), diplopia  Extreme muscle
legs, jaw, or forgetfulness, the affected neuron.  Impaired sensation: touch, weakness,↑ed with
head clumsiness, frequent Generally, the following pain, temperature, or position activity and
 Stiffness of falls presentations are evident: sense; numbness, tingling reduced with rest
the limbs and  Problems with speech,  Fatigue  Impaired motor function: (identifying
trunk coordination and  Progressive weakness, paralysis, spasticity characteristic)
 Slowness of balance muscle weakness  Impaired cerebellar function:  Masklike facial
movement  Depression, memory  Cramps scanning speech, ataxic gait, expression
 Impaired loss, emotional lability  Twitching nystagmus, dysarthria,  Weak voice,
balance and and impulsiveness  Incoordination intention tremor hoarseness
coordination,  Facial grimaces, Anterior horns  Euphoria or mood swings
sometimes protrusion of the  Progressive  Bladder: retention or
leading to falls tongue, jerky weakness incontinence
Other symptoms: movements of the  Muscle atrophy
 Constipation
 depression a arms and legs (arms, trunk, legs)
 Sexual impotence in the male
 other  Gait disturbances,  Spasticity
emotional patient at risk for falls  Brisk or
changes; overreactive muscle
 difficulty reflexes
swallowing, Cranial nerves
 chewing,  Muscle weakness
 speaking;  Difficulty talking
 urinary  Difficulty
problems or swallowing
  constipation;  Difficulty
 skin problems; breathing
 and sleep  Soft palate and
disruptions. upper esophageal
weakness
 Weakness on the
posterior tongue
Bulbar muscles
 Progressive
difficulty in speaking
 Difficulty in
swallowing
 Articulation and
speech effects
 Compromised
respiratory function

NURSING 1. Assess 1. Prevent injury and 1. Provide 1. Provide bed rest during 1. Monitor 1. Monitor respiratory
MANAGEMENT neurological possible skin intellectual exacerbation. respiratory status status through vital
status. breakdown stimulating activities, 2. Protect the client from and ability to capacity measurements,
2. Assess  Pad the because the client injury by providing safety cough and deep rate and depth of
ability to sides and head typically experiences measures. breathe respirations, and breath
swallow and of the bed no cognitive deficits 3. Place an eye patch on the adequately. sounds.
chew.  Keep the and retains mental eye for diplopia. 2. Monitor for 2. Monitor level of muscle
3. Provide skin abilities. 4. Monitor for potential respiratory failure. weakness as it ascends
high-calorie, meticulously 2. Provide client and complications such as 3. Maintain toward respiratory muscles.
high-protien, clean family teaching. urinary tract infections, suctioning and Watch for breathlessness
high-fiber  Encourage 3. Promote measures calculuses, decubitus ulcers, emergency while talking which is a
soft diet with ambulation with to enhance body respiratory tract infections, equipment at the sign of respiratory fatigue.
small, assistance image. and contractures. bedside. 3. Monitor the patient for
frequent to maintain mus 4. Promote client 5. Promote regular 4. Monitor vital signs of impending
feedings. cle tone and family coping as elimination by bladder and signs. respiratory failure.
4. Increase  Secure the the client and his bowel training. 5. Monitor speech 4. Monitor gag reflex and
fluid intake patient in bed or family deal with the 6. Encourage independence. and swallowing swallowing ability.
to 2000 chair with poor prognosis and 7. Assist the client to abilities to prevent 5. Position patient with the
mL/day. padded the grieving process establish a regular exercise aspiration. head of bed elevated to
5. Monitor protective 5. Provide referrals. and rest program. 6. Encourage the provide for maximum chest
for devices making 6. Maximize 8. Instruct the client to client to sit up excursion.
constipation. sure they are functional abilities balance moderate activity when eating. 6. Avoid giving opioids
6. Promote loosened  Prevent with rest periods. 7. Assess muscle and sedatives that may
independence frequently complications 9. Assess the need for and status. depress respirations.
along with 2. Keep patient as of immobility provide assistive devices. 8. Instruct the 7. Position patient
safety close to upright as  Promote 10. Initiate physical and client to conserve correctly and provide
measures. possible while self-care speech therapy. strength. range-of-motion exercises.
7. Avoid feeding. Stabilize  Maximize 11. Instruct the client to avoid 9. Plan short 8. Provide good body
rushing the patient’s head gently effective fatigue, stress, infection, activities that alignment, range-of-motion
client with with one hand while communication overheating, and chilling. coincide with exercises, and change of
activities. feeding 7. Ensure adequate 12. Instruct the client to times of maximal position to prevent
8. Assist 3. The nurse needs to nutrition increase fluid intake and eat muscle strength. complications such as
with educate and support 8. Prevent a balanced diet, including 10. Monitor for contractures, pressure
ambulation the patient and family respiratory low-fat, high-fiber foods and myasthenic and sores, and dependent
and provide as they adjust to the complications foods high in potassium. cholinergic crises. edema.
assistive lifestyle changes that  Promote 13. Instruct the client in 11. Administer 9. Ensure adequate
devices. are required. measures to safety measures related to anticholinesterase nutrition without the risk of
9. Instruct 4. The actions and maintain sensory loss, such as medications as aspiration.
client to rock potential side effects adequate airway regulating the temperature of prescribed. 10. Encourage physical and
back and of medication  Promote bath water and avoiding 12. Instruct the occupational therapy
forth to regimen need to be measures to heating pads. client to avoid exercises to help the patient
initiate taught, monitored and enhance gas 14. Instruct the client in stress, infection, regain strength during
movement. adjusted to the exchange, such safety measures related to fatigue, and over- rehabilitation phase.
10. Instruct desired patient as oxygen motor loss, such as avoiding the counter 11. Provide assistive devices
the client to response. therapy and the use of scatter rugs and medications. as needed (cane or
wear low- 5. Regular moderate ventilator using assistive devices. 13. Instruct the wheelchair) to maximize
heeled shoes. exercise can reduce assistance. 15. Instruct the client in the client to wear a independence and activity.
11. Encourage stiffness and tremors.  Promote self-administration of Medic-Alert 12. If verbal communication
the client to 6. As the disease measures to prescribed medications. bracelet. is possible, discuss the
lift feet when progresses, the prevent 16. Provide information about 14. Inform the patient’s fears and
walking and patient and family respiratory the National Multiple client about concerns.
avoid will require more infection Sclerosis Society. services from the 13. Provide choices in care
prolonged assistance with Myasthenia to give the patient a sense
sitting. activities of daily Gravis of control.
12. Provide a living, emotional Foundation. 14. Teach patient about
 firm support, and potential breathing exercises or use
mattress, and financial concerns. of an incentive spirometer
position the to reestablish normal
client prone, breathing patterns.
without a 15. Instruct patient to wear
pillow, to good supportive and
facilitate protective shoes while out
proper of bed to prevent injuries
posture. due to weakness and
13. Instruct in paresthesia.
proper 16. Instruct patient to check
posture by feet routinely for injuries
teaching the because trauma may go
client to hold unnoticed due to sensory
the hands changes.
behind the 17. Urge the patient to
back to keep maintain normal weight
the spine and because additional weight
neck erect. will further stress monitor
14. Promote function.
physical 18. Encourage scheduled
therapy and rest periods to avoid
rehabilitation fatigue.
.
15. Administe
r
anticholinerg
ic
medications
as prescribed
to treat
tremors and
rigidity and
to inhibit the
action of
acetylcholine
 .
16. Administe
r
antiparkinson
ian
medications
to increase
the level of
dopamine in
the CNS.
17. Instruct
the client to
avoid foods
high in
vitamin B6
because they
block the
effects of
antiparkinson
ian
medications.
18. Instruct
the client to
avoid
monoamine
oxidase
inhibitors
because they
will
precipitate
hypertensive
crisis.
occurs 10-20 yrs after inset 3 yrs after onset of dse may take only a few hours to
DEATH
of dse reach the most severe sxs