Neurodegenerative Disorders

Question 1

Akinesia

Answer 1

• Impairment of voluntary and spontaneous movement initiation that results in FREEZING, especially during gait activities
• Inability to move
• Can also present as a delayed response or freezing mid-action

Question 2

Bradykinesia

Answer 2

• Slowed motor movements
• Extreme slowness is observed when the person initiates or performs volitional movements, especially in Parkinson’s disease.
• Common examples include shuffling gait, difficulty moving from sitting to standing, and increased time required for fine motor tasks such as shaving

Question 3

Dysmetria

Answer 3

• Decreased coordination of movements due to an inability to estimate the ROM necessary to reach the target of movement
• Due to a cerebellar disorder
• Hypermetria = the limb overshoots the target
• Hypometria = the limb undershoots the target

Question 4

Rigidity

Answer 4

• Muscle stiffness that impairs movement
• Common in Parkinson’s disease (leading to cogwheel-like motions, which are jerky, sometimes painful movements with joint mobility, most commonly in the upper extremities)
• Results in patient needing increased effort to produce voluntary movement (and thus increases fatigue which becomes a barrier to occupational performance)

Question 5

Fasciculation

Answer 5

• Involuntary muscle contraction and relaxation
• Observed as a muscle twitch

Question 6

Festinating gait

Answer 6

• Small rapid steps that result from a forward-tilted head and trunk posture

Question 7

Paresthesia

Answer 7

Numbness and tingling because of sensory nerve changes

Question 8

Ataxia

Answer 8

• Lack of coordination
• Results in jerky, poorly controlled movements (e.g., a staggering, wide-based gait with little to no arm swing)

Question 9

Intention Tremors

Answer 9

• Tremors occurring when a person attempts to engage in meaningful activity, such as reaching for an object
• Common motor symptom of Multiple Sclerosis

Question 10

Resting Tremor

Answer 10

• Tremors that occur when the person is at rest (and are absent with voluntary movement)
• Typically an early sign of Parkinson’s Disease
• Can result in a “pill rolling” movement of one hand
• Tremors vary, though typically disappear when the person is asleep or calm, and increase with stress or cognitive tasks

Question 11

Parkinson’s Disease - Stage 1

Answer 11

• Unilateral symptoms (typically a resting tremor) with no or minimal loss of function

Question 12

Parkinson’s Disease - Stage 2

Answer 12

• Bilateral symptoms
• Balance is not affected, although problems develop with trunk mobility and postural reflexes

Question 13

Parkinson’s Disease - Stage 3

Answer 13

• Impaired balance secondary to postural instability resulting in mild to moderate impairments in function

Question 14

Parkinson’s Disease - Stage 4

Answer 14

• Decrease in postural stability
• Decrease in function
• Impaired mobility
• Poor fine motor and dexterity
• Need for assistance with ADLs

Question 15

Parkinson’s Disease - Stage 5

Answer 15

• Total dependence for mobility and ADLs

Question 16

Amyotrophic Lateral Sclerosis (ALS)

Answer 16

• Aka Lou Gehrig’s disease
• Progressive, degenerative disease in which the motor neurons in the brain, spinal cord, and peripheral system are destroyed and replaced by scar tissue
• The resulting plaques lead to progressive muscle atrophy
• Exact symptoms depend on the part of the nervous system affected; however, typically progress distal to proximal
• Most typical initial symptom is weakness of the small muscles of the hand OR an asymmetrical foot drop with or without night cramps, usually in the calves
• If the bulbar tract is affected, initial symptoms might include difficulty breathing, slurred speech, decreased volume in speech, or impaired swallowing
• People eventually develop ALL symptoms, becoming progressively weak and immobile
• Cognition, sensation, vision and hearing, and bowel and bladder control are typically NOT affected
• Life expectancy is 1 to 5 years after diagnosis (average is 3 years); disease course may be longer for younger people whose symptoms are milder. Death usually occurs secondary to respiratory failure

Question 17

ALS - Stage I

Answer 17

• The person can walk and has some weakness
• Independent with ADLs

Question 18

ALS - Stage II

Answer 18

• The person can walk and has moderate weakness

Question 19

ALS - Stage III

Answer 19

• The person can walk but has severe weakness

Question 20

ALS - Stage IV

Answer 20

• The person requires a wheelchair for mobility, and has severe weakness in the legs
• Needs some assistance with ADLs

Question 21

ALS - Stage V

Answer 21

• The person requires a wheelchair for mobility and has severe weakness in the arms AND legs
• Dependent for ADLs

Question 22

ALS - Stage VI

Answer 22

• The person is confined to bed
• Dependent for ADLs and most self-care tasks

Question 23

Guillain-Barre Syndrome

Answer 23

• An inflammatory disease that causes demyelination of axons in peripheral nerves
• Has three phases and NO cure, although some medications can help reduce severity and speed recovery (i.e., IV immunoglobulin, plasma exchange, steroids).
• Involves rapid progression of symptoms, typically in a SYMMETRICAL (bilateral) ascending pattern of flaccid paralysis that begins in the feet. (Paralysis may also occur in respiratory muscles.)
  -Typically requires extensive medical care (including intensive care curing plateau phase and in/outpatient rehab during recovery)
• Signs and symptoms:
  1. Pain (mostly in lower extremities)
  2. Fatigue
  3. Absence of deep tendon reflexes
  4. Mild sensory loss in hands and legs (“glove and stocking” distribution)
  5. Dysfunction of cranial nerves, including possible facial palsy
  6. ANS involvement, including postural hypotension, arrhythmias, facial flushing, diarrhea, impotence, urinary retention, and increased sweating
  7. Bladder dysfunction

Question 24

GBS - Onset & acute inflammatory phase

Answer 24

• First phase of GBS
• Acute weakness that occurs in at least 2 extremities advances and reaches its maximum in 2 to 4 weeks
• 20 to 30% of people with GBS need mechanical ventilation

Question 25

GBS - Plateau phase

Answer 25

• Symptoms are at their most disabling, with little or no change over a few days or weeks
• Spoken communication is often impaired in the plateau phase of GBS because of paralysis of muscles in the head and neck
• HYPERsensitivity to tactile input is also typical

Question 26

GBS - Progressive recovery phase

Answer 26

• Remyelination and axonal regeneration occur over a period as long as 2 years
• Recovery tends to start at the head and neck and travel distally
• Most people experience significant if not complete return of function. (15% are left with more permanent dysfunction)
• Fatigue is the most commonly reported residual symptom, as well as minor cognitive difficulties (e.g., impaired executive function, short-term memory, & decision-making).

Question 27

Huntington’s Disease (HD)

Answer 27

• A hereditary, progressive neurological disorder that leads to severe physical and mental disabilities
• Over time, HD causes progressive loss of nerve cells in the brain affecting movement, cognition, emotions, and behavior
• Symptoms usually appear in 30s or 40s and progress over 15- to 20-year period, leading to long-term care/hospitalization
• Death typically results from secondary causes related to the disease
• Signs & Symptoms:
  1. Motor difficulties, including both involuntary and voluntary movement patterns (Involuntary: choreiform movements of hands, chorea, akathisia, dystonia, hypertonicity. Voluntary: bradykinesia, akinesia, and incoordination of movement)
  2. Deterioration of cognitive and behavioral abilities (e.g., forgetfulness, difficulty concentrating, irritability and depression)
  3. Slowing of saccadic eye movements and ocular pursuits
  4. Dysphagia

Question 28

Chorea

Answer 28

• Aka choreiform movement
• Rapid, involuntary, irregular movements
• Typically increasing during stressful situations and lessening or absent during voluntary motor activities and sleep
• Is a common symptom of Huntington’s disease

Question 29

Akathisia

Answer 29

• Involuntary motor restlessness
• Inability to remain still
• Can be accompanied by an intense sensation of unease or inner restlessness that usually involves the lower extremities
• Is a symptom of Huntington’s disease
• Also, is a frequent side effect of antipsychotic drugs that block dopamine receptors

Question 30

Dystonia

Answer 30

• Abnormal, sustained posturing of a body part, typically the arms, head or trunk
• Can be painful due to prolonged muscle contractions causing the abnormal movements and postures
• Is a symptom of Huntington’s disease and Parkinson’s

Question 31

Alzheimer’s Disease

Answer 31

• Type of dementia or neurocognitive disorder
• Caused by an accumulation of amyloid-beta senile plaques and tau protein neurofibrillary tangles, leading to cortical atrophy of the frontal, parietal, and temporal lobes and hippocampal region
• Results in slow & progressive impairment of memory, executive function, attention, language, visual processing, and praxis
• Three stages (early/mild, middle/moderate, late/severe)

Question 32

Vascular dementia

Answer 32

• Cerebrovascular disease (often a series of small strokes) that leads to focal lesions on the brain and neurotransmitter disruption
• Causes cognitive decline similar to AD but often less severe memory impairment
• Also commonly causes gait disturbances
• Typically has a sudden appearance of symptoms with a step-wise pattern of increased symptoms (though may also progress slowly like AD)
• Requires immediate emergency medical treatment for cardiovascular factors (blood clots)

Question 33

Frontotemporal dementia

Answer 33

• Pathology: neuronal, intranuclear inclusions (build-up of proteins in central and/or peripheral nervous systems)
• Is a progressive disorder with an immediate, distinct onset and varied symptom patterns
• Has two main variants/presentations:
  1. Behavioral = prominent changes in personality and behavior
  2. Semantic = progressive aphasia (language skills, speaking, writing, and comprehension)

Question 34

Dementia with Lewy Bodies

Answer 34

• Caused by Lewy body proteins being present in the brain (usually predominant in the limbic or neocorticol areas), as well as an overall declines in acetylcholine and dopamine levels
• Gradual and insidious progression
• Distinguishing features: VISUAL HALLUCINATIONS and PARKINSON-LIKE MOTOR SYMPTOMS
• Early deficits: executive function, visuospatial abilities, attention, and memory function
• Later symptoms: aphasia, apraxia, and spatial disorientation
• Medical management includes cholinesterase inhibitors (decease the breakdown of acetylcholine)

Question 35

Paratonia

Answer 35

• Involuntary resistance to passive movement of the extremities
• Possible motor deficit with dementia

Question 36

Early Stage - Effects of Dementia on Occupational Performance

Answer 36

• Recent memory (recall of recent events) and IADLs are affected first.
• FORGETFULNESS
• ADLs remain intact.
• Orientation to place (wayfinding) is also impaired, which impairs community mobility
• Withdrawal from social participation is common
• Patients tend to cling to old habits and repetitive routines; may be quite rigid in their routines

Question 37

Procedural Memory

Answer 37

• Recall of information on how to perform a task, such as knowing how to write or ride a bike.
• Typically remains intact in dementia patients in early stages (begins to worsen in middle stages).

Question 38

Personal Episodic Memory

Answer 38

• Recall of time-related information about oneself, such as where and whether one ate breakfast
• Typically is impaired with dementia patients

Question 39

Semantic Memory

Answer 39

• Ability to remember the names of objects
• Shows deficits in dementia patients

Question 40

Middle Stage - Effects of Dementia on Occupational Performance

Answer 40

• Impairments occur in all areas of occupation, including ADLs
• CONFUSION
• Person can no longer live alone
• Some home tasks (such as cooking and cleaning) can be completed with assistance or supervision
• SAFETY is a major concern, especially due to wandering behaviors
• Person cannot work or perform education activities, and social participation is typically limited to caregivers
• All types of memory begin to worsen, including procedural memory

Question 41

Late Stage - Effects of Dementia on Occupational Performance

Answer 41

• All areas of occupation are lost, including communication (speech often consists of only one word and may not make sense; comprehension is also impaired)
• VISUAL HALLUCINATIONS are possible
• ALL memory is lost
• Dependent with ADLs
• Can no longer ambulate safely
• No understanding of cultural, social, or spiritual contexts