Degenerative neurologic disorders

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 Learning objectives

• At the end of this session you will be able to:

– Define degenerative neurological disease
– List degenerative neurological disease
– Describe degenerative neurological disease
• Parkinson’s disease
• Alzheimer's diseases
– Apply medical and nursing knowledge to manage
degenerative neurological disease
• Parkinson’s disease

• Alzheimer's diseases 2
 Neurotransmitters(NT)
•Communicate messages from one neuron to another
/to a specific target tissue.

•Manufactured and stored in synaptic vesicles

•Potentiates, terminates, or modulates a specific action,

and it can either excite or inhibit activity of the target
cell.
•Once released, enzymes either destroy the NT or
reabsorb it into the cell for future use. 3
 NT….
• All brain functions are modulated through
neurotransmitter receptor site activity, including
memory and other cognitive processes

• Many neurologic disorders are due, at least in part, to

an imbalance in NT
E.g. Parkinson's disease – decreased dopamine,
 Alzheimer’s disease – decreased acetylcholine
 Myasthenia gravis - acetylcholine binding to
muscle cells is impaired 4
 Degenerative neurologic disorders
• Disorders in central & peripheral nervous
system that leads to deterioration of normal
function in nervous system
• E.g.
– Parkinson’s disease
– Alzheimer’s disease
– Huntington disease-progressive involuntary
choreiform movement and dementia
– Amyotrophic lateral sclerosis (ALS)( Lou
Gehrig’s disease) - a loss of motor neurons in the
anterior horns of spinal cord and the motor nuclei5
of the lower brain stem.
 A. Parkinson’s disease
• PD: caused by the progressive impairment or
deterioration of neurons (nerve cells)
– Substantia nigra dopamine producing
– Dopamine communicate substantia nigra and
corpus striatum.
– This communication coordinates smooth and
balanced muscle movement.
– A lack of dopamine results in abnormal nerve
functioning, causing a loss in the ability to
control body movements.
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 Parkinson’s disease….
•Neurotransmitters acetylcholine (excitatory) and
dopamine (inhibitory), striatal neurons relay messages
to the higher motor centers that control and refine
motor movements.

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 Parkinson’s disease….
• Fourth most common neurodegenerative
disease,
• Men affected > women.
• Symptoms usually first appear in the fifth
decade of life;
• Two types :
– Degenerative/idiopathic - most common
– Secondary - a known or suspected cause.
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 Causes
• In most cases is unknown, research suggests several causative
factors, including:
– Genetics,

– Atherosclerosis,

– Excessive accumulation of oxygen free radicals,

– Viral infections,

– Head trauma,

– Chronic use of antipsychotic medications, and reserpine,

chlorpromazine, haloperidol & other antipsychotic that block D2

– Some environmental exposures (manganese dust, carbondisulfide, etc

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Pathophysiology….

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 CM

• Appear after 60% of the pigmented neurons are lost

& striatal dopamine level is < 80%.
• Has a gradual onset, and progress slowly over a
chronic, prolonged course
• Can Be :

1. Motor symptoms
– Vary from pt to pt

2. Non motor symptoms:

– Important as motor symptoms
– Affect pt quality of life 14
 CM…
1. Motor symptoms

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 CM…

1. Motor symptoms
– Cardinal /classic signs:
• Tremor- involuntary shaking of limbs
• Rigidity/ Snuffling gate
• Bradykinesia - abnormally slow/ paucity of
movements and most disabling symptom
• Postural instability/Flexed posture- poor
balance

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 Motor symptoms

Tremor
• A slow, unilateral resting tremor(80%)

– Affecting first distally involving the digits and wrist

where it may present with “pill –rolling” character.

• Tremor usually spreads proximally, ipsilateral

and occasionally to the leg, before crossing to
other side after a year or two.

• It may appear later in the lips, tongue, and jaw

but spears the head.
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 Tremor….
• Disappears with purposeful movement &
evident when the extremities are motionless
(sleep)
• Rhythmic, slow turning motion (pronation–
supination) of the forearm and the hand
and a motion of the thumb against the
fingers as if rolling a pill between the fingers

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 Motor symptoms …
Rigidity- muscle tone stiffness
• Resistance to passive limb movement.

– May cause the limb to move in jerky increments,

referred to as lead-pipe or cog-wheel movements
• Decrease in ROM

• Common in arms, legs, face, and posture

• Early in the disease, the patient may complain of

shoulder pain due to rigidity.
• Cause

– Discomfort and pain

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– Sever form result in stooped posture
 Motor symptoms…
Bradykinesia/ akinesia
• Overall slowing of active movement

– slowness of mov’t or reaction time

– Decreased spontaneous mov’t

• Most common features of PD- in all patient

• Take longer to complete activities and have difficulty

initiating movement, such as rising from a sitting
position or turning in bed
• Reduction in facial expression and non verbal
communication= masked face or poker face- makes
to seem no emotion 20
Bradykinesia…

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 Motor symptoms…

Postural instability- reduced balance

• Loss of ability to maintain upright posture

• Loss of postural reflexes & stands with head bent

forward & walks with a propulsive gait.

• Posture is caused by the forward flexion of neck, hips,

knees, and elbows.

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 Postural instability…

• Walk faster and faster, trying to move the feet forward

under the body’s center of gravity (shuffling gait).
• Difficulty in pivoting causes loss of balance (either
forward or backward)- patient at increased risk for
falls
• Most disabling motor symptoms
• Levodopa is less effective

• Assesses by pulling test

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 Other motor symptoms
• Gait disorders:
– Abnormal manner of walking:
– Lack of arm swing
– Arms flexed at elbow
– Dragging of one leg small shuffling
steps(smaller steps)
– Freezing =stick to flower
• Require extra effort to start walking –
prevent patient from getting out of bed or
eating

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Other motor symptoms …

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 Other motor symptoms…
• Dysarthria
– Speech disorder- quiet, mono pitch
(hypotonia),altered
• Swallowing difficulty
– Cause drooling and change in eating
• Micro graphia
– Small handwriting

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 2. Non motor symptoms
 Sleep disorders

– 6-9%
– Early symptom
– Fragment sleep
• Due to frequent urination b/c of muscle
contraction
• Excess day time sleep
• Drug S/E(dopamine)=sedation

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 2. Non motor symptoms…
 Decrease smelling
 Eventually lost- Anosmia
 Effect in basal ganglia often produces autonomic
symptoms:
– Excessive and uncontrolled sweating,
– Paroxysmal flushing,
– Orthostatic hypotension- may be drug S/E
– Gastric and urinary retention,
– Constipation
– Sexual dysfunction 28
 2. Non motor symptoms…
 Psychiatric changes include
– Depression, dementia (progressive mental
deterioration),
– Delirium
– Hallucinations
• Usually visual
• Insulting injuries
– Most psychiatric medication PD symptoms
• Pain (muscular pain)
– Muscle spasm or contraction
– Usually in one side of body –cause more
physician visit
– Cause stooped posture
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 Assessment and Diagnostic Findings
Clinically
• Patient’s history
• Presence of two of the four cardinal
manifestations:
– Tremor,
– Rigidity,
– Bradykinesia, and
– Postural changes.
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Medical Management

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 Medical Management….
A. Therapeutic Goal
• To improve ability for activities of daily life (only symptom
relief)
• Currently, no medical or surgical approaches in use to
prevent disease progression.

B. Treatment Strategy
– Restore balance between DA and ACh by activating DA
receptors or blocking ACh receptors

C. Overview of Drugs Employed-Two major categories

– dopaminergic drugs & anticholinergic drugs
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 Medical Management….
Pharmacologic Therapy
• Antiparkinsonian medications act by
 Increasing striatal dopaminergic activity;
 Reducing the excessive influence of excitatory
cholinergic neurons on the extrapyramidal
tract,
 Acting on neurotransmitter pathways other

than the dopaminergic pathway.

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 Medical Management….
• Levodopa (larodopa)
– Most effective agent and the mainstay of treatment.
– Converted to dopamine in the basal ganglia,
producing symptom relief.
– Available in three forms:
• Immediate-release,
• Orally disintegrating,
• Sustained-release tablets
– Beneficial effects of levodopa are most pronounced
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 Medical Management….

• Carbidopa: blocks systemic/peripheral conversion

of levodopa to dopamine, thereby decreasing the
undesirable systemic effects of levodopa such as
nausea and orthostatic hypotension.
Dose:
• Carbidopa/levodopa IR 25/100 mg Initial dose: t/2
tab PO TID, to maximum target dose of 3-6 25/100
mg tabs /day (i.e. 1 -2 tabs PO TID)
• Carbidopa/levodopa CR 50/200 mg tabs : dose
1tab BID or TID
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 Medical Management….
Surgical management
• In disabling tremor, rigidity, or severe
levodopa-induced dyskinesia, surgery may be
considered.
• Provides symptom relief in selected patients,

• It has not been shown to alter the course of

the disease or to produce permanent

improvement.
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 Nursing Diagnoses

• Impaired physical mobility related to muscle

rigidity and motor weakness
• Self-care deficits (feeding, dressing, hygiene, and
toileting) related to tremor and motor disturbance
• Constipation related to medication and reduced
activity
• Imbalanced nutrition, less than body
requirements, related to tremor, slowness in eating,
difficulty in chewing and swallowing 38
 Nursing Diagnoses…

• Impaired verbal communication related to decreased

speech volume, slowness of speech, inability to move
facial muscles
• Ineffective coping related to depression and
dysfunction due to disease progression
• Sleep pattern disturbances,

• Deficient knowledge,

• Risk for injury,

• Risk for activity intolerance,

• Disturbed thought processes 39

 Nursing Interventions

• Improving mobility
• Enhancing self-care activities
• Improving bowel elimination
• Improving nutrition
• Enhancing swallowing
• Encouraging the use of assistive devices
• Improving communication
• Supporting coping abilities
• Promoting home and community-based care

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2. Alzheimer’s disease/ senile dementia

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 Alzheimer’s disease…..
• Chronic, progressive, irreversible, & degenerative with
profound effects on memory, cognition, and ability for
self-care.
• It is a regressive brain disorder

• Called memory thief

• One of the most feared disorders of b/c of its

catastrophic consequences for the patient, family, and
caregivers,
• Occur in 40 years of age & uncommon before 65 yrs.
• Increases dramatically with increasing age, but it is not
a normal part of aging. 42
 cause
Risk factor
• Increasing age,
• Environmental,
• Dietary, and
• Inflammatory factors.
• Genetics,
• neurotransmitter changes,
• Vascular abnormalities,
• Stress hormones,
• Circadian changes,
• Head Trauma,
• Seizure disorders. 43
 Classification

Two types:
• Familial or early onset AD
– <10% &
– Associated with genetic mutations
– Occurs in middle- aged adults
• Sporadic or late-onset AD.

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 Pathophysiology
• Neuropathologic and biochemical changes

– Neurofibrillary tangles (tangled masses of nonfunctioning

neurons) and senile or neuritic plaques (deposits of
amyloid protein, part of a larger protein called amyloid
precursor protein in the brain)

• Neuronal damage :
– Cerebral cortex and results in decreased brain size.

– Neurotransmitter acetylcholine are principally affected

– Enzyme active in producing acetylcholine, which is

specifically involved in memory processing, is decreased.
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 Clinical Manifestations
• Early stages
– Forgetfulness and subtle memory loss
– Small difficulties in work or social activities
• Progression of AD
– Forgetfulness in many daily actions; lose ability to
recognize familiar faces, places, and objects, and lost
in a familiar environment.
– Repeat the same stories b/c they forget that they
have already told .
– Conversation becomes difficult,
– word-finding difficulties
– concepts formulation and abstractly thinking
disappears
– unable to recognize the consequences of their actions
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 Clinical Manifestations
• Personality changes - depressed, suspicious,
paranoid, hostile,
• Speaking skills deteriorate to nonsense syllables,
agitation and physical activity increase, and
patients may wander at night.
• Terminal stage, immobility and require total care,
may last months or years.
• Death occurs as a result of complications such as
pneumonia, malnutrition, or dehydration. 47
 Assessment and Diagnostic Findings
• Clinical diagnosis-90%
• Diagnostic tests
– CBC,
– Chemistry profile, and
– Vitamin B12 and thyroid hormone levels
– Electroencephalography,
– Computed tomography (CT),
– Magnetic resonance imaging (MRI),
– Cerebrospinal fluid 48
 Medical Management
Goal :
• Manage the cognitive and behavioral
symptoms.
• No cure and no way to slow the progression of
the disease.
• Four US. Food and drug administration
(FDA)–approved medications are available to
treat AD symptoms; however, none of these
agents stops the progression of the disease. 49
 Medical Management……

• Cholinesterase inhibitors
– Donepezil hydrochloride (aricept),
– Rivastigmine tartrate (exelon),
– Galantamine hydrobromide (razadyne
[formerly known as reminyl]), and
– Tacrine (cognex) enhance acetylcholine

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 Nursing Management
• Supporting Cognitive Function.

• Promoting Physical Safety

• Promoting Independence in Self-Care Activities

• Reducing Anxiety and Agitation

• Improving Communication

• Providing for Socialization and Intimacy Needs

• Promoting Adequate Nutrition

• Promoting Balanced Activity and Rest

• Supporting Home and Community-Based Care 51