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Clinical Conditions > Progressive Neurodegenerative Conditions (PND) > Flashcards

Progressive Neurodegenerative Conditions (PND) Flashcards

1
Q

Progressive neurogenerative disorders (PND)

A
  • progressive conditions that affect the brain and spinal cord’s nerve cells, or neurons
  • characterized by the gradual loss of neurons
  • leads to a decline in brain functions like memory, movement, and cognition

Common neurogenerative disorders:
- Alzheimer’s
- Multiple Sclerosis (MS)
- Parkinson’s Disease (PD)
- Amyotrophic Lateral Sclerosis (ALS)

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2
Q

Multiple sclerosis

A
  • a debilitating immunological and neurodegenerative disease
  • genetically susceptible person
  • autoimmune disease
  • causes demyelination = the immune system attacks the myelin sheath that surrounds the brain, spinal cord, and optic nerve
  • chronic inflammation and diffuse demyelination in white matter and grey matter and the axons
  • demyelination of the neurons in the CNS results in scar tissue formation or plaques that reduce the axons’ ability to conduct impulses
  • relapsing and progressive MS
  • location of demyelination varies from person to person = various symptoms
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3
Q

Importance of myelin (MS)

A
  • protein and fatty substance that insulates and protects nerve cells, allowing electrical impulses to travel faster and more efficiently
  • required to maintain stability, function, and normal lifespan of the neuron
  • it damaged, these impulses slow down (doesn’t get to the muscles)
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4
Q

What occurs during MS

A
  • disseminated patches of demyelination on nerves
  • replaced by sclerotic lesions or plaques
  • slowly progressive disease
  • degeneration becomes irreversible and function is lost permanently
  • with each reoccurrence, additional areas of the CNS are involved
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5
Q

Incidence and prevalence of MS

A
  • most common non traumatic neurological disorder among adults under 40
  • currently thought to affect 730,000 people in the U.S.
  • unevenly distributed around the world
  • increased in countries with higher latitudes, where the sunlight is limited
  • differ by race and ethnicity
  • African descent generally lower risk than Caucasians
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6
Q

Stats in MS (slide 9)

A
  • 3 times more common in females than males
  • Germany = highest prevalence
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7
Q

Diagnostic tests for MS

A
  • not single test can confirm on MS diagnosis (what will be listed are a few test that can aid in diagnosing the disease)
  • MRI = uses a strong magnetic field and radio waves to assess water content in tissues, which can then be used to create a detailed image
  • evoked potential = used to measure the electrical activity of the brain in response to a stimulus by placing electrodes on specific parts of the body
  • spinal tab = a procedure in which a small sample of the cerebrospinal fluid is collected
  • vision tests = can help to assess optic neuritis and other eye-related complications
  • most common progressive neurodegenerative disease
  • usually diagnoses between 20-40
  • peak age of onset = 30
  • rarely seen in children or diagnosed over 60
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8
Q

Diagram of symptoms of MS (slide 11)

A
  • numbness, tingling
  • vision problems
  • walking difficulty
  • cognitive dysfunction
  • depression
  • fatigue
  • muscle spasms
  • weakness
  • dizziness
  • pain
  • bladder dysfunction
  • bowel dysfunction

*exercise can prolong your life with MS

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9
Q

Signs and symptoms of MS

A

Muscle symptoms:
- weakness in legs
- progressive weakness and paralysis extending to the upper limbs
- spasticity
- ataxia
- facial weakness = Bell’s palsy
Visual symptoms:
- scotoma
- visual blurring
- diplopia
- unilateral optic neuritis
Sensory symptoms:
- paresthesia
- facial pain or pain from muscle spasms
Speech and swelling symptoms:
- dysarthria
- trouble chewing and swallowing
Other symptoms:
- bowel and bladder dysfunction
- sexual dysfunction
- chronic fatigue
- depression or euphoria may develop
- decreased attention span, poor judgment, and memory loss
- difficulty reasoning and solving problems
Cognition symptoms:
- short-term memory deficit
- problems with abstract conceptualization
- attention deficit
- slowed information processing

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10
Q

Spasticity

A
  • spontaneous and movement induced muscle spasms
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11
Q

Scotoma

A
  • spot in the visual field (usually the first symptom)
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12
Q

Diplopia

A
  • double vision
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13
Q

Unilateral optic neuritis

A
  • inflammation in the optic nerve on one side
  • loss of color vision, pain when moving eye, loss of vision in one eye, change in pupillary reaction
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14
Q

Paresthesia

A
  • areas of numbness, burning, and tingling
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15
Q

Dysarthria

A
  • slurred and difficulty to understand speech
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16
Q

Chronic fatigue

A
  • often worse in the afternoon (most common complaint and can be the most debilitating)
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17
Q

Lesion at cerebrum (MS)

A

Impairments:
- cognitive
- hemisensory and motor
- affective
Sign and symptoms:
- impaired attention, memory, and executive functions
- upper motor neuron signs
- depression or euphoria

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18
Q

Lesion on optic nerve (MS)

A

Impairments:
- loss of vision
Sign and symptoms:
- scotoma, reduced visual acuity, color blindness, and ocular pain

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19
Q

Lesion on cerebellum (MS)

A

Impairments:
- tremor
- dysarthria
- incoordination and poor balance
Sign and symptoms:
- postural and limb instability during movement
- poor articulation
- clumsiness, ataxic movements, and falls

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20
Q

Lesion on brainstem (MS)

A

Impairments:
- loss of vision
- vertigo
- dysarthria
- pseudobulbar palsy
Sign and symptoms:
- nystagmus, reduced oculomotor control
- decreased balance
- impaired swallowing
- impaired speech and emotional lability

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21
Q

Lesion on spinal cord (MS)

A

Impairments:
- impaired muscle tone
- bowel and bladder dysfunction
- erectile dysfunction
Sign and symptoms:
- spasticity, weakness, stiffness, and painful spasms
- incontinence and constipation
- impotence

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22
Q

Lesion on other site (MS)

A

Impairments:
- fatigue
- pain
- desregulation and temperature
Sign and symptoms:
- unexplained lethargy, exercise intolerance
- complains of pain in various body regions
- hypersensitivity to heat

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23
Q

Types of multiple sclerosis

A
  • benign (clinically isolated)
  • relapsing-remitting MS (RRMS)
  • secondary progressive MS (SPMS)
  • primary-progressive MS (PPMS)
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24
Q

Benign (clinically isolated syndrome - MS)

A
  • one or two more episodes of neurological deficits
  • no residual impairments
  • the person’s chance of remaining symptoms free increases with each asymptomatic year
  • 5-10%

Key characteristics:
- minimal disability = patients with benign MS maintain a high level of function and experience few, if any, significant physical or cognitive impairments
- long period of stability = the disease shows little to no progression in terms of disability over a long period, often with infrequent relapses
- retrospective diagnosis = the label “benign MS” can only be applied after years of disease stability, typically around 10-15 years, making early prediction challenging

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25
Q

Relapsing-remitting MS (RRMS)

A
  • most common form of MS
  • 80-85%
  • periods of new or worsening symptoms (relapses) followed by periods of partial or complete recovery (remissions)
  • during remissions, symptoms may disappear entirely or improve significantly, but the disease does not progress between relapses
  • relapses = sudden flare-ups of symptoms that can last for days, weeks, or months (can include fatigue, numbness, vision problems, muscle weakness, difficulty walking, and cognitive changes)
  • remissions = periods of recovery where symptoms may subside or improve (no apparent disease progression during these times)
  • disease course = RRMS can transition into secondary MS (SPMS) over time, where the disease progresses more steadily with fewer or no remissions

*when the person gets more and more attacks, they don’t always recover to the baseline

26
Q

Secondary progressive MS (SPMS)

A
  • typically follows the relapsing-remitting form (RRMS)
  • disease progresses more steadily, with or without relapses, and the recovery during remission periods is less complete
  • symptoms gradually worsen and disability increases

Key features of SPMS:
- transition from RRMS = many people with RRMS eventually transition to SPMS (the exact time varies, but usually occurs 10-20 years after the initial RRMS diagnosis)
- progression = unlike RRMS, SPMS is characterized by a continuous progression of symptoms, with or without relapses (remissions become less common and when they do occur, recover is typically incomplete)
- symptoms = increased mobility issues, spasticity, worsening cognitive function, fatigue, and bladder or bowel problems
- disability = over time, individuals with SPMS often experience increasing disability, which can significantly impact their daily life and independence

27
Q

Primary-progressive MS (PPMS)

A
  • less common forms of MS
  • 10-15% of all MS cases
  • characterized by a gradual and continuous worsening of symptoms from the onset
  • no distinct relapses and remissions seen in other forms of MS

Key features of PPMS:
- progressive course = from the very beginning, PPMS involves a steady progression of symptoms (there may be occasional plateaus or minor improvements, but overall, the disease consistently advances)
- no relapses = unlike RRMS or SPMS, PPMS typically does not involve relapses or significant periods of remission (symptoms worsen gradually over time)
- symptoms = common symptoms include progressive muscle weakness, particularly in the legs, difficulty walking, spasticity, balance problems, fatigue, and sometimes cognitive changes
- onset age = PPMS usually has a later onset compared to RRMS, often being diagnosed in people in their 40s or 50s
- gender = unlike RRMS (which is more common in women, PPMS affects men and women almost equally

28
Q

Medication management of MS

A

Medications designed to reduce the frequency of relapses, delay the progression of disability, and decrease the number of new lesions seen on MRI:
- injections
- oral medications
- infusions
Oral medication for symptomatic treatments:
- spasticity
- pain
- fatigue
- bladder dysfunction
- cognitive dysfunction
- mobility

  • steroids are used to reduce severity and duration of relapses
  • deep brain stimulator = electrodes implanted in the brain to modulate abnormal neural activity
29
Q

Deep brain stimulation (MS)

A

1.) DBS leads = thin insulated wires are inserted into the brain through small holes in the skull
2.) extension wires = the wires are threaded under the skin and down the side of the head and neck, then connected to the battery pack
3.) implantable pulse generator (IPG) = this pacemaker-like device is implanted near the collar bone and sends pulses to targeted structures within the brain

30
Q

Occupational implications of MS

A
  • fatigue
  • mobility and balance issues
  • spasticity and muscle weakness
  • cognitive impairments
  • bladder and bowel dysfunction
  • speech and swallowing difficulties
  • emotional and psychological challenges
  • social participation
  • work and vocational challenges
31
Q

Parkinson’s Disease (PD)

A
  • a progressive neurological disorder that primarily affects movement (when you don’t have enough dopamine)
  • degeneration of dopamine-producing neurons in a part of the brain called substantia nigra
  • dopamine is a neurotransmitter crucial for coordinating smooth and balanced muscle movements
  • as the disease progresses, it leads to a variety of motor and non-motor symptoms that can significantly impact daily life and occupational performance
32
Q

Etiology of PD

A
  • unclear cause
  • potentially an interaction of genetic and environmental factors
  • familial PD (10% of the cases, genetic association)
  • sporadic PD

Environmental factors that are associated may be:
- dietary intake
- exposure to environmental elements
- history of head trauma
- Type II diabetes

33
Q

Incidence and prevalence of PD

A
  • 2nd most common neurodegenerative disorder in older adults (Alzeihmer’s = 1st)
  • worldwide = 1% of population over age 65
  • PD affects males slightly more than females (3-2 ratio)
  • primary risk factor = age
  • 1,000,000 people in the U.S. lives with PD
  • 90,000 people in the U.S. are diagnosed with PD every year
34
Q

Early warning signs of PD

A
  • tremor = slight shaking in hand, finger, or chin while at rest (pill tremor)
  • small handwriting = may include smaller letters and/or words crowded together
  • trouble moving = stiffness in limbs, body, or shoulders; feet feeling “stuck to the floor”
  • voice changes = voice may sound soft or hoarse
  • dizziness = feel dizzy or faint upon standing
  • loss of smell = inability to smell certain foods, such as bananas, pickles, and licorice
  • sleep problems = sudden movements during sleep, acting out dreams
  • constipation = difficulty moving bowels without straining
  • masked face = face looks angry, serious, or depressed even when happy (their skin is really tight that they cannot do a full smile anymore to raise their eyebrows)
  • stooping = change in posture when you stand, such as stooping or slouching
35
Q

Signs and symptoms of PD

A

Motor:
- bradykinesia
- tremors
- rigidity
- postural instability
- gait difficulties
- vocal
- pill rolling
- general symptoms
- lead pip rigidity
- cogswell rigidity
Nonmotor:
- fatigue
- muscle weakness/aching
- decreased spontaneous change in facial expression/masking
- cognitive impairments

36
Q

Bradykinesia

A
  • slow, rhythmic movements
  • difficulty initiating movement
37
Q

Tremors

A
  • usually occurs when at rest
  • fingers, hands, forearms, foot, mouth, chin
  • pill rolling tremors
  • tremors cease with voluntary movements and during sleep
38
Q

Rigidity

A
  • joint stiffness
  • can cause joint and muscle pain
  • lead pipe and cogwheel
39
Q

Postural instability

A
  • partially due to loss of reflexes
  • often leads to falls
40
Q

Gait difficulties

A
  • festination of gait = short shuffling steps with increased acceleration, lack of arm swing
  • freezing
41
Q

Vocal

A
  • considered to be partly due to bradykinesia
  • loss of variation and emotion or more rapid or stuttering
42
Q

Lead pipe rigidity

A
  • rigidity is all throughout (tight all throughout the range of motion)
43
Q

Cogswell rigidity

A
  • you get little bit tight, then pause
44
Q

Cognitive impairments of PD

A
  • functional cognition/executive function
  • memory problems
  • word finding
  • maybe due to certain type of PD = Lewy bodies dementia
45
Q

Secondary symptoms of PD

A
  • dysarthria (can’t make facial muscles)
  • dysphasia and difficulty swallowing (can’t make facial muscles)
  • flexed forward position (leaning forward with head and neck flexed)
  • slowed responsiveness
  • anxiety
  • depression
  • sexual function changes

Later stage changes:
- autonomic dysfunction
- urinary retention
- constipation
- orthostatic hypotension
- dementia

46
Q

Medical interventions for PD

A

Dopamine replacement medication:
- on/off times
- Levodopa/Sinemet (medication)
- causes dyskinesia = excessive movements
- when you are on the meds, you have to increase dosage each time to make it work

  • surgery = deep brain stimulation
  • medication for secondary conditions
47
Q

Stages of PD (modified Hohn and Yahr scale)

A

Stage 1:
- unilateral involvement with minimal functional impairment
- resting tremors
Stage 1.5:
- unilateral and axial involvement
Stage 2:
- bilateral involvement without impairment of balance
Stage 2.5:
- mild bilateral disease with recovery on pull test
Stage 3:
- mild to moderate functional impairments; bilateral disease
- postural instability
- physically independent
Stage 4:
- severe disability
- can walk or stand independently
Stage 5:
- the person requires use of wheelchair

*the key is to mange the symptoms and to manage it medically, to keep them strong, and to maybe slow down the progression

48
Q

Stages diagram (slide 39)

A
  • stage 1 = develop mild symptoms but able to hold about day-to-day life
  • stage 2 = symptoms such as tremors and stiffness begin to worsen, may develop poor posture or have trouble walking
  • stage 3 = movement begins to slow down, loss of balance
  • stage 4 = symptoms are severe and cause significant issues with day-to-day living, unable to live alone and will need care
  • stage 5 = walking or standing may be impossible at this point, people at this stage are often confined to a wheelchair or bed
49
Q

PD’s dementia

A
  • up to 80% of people with PD eventually develop dementia
  • the average time from onset of movement problems to the development of dementia is about 10 years
  • trouble focusing
  • trouble remembering things or making sound judgments
  • may develop depression, anxiety, or irritability
  • they may also hallucinate and see people,objects, or animals that are not there
  • sleep disturbances or “REM behavior disorder”, which involves acting out dreams
  • research suggests that a person with PDD may live an average of 5-7 years with the disease, although this can very from person to person
50
Q

Occupational implications of PD

A

ADLs:
- mobility challenges
- fine motor skills
- balance and coordination
IADLs:
- cooking and meal preparation
- household management
- driving
Work and Productivity:
- job performance employment status
- workplace accomodations
Leisure and Social Participation:
- social isolation
- engagement in hobbies
- physical activity
Mental Health:
- cognitive impairments
- depression and anxiety
Sleep and Rest:
- sleep disturbances
Communication:
- speech difficulties

51
Q

Amyotrophic lateral sclerosis (ALS)

A
  • also known as Lieu Gehrig’s disease
  • amyotrophic = derived from the Greek works “a” (no), “myo” (muscle), and “trophic” (nourishment), meaning “no muscle nourishment”
  • literally means muscle wasting and weakness seen in the disease
  • the nerves that feed the muscles die off
  • lateral = refers to the lateral columns of the spinal cord where the motor neurons are located
  • sclerosis = refers to the hardening or scarring of the tissue due to loss of neurons

*the brain and its cognition is still fully functioning

  • a motor neuron disease that attacked the motor neurons located in the brain, brainstem, and spinal cord
  • causes death to nerve cells controlling voluntary muscles of the body, resulting in paralysis and is ultimately fatal
  • loses the ability to walk, talk, eat, and then breathe
  • a rapidly progressive with no cure
  • the lived experience includes dealing with emotional, physical, caregiving, technology, and end of life issues
52
Q

Incidence and prevalence of ALS

A
  • average life expectancy = 2-5 years
  • every 90 minutes, someone is diagnosed with ALS and someone passes away from it
  • most people who develop ALS are between the ages of 40 to 70
  • average age at the time of diagnosis = 55 years old
  • cases of ALS do not occur in people in their 20s or 30s
  • ALS is 20% more common in men than woman (with increasing age, the incidence of ALS is more equal between men and women
  • about 90% of ALS cases occur without any known family history or genetic cause
  • the remaining 10% of ALS cases are inherited through a mutated gene with a known connection to the disease
  • for unknown reasons, military veterans are more than likely to be diagnosed with the disease than the general public
53
Q

Types of ALS

A
  • familial ALS = genetic mutation (10%)
  • sporadic ALS = no known cause (90%)

Various characteristics of ALS depends on initial effects:
- bulbar onset = initially affects the muscles controlling speech and swallowing
- spinal onset = initially affects muscles controlling the arms and legs

*eventually ALS affects all muscles in the body

54
Q

Signs and symptoms of ALS

A
  • no clear onset = the disease affects people at different rates and can have a subtle beginning
  • muscle weakness and atrophy are common first signs, often progressing in a symmetrical fashion DISTAL to PROXIMAL
  • usually noted first in the hands during fine motor tasks but sometimes noted in the feet when walking
  • damage to lower motor neurons = flaccid paralysis with decreased muscle tone and reflexes
  • loss of balance and falls
  • sensory systems, cognition, and urinary sphincters are usually spared
  • depression
    Lower motor neuron:
  • focal and multifocal weakness
  • atrophy (progressive; distal to proximal)
  • muscle cramping
  • fasciculation (muscle twitching)
    Corticospinal tract:
  • spasticity
  • hyperreactive reflexes
  • dysphasia (impaired quality of speech production)
    Corticobulbar tract:
  • dysphagia (difficulty swallowing)
  • dysarthria (impaired quality of speech production)
55
Q

How early stage ALS affects the body

A
  • poor posture
  • slurred speech
  • trouble breathing
  • twitching
  • lower limb weakness
  • tripping
  • muscle cramps
  • clumsy hands and fingers
  • trouble swallowing
56
Q

How is ALS diagnosed?

A
  • by ruling things out
  • nerve conduction study = measured the speed at which nerve cells transmit electrical impulses
  • electromyography = records the electrical activity of the muscle at rest and during muscle contractions
  • MRI = produces detailed images of structures inside the body
  • muscle biopsy = detects abnormalities in muscle tissue
  • spinal tab = provides information about inflammation and damage in the brain and spinal cord
57
Q

Cognitive and behavioral deficits of ALS

A
  • overlooked for years, now being researched
  • 35-50% have cognitive deficits
  • risk factors = older age, bulbar disease, family history dementia, presence of one abnormal gene repeat
  • described as frontal/executive dysfunction in nature, some develop fronto-temporal dementia
58
Q

Frontal/executive dysfunction and front-temporal dementia of ALS

A
  • functional cognition
  • decreased language fluency
  • memory deficits
  • verbal memory
  • social cognition
59
Q

Medical intervention of ALS

A
  • respiratory support (C paps)
  • gastrostomy tube (surgical opening into the stomach)
  • medication to manage symptoms
    3 FDA approved drugs:
  • Riluzole = helps people stay in older stage for longer and increases survival for 3-6 months
  • Radicava = does slow functional decline but has side effects
60
Q

Occupational implications of ALS

A

Early-stage ALS:
- trouble buttoning clothes or gripping objects
- balance problems and frequent tripping
- difficulty swallowing or speech problems
-muscle twitching and cramping
Middle-stage ALS:
- some muscles become paralyzed
- walkers and wheelchairs may be needed
- inability to drive
- breathing problems
Late-stage ALS (end of life planning):
- mobility is extremely limited
- most people require a feeding tube
- inability to communicate without assistance
- breathing function becomes severely impaired
End-stage ALS (end of life planning):
- paralysis of voluntary muscles
- respiratory complications are the most common cause of death
- hospice care may be necessary
- medications can be given to was pain and anxiety

Clinical Conditions (7 decks)

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