There are extensive number of neurodegenerative diseases but i have briefly discuss about the eight disease conditions. Hopefully it will be helpful to prospect learner. Good Luck

1. Neurodegenerative
disorders
Presented by
Tenzin Dolma

3. Dementia
– Refers to the loss of memory, reasoning, judgment and
language to such an extent that it interferes with everyday life.
– Cognition is the act or process of thinking, perceiving and
learning.

4. – Cognitive activities that become impaired in dementia include
decision-making, judgment, memory, orientation, thinking,
reasoning and verbal communication. A client with dementia may
undergo behavioral and personality changes as well

5. Alzheimer’s disease

6. Alzheimer’s disease
– Alzheimer’s disease is the most common form of dementia
among people 65 years of age and older.
– Dementia is intellectual deterioration severe enough to interfere
with occupational or social performance.
– Progressive decline in two or more areas of cognition, usually
memory and language, calculation, perception, constructional
praxis, judgment, abstraction, or personality.

7. Lewy body dementia
Is the second most common type of progressive dementia after Alzheimer's
disease dementia. Protein deposits, called Lewy bodies, develop in nerve cells in
the brain regions involved in thinking, memory and movement (motor control).

8. Pick’s Disease:
– Pick’s disease is marked by Pick Bodies, rounded,
microscopic structures found within affected
cells.
– Neurons swell, taking on a “ballooned”
appearance. Neither of the changes appears in
Alzheimer’s disease
– Pick’s disease is usually sharply confined to the
front parts of the brain, particularly the frontal
and anterior temporal lobes.

9. Alzheimer’s disease
– Increasing age
– Genetic factor linked to AD. At lease five chromosomes
(1,12,14,19,21) are involved in some forms of familial AD. Four
genetically link have also been identified as contributing to AD,
including amyloid precursor gene, the presenilin 1 gene,
presenilin 2 gene, and the apolipoprotein E gene

10. PATHOPHYSIOLOGY:
– Communication
– Metabolism
– Repair

11. – The changes are noted in beta-amyloid plaques and neurofibrillary
tangles
– Plaque is a cluster of beta-amyloid, a protein fragment snipped
from a larger protein called amyloid precursor protein

12. – Plaques have been described as dense, mostly insoluble
deposits of protein and cellular material outside and around
the neuron
– Plaques develop in the hippocampus. Degenerating nerve
terminals, both dendritic and axonal, contain amyloid protein.

13. – Healthy neurons have an internal support structure called
microtubules.
– These tubules serve as tract to guide nutrients to the end of the
axon and back. The tubule is stable due to a protein called tau.
– In AD tau is changed chemically and becomes tangled.
– Once tangled, the tubules degenerate and so do the cells they
support.

15. – The destruction leads to memory failure, personality changes
and problems carrying out activities of daily living (ADL)
– Gross brain changes evident in client with AD include
thickening of leptomeninges, shrunken gyri, widened sulci,
enlarged ventricles, hippocampal shrinkage, and generalized
atrophy.

16. – In addition to structural changes, neurotransmitter
changes, for example acetylcholine,
– Acetylcholine decreases because there is a decline in
cholinergic neurons in the basal nucleus that leads to
loss of choline acetyltransferase in the neocortex and
hippocampus

17. CLINICAL MANIFESTATIONS:
– Preclinical Alzheimer’s disease
– Begins near hippocampus, a structure essential to the
formation of both short and long term memories
affected lead to memory loss

18. Mild Alzheimer’s disease:
– Memory disturbance
– Poor judgments and problem solving skills
– Become careless at work, habits and household chores
– Confused about whereabouts and begin to get lost easily

19. – Routine activities such as bill paying and other daily tasks
take longer
– Do well in familiar routines but lack the ability to adapt
to new challenges
– Become irritable, suspicious, agitation, apathy dysphoria

20. Moderate Alzheimer’s Disease:
– Language disturbance
– Circumlocution (talking around a subject rather than about it
directly
– Paraphasia (words used in the wrong content)
– Palilalia (repeat words and phrases just spoken to themselves)
– Echolalia repeat words spoken by others)

21. – Motor disturbance is characterized by using daily objects such as toothbrush,
comb, razor and utensils
– Apraxia combined with forgetfulness
– Hyperorality (desire to take everything into mouth to suck)
– Swallowing difficulty
– Depression and irritability, delusion, psychosis
– Wandering at night

22. Severe Alzheimer’s Disease:
– Cannot recognize family or friends
– Do not communicate
– Voluntary movement is minimal
– Limbs become rigid with flexor posturing
– Aspiration and aspiration pneumonia is frequent

23. PARKINSON’S DISEASE:

24. PARKINSON’S DISEASE:
– Is a chronic, progressive, neurologic disorder that results from the loss
of the neurotransmitter dopamine in a group of structures that control
movements.
– Degenerative changes are found in an area of the brain known as
substantia nigra which produces dopamine, a chemical substance that
enables people to move normally and smoothly.
– Once cell loss in the substantia nigra reaches 80%, manifestations
appear.

26. CLINICAL MANIFESTATION:
– Tremor at rest on one side (coarse pill rolling movement of the thumb against
fingers)
– Rigidity: increased tone, and stiff in the muscles at rest
– Bradykinesia: slow movement, fine movement become clumsy
– Flexed posture of the neck, trunk, and limbs
– Loss of postural reflexes
– Freezing movement
– Inability to perform ADL (bradykinesia
– Diffuse muscular pain

28. MEDICATIONS:
– Dopaminergic:
– e.g. Levodopa, carbidopa, amantadine
– They have major effect on the akinesia of Parkinson’s
disease, improving mobility while decreasing muscle
rigidity and tremor.

29. – Monoamine oxidase inhibitors: e.g. selegiline
It works by selectively inhibiting enzyme that inactivates dopamine in the
brain. It inhibits the enzyme system that breakdown and destroy dopamine.
– Dopamine agonists:
– e.g. Bromocriptine, Pergolide, Pramipexole, Ropinirole
– Act directly activating dopamine receptors in the brain. They are
frequently used in combination with levodopa therapy. They increase the
therapeutic effects of levodopa and reduce fluctuations in motor
symptoms.

30. Anticholinergics:
– e.g. Trihexyphenidyl, Benztropine, Biperiden, Cycrimine,
Procyclidine, Chlorphenoxamine
– Effective in Parkinson’s disease because they block the
excitatory action of the neurotransmitter acetylcholine.
These medication ease drooling, tremors and rigidity.

31. CREUTZFELDT-JAKOB DISEASE:
– Fatal brain disease that produces progressive dementia,
myoclonus and distinctive electroencephalographic (EEG) changes.
– CJD is a unique disease that apparently can arise from two
separate mechanisms: genetic and infectious. People with the
genetic form have a mutated gene.

33. CLINICAL MANIFESTATIONS:
– Vague psychiatric or behavior changes
– Weight loss
– Anorexia
– Insomnia
– Malaise
– Dizziness for a periods of weeks to months

34. IN Early Stages:
– Progressive memory loss
– Visual impairment
– Dysphagia
– Within a few weeks or months, a relentlessly progressive dementia
develops and marked deterioration is noted from week to week
– Deterioration is rapid with 90% of clients dying within 1 year

35. DIAGNOSTIC EVALUATION:
– WHO diagnostic criteria for CJD include progressive dementia
and the presence of at least two of the following
– Myoclonus
– Extraocular or cerebellar disturbance
– Pyramidal/extrapyramidal dysfunction, and
– Akinetic mutism
– EEG typical for CJD
– Positive 14-3-3 assay for cerebrospinal fluid

36. HUNTINGTON DISEASE:

37. HUNTINGTON DISEASE:
– Is also known as Huntington’s Chorea, is an autosomal-
dominant degenerative neurologic disease. It is
characterized by
– abnormal movements,
– intellectual decline, and
– emotional disturbance.

38. RISK FACTOR
– Begin in the 30s and 40s
– Relentlessly progressive, leading to disability and death within 15 to 20 years.
– Death usually results from respiratory tract complications caused by aspiration,
– The disease is autosomal dominant; offspring of an affected person have a 50%
chance of inheriting the disease. Because HD doesn’t skip generations.
– Those who have not inherited the disease will not pass it on to their offspring. The
abnormal gene has been isolated on chromosome 4.

39. PATHOPHYSIOLOGY:
– Degeneration of Striatum (Caudate and Putamen) in the basal
ganglia
– Subtle changes occur in the cortex and cerebellum,
– Loss of neurons and an increased number of glial cells (gliosis)
– The degeneration of the caudate nucleus leads to a reduction in
several neurotransmitters, including gamma-aminobutyric acid,
Ach, substance P and metenkephalin and their synthetic enzymes

40. CLINICAL MANIFESTATION:
– Emotional disturbances
– Mental deterioration precedes the abnormal movements
– Person may become negative, suspicious and irritable
– Depression and psychosis
– Temper outburst and sexual promiscuity
– Mood swings

41. – Cognitive decline e
– Eventually dementia, incontinent and completely unable to provide
self-care
– Restless or fidgety
– Person may be aware of these movements and try to mask them by
making them seem to be parts of intentional movements such as head
scratching, or leg crossing.
– As the disease progresses, the rapid, jerky choreiform movements
become more pronounced and involve all muscles.

42. DIAGNOSTIC EVALUATION:
– The diagnosis of HD is made on the basis of clinical
manifestations and family history because there is no
specific diagnostic test for the disease itself.
– CT or MRI imaging of the brain may show atrophy of the
head of the caudate

43. MEDICAL MANAGEMENT:
– Antipsychotics agents specifically phenothiazine and
butyrophenones, they block dopamine receptors in brain
– Antidepressant are prescribed in early stage of the
disease; however, the medications are not substitute for
intense follow-up

45. MULTIPLE SCLEROSIS:
– Is a chronic demyelinating disease that affects the myelin sheath of
neurons in the central nervous system (CNS). The myelin sheath is
essential for normal conduction of nerve impulses.
– Patches of myelin deteriorate at irregular intervals along the nerve
axon, causing slow of nerve conduction.
– Axonal destruction also occurs in MS.

46. RISK FACTOR
– The onset of MS usually occurs between 20 and 40 years
of age, and
– It affects women twice as often as men.
– Whites are affected more often than Hispanics, blacks or
Asians.
– The disease is more prevalent in the colder climates of
North America and Europe.

47. ETIOLOGY:
– Immunogenic-viral disease, probably with the Epstein-Barr virus
– Multiple Genes on the human leukocyte antigen (HLA) gene
complex on chromosome 6
– Varity of precipitating factor precede the onset such as infection,
physical injury, emotional stress, pregnancy, and fatigue.
– Pregnancy related exacerbation occur 3 months postpartum

48. PATHOPHYSIOLOGY:
– Myelin is a highly conductive fatty material that surrounds the axon
and speeds conduction of nerve impulses along axon
– Autoimmune process and infectious agents have been implicated in
pathogenesis of multiple sclerosis
– Activated T cells, recognize self-antigens expressed in the CNS
– Macrophages enter the brain from the peripheral circulation and
initiate the inflammation

49. – Production of inflammatory cytokines and reactive oxygen species,
– activated T lymphocytes and microglia/macrophages cause the demyelination and
destruction of oligodendrocytes
– Plaques from along the myelin sheath, eventually causing scaring and destruction
– When edema and inflammation subside, some myelination occurs but is often
incomplete.
– Although plaques occur anywhere in the white mater of the CNS, the areas most
commonly involved are optic nerves, cerebrum and cervical spinal cord.

50. CLINICAL MANIFESTATIONS:
– Weakness or tingling sensations (paresthesia) of one or more
extremities caused by involvement of the cerebrum or spinal cord
– Vision loss from optic neuritis
– Incoordination that is due to cerebellar involvement
– Bowel and bladder dysfunction such as hesitancy, frequency, loss of
sensation, incontinence, and retention- as a result of spinal cord
involvement

51. – Constipation result from one or more of the following factors; spinal
cord lesion, immobility, dehydration, medications, and nutritional
deficiencies
– Fatigue and spasticity
– Depression occurs in clients but it is not clear whether depression is a
reaction to disability or a function of the disease itself.
– Euphoria, emotional instability or apathy

52. DIAGNOSTIC EVALUATION:
– Cerebrospinal fluid (CSF) evaluation for the presence of oligoclonal
banding
– Evoked potentials of the optic pathways and auditory system to
assess the presence of slowed nerve conduction
– MRI of the brain and spinal cord to determine the presence of MS
plaques
– Positron Emission tomography (PET) measures brain activity.

53. MEDICATION:
– Combination of adrenal corticosteroid hormone (ACTH) and
glucocorticoids is used to decrease inflammation and suppress the
immune system.
– Immunosuppressive agents, including azathioprine and
cyclophosphamide
– Immunomodulators: Interferon alpha, beta and glatiramer acetate
used to reduce exacerbations in clients with relapse remitting.

54. – Anticholinergics are administered for bladder spasticity
– Muscle relaxants to relieve muscle spasms. Such as
baclofen, dantrolene and diazepam

56. GUILLAIN-BARRE
SYNDROME
– Guillain-Barre syndrome (GBS) is an inflammatory disease of unknown
origin that involves degeneration of the myelin sheath of peripheral
nerves.
– GBS has become the most common cause of acute generalized
paralysis, which affects between 1 and 4 per 100,000 of the population
annually throughout the world.
– In one half to two thirds of cases, an upper respiratory or
gastrointestinal tract infection precedes the onset of the syndrome by
1 to 4 weeks.

57. ETIOLOGY:
– Many organisms have been suspected including
cytomegalovirus and Epstein-Barr virus, Campylobacter jejuni
– Gram negative rod is found in poultry, pets, raw milk and
contaminated water
– Association between HIV and GBS has also been reported

58. CLINICAL MANIFESTATIONS:
– Ascending weakness, beginning in the lower extremities,
weakness evolves over hours to days, with maximal
deficit by 4 weeks
– Paresthesia’s (tingling sensation) in the limbs

59. INITIAL PHASE
– Deep aching muscle pain in shoulder girdle and thigh
– The two most dangerous features of the disease are
respiratory muscle weakness and autonomic neuropathy
involving both the sympathetic and parasympathetic
systems.

60. LATTER FEATURES
– Orthostatic hypotension
– Hypertension
– Pupillary disturbances,
– Sweating dysfunction
– Cardiac dysrhythmias
– Paralytic ileus
– Urinary retention

61. The third Phase is recovery
phase:
– Improvement and recovery occur with demyelination.
– It occurs in descending pattern. The function lost last are
thus the first to be regained.
– Recovery is usually maximal at 6 months, although severe
cases may take up to 2 years for maximal recovery.
– Fortunately, 85% to 90% of clients with GBS recover
completely.

62. DIAGNOSTIC EVALUATION:
– History and physical examination
– CSF examination: CSF contains increased protein,
with few or no white blood cells.
– Nerve conduction velocity is slowed, although it
may be normal in the early sage of illness.

63. MEDICAL MANAGEMENT:
– Plasma exchange or IV immunoglobulin which hasten
recovery of Guillain Barre Syndrome
– Removal of circulating antibodies or other humoral
myelinotoxic or immunopathogenic factors

64. MYASTHENIA GRAVIS:

65. MYASTHENIA GRAVIS:
– Is an autoimmune disease that presents as muscular weakness and
fatigue that worsens with exercise and improves with rest. The
manifestations result from a loss of ACh receptors in the
postsynaptic neurons of the neuromuscular junction.

66. ETIOLOGY:
– The cause of myasthenia Gravis is unknown,
– But 80% of people with the generalized form of the disease have
elevated titers of antibodies to the Ach receptor in their serum.
– Myasthenia Gravis occurs at any age, but there are two peaks of
onset. In early onset, at age 20 to 30 years
– Women are more often affected than men.
– In late-onset Myasthenia Gravis: after age 50, men are more often
affected.

67. CLINICAL
MANIFESTATION:
– Increasing weakness and muscle contraction
– Ptosis (drooping of the upper eyelid) or diplopia
(double vision)
– Ptosis is due to weakness of the levator
palperbrae muscles of the eye
– Diplopia is a result of weakness or fatigue of the
extraocular muscles
– Weakness of the orbicularis oculi muscles

68. – Weakness of the facial and levator palpebrae muscles
produces an expressionless face, with droopy eyelids,
– Tendency for the mouth to hang open
– Smile often turns into a snarl because of the weakness
– Dysphagia and nasal quality to speech

69. DIAGNOSTIC EVALUATION:
– Endrophonium (Tensilon) is a short-acting drug that is given
intravenously (Tensilon Test). A test dose of 2 mg is injected first.
If no untoward reaction occurs (such as increased weakness,
change in heart rate or rhythm, nausea, or abdominal cramps),
the remaining 8 mg is injected. The client is then observed for
objective manifestations of improvement in muscle strength. The
effect is transitory, wearing off after 3 to 5 minutes.

71. – Another drug, neostigmine methylsulfate
(Prostigmin), may be used because of its longer
duration of effect on muscle strength (1 to 2
hours), which allows better analysis of its effect.
– Electromyography (EMG) helps confirm the
diagnosis. Repetitive stimulation of the nerve
with recording from the involved muscle shows a
characteristic decrementing response of the
muscle action potential.

72. OUTCOME MANAGEMENT:
– No cure for Myasthenia Gravis. Pharmacologic intervention
consists of two groups of medications
– Short-acting anticholinesterase
– Corticosteroids

73. – The most effective anticholinesterase drugs are pyridostigmine and
neostigmine. The goal is to achieve the maximum benefit (muscle
strength and endurance) with the fewest side effects.
– Corticosteroids (usually prednisone) are directed toward reducing
the levels of serum Ach receptor antibodies. Corticosteroids may
temporarily worsen manifestations; however, this is followed by
gradual improvement in muscle strength.

74. PLASMAPHERESIS:
– Adjuvant therapy for clients with refractory Myasthenia Gravis. It is a
process by which plasma is separated from formed elements of blood.
The plasma is discarded and the packed red blood cells are joined with
albumin, normal saline, and electrolytes and returned to the client.
– The purpose is to remove plasma proteins containing antibodies that are
believed to cause Myasthenia Gravis.

75. – Plasmapheresis may produce transient improvement in clients
who have actual or pending respiratory failure.
– Three to five treatments given once daily over 5 to 7 days are
required

77. COMPLICATION
– The two major complications of Myasthenia Gravis
A. Myasthenia Crisis and
B. Cholinergic Crisis.

78. Myasthenia Crisis
– Client with moderate or severe generalized Myasthenia
Gravis, especially those who have difficulty swallowing or
breathing, may experience a sudden worsening of their
condition.
– Usually precipitated by an intercurrent infection or
sudden withdrawal of anticholinesterase drugs, but it
may occur spontaneously.

79. Clinical Manifestation:
– Sudden marked rise in blood pressure because of hypoxia
– Increased heart rate
– Severe respiratory distress and cyanosis
– Absent cough and swallow reflexes
– Increased secretion, increased diaphoresis and increased lacrimation
– Restlessness, dysarthria
– Bowel and bladder incontinence

80. INTERVENTION:
– Increased doses of cholinergic drugs as long as the client
responds positively to edrophonium treatment
– Possible mechanical ventilation if respiratory muscle
paralysis is acute

81. CHOLINERGIC CRISIS:
– Occurs as a result of overmedication.
– Muscarinic effect of a toxic level of anticholinesterase
medication causes abdominal cramps, diarrhea, and
excessive pulmonary secretions.
– The nicotinic effect paradoxically worsens weakness and
can cause bronchial spasm

82. CLINICAL MANIFESTATIONS:
– Weakness with difficulty swallowing, chewing, speaking and
breathing
– Apprehension, nausea and vomiting
– Abdominal cramps and diarrhea
– Increased secretions and saliva
– Sweating, lacrimation, fasciculation, and blurred vision

83. AMYOTROPHIC LATERAL
SCLEROSIS:

84. AMYOTROPHIC LATERAL
SCLEROSIS:
– Amyotrophic lateral sclerosis (ALS) is the most common of the motor
neuron disease. It is an age-dependent, fatal paralytic disorder also
known as Charcot’s disease and Lou Gehrig’s disease.
– Onset is usually in middle age. Men are affected more often than
women
– Amyotrophic lateral sclerosis involves degeneration of both the anterior
horn cells and lower motor neuron.

86. PATHOPHYSIOLOGY:
– Degeneration and demyelination of both upper and lower motor neurons
in the anterior horn of the spinal cord, brainstem and cerebral cortex
– Axonal degeneration, demyelination, glial proliferation and scarring along
the corticospinal tract
– Surviving motor neurons sprout new branches to reinnervate affected
muscle fibres, preventing muscle strength
– When more than half of lower motor neurons are affected, reinnervation
fails and weakness is evidenced.

87. CLINICAL MANIFESTATION:
– Lower motor neuron clinical manifestations are:
– Weakness,
– Atrophy,
– Cramps and
– Fasciculation (irregular twitching of muscle fibers or
bundles.

88. Upper motor neuron
manifestations include
– Spasticity and hyperreflexia.
– Involvement of corticobulbar tracts causes dysphagia and dysarthria
(slurred speech)
– Suboptimal caloric and fluid intake and a worsening of muscle atrophy,
weakness, and fatigue.
– The sensory system is not involved, cognition is not affected
– Death usually results from pneumonia caused by respiratory compromise
within 2-5 years.

89. MEDICATION:
– Riluzole, an antiglutamate, is the first medication
developed to treat ALS.
– It inhibits the presynaptic release of glutamic acid in the
CNS and protects neuron against the excitotoxicity of
glutamic acid.

90. NURSING MANAGEMENT
– Anxiety related to threat to self concept as evidenced by restless,
insomnia and distress
– Ineffective airway clearance related to respiratory muscle weakness as
evidenced by abnormal lung sounds or patient complaint of breathing
difficulty
– Impaired physical mobility related to neuromuscular impairment as
evidenced by impaired coordination, limited ROM.

91. Anxiety related to threat to self concept
as evidenced by restless, insomnia and
distress
– Assess level of anxiety, (mild, severe). Note signs and symptoms, including nonverbal
communication
– Assess prior coping patterns and evaluate support resources available to patient
– Display confident, calm manner and tolerant, understanding attitude
– Encourage ventilation of feelings and concerns about dependency:
– Listen carefully and give unhurried attentive appearance; be awaer of defense
mechanisms used.
– Suggest use of supportive measures (e.g. medications, clergy, social services, support
groups)

92. – Provide accurate information about disease, medications, test or
procedures, and self care
– Understand that patient may have inappropriate behaviours (e.g.
outburst of laughing/crying)
– Try to direct patient to positive aspects of living to the maximum for
the present.
– Reinforce the things the patient can do versus what he or she cannot

93. Ineffective airway clearance related to respiratory muscle
weakness as evidenced by abnormal lung sounds or
patient complaint of breathing difficulty
– Assess lung sounds and respiratory movement as indicated
– Observe for signs of respiratory distress (e.g., increased respiratory rate,
restlessness, rales, rhonchi)
– Evaluate cough reflexes
– Observe for signs or symptoms of infection (change in sputum color, amount,
character; increased white blood cell count)
– Elevate head of bed and change position every 2 hours and as needed

94. – Encourage deep breathing exercises and use of incentive spirometry
– Encourage fluid intake to 2000ml daily within level of cardiac reserve.
Encourage warm liquids
– Suction the patient if needed.
– Implement transcutaneous nerve stimulation of the diaphragm as
indicated

95. Physical mobility related to neuromuscular
impairment as evidenced by impaired
coordination, limited ROM.
– Assess ROM, muscle strength, previous activity level, gait, coordination and
movement
– Assess patient’s current level of independence; self care ability
– Assess patient’s endurance in performing activities of daily living (ADL)
– Evaluate requirements for assistive device
– Demonstrate positioning the patient for optimum comfort, facilitation of
ventilation, and prevention of skin breakdown.
– Maintain exercise program: active or passive ROM

96. – Alternate periods of activity with adequate rest periods
– Encourage patient’s and significant others involvement in care; help them
learn ways to manage problems of immobility
– Instruct in provision of safety measures as indicated by individual situation
– Encourage participation in activities and occupational or recreational therapy
– Instruct in provision of skin care; wash and dry skin well, use gentle massage
and lotion