Progressive Neurological Disorders (PND)

Question 1

Progressive neurological disorders (PNDs)

Answer 1

• A cluster of disorders with similar attributes
• Impact the neurological system in different ways and are progressive in nature, meaning that clients with these disorders will continue to lose function over time (does not mean that we go into adapt-only mode)

Question 2

ALS Description and Definitions

Answer 2

• Lou Gehrig disease
• Progressive / fatal
• Motor neuron disease; scars form on upper motor neurons in corticospinal pathways
• Lower motor neurons also affected
• Inflammatory component in the process

Question 3

2 ALS Types

Answer 3

• Familial

- Sporadic

Question 4

Familial (Types of ALS)

Answer 4

10% of cases, genetic mutation, family history

Question 5

Sporadic (Types of ALS)

Answer 5

Most cases, speculation viral, retroviral, environmental causes, genetics

Question 6

ALS Etiology

Answer 6

Exposure to chemicals / lead, electromagnetic fields

Question 7

ALS Incidence and Prevalence

Answer 7

• Most affected worldwide: white males older than 60 years
• 3.9 cases per 100,000 persons
• Sporadic ALS affects more males vs females
• Familial ALS affects males and females relatively the same

Question 8

ALS Signs and Symptoms

Answer 8

• Weakness of fine motor muscles of the hand / asymmetrical foot drop (most common)
• Night cramps (calf muscles)
• Spasticity
• Loss of emotional control
• Difficulty speaking/swallowing
• Reduced body temp regulation
• Reduced executive functions
• Respiratory failure
• Distal to proximal progression
• Hyperresponsive reflexes
• Distorted speech

Question 9

ALS Course and Prognosis

Answer 9

• Age of onset between 16 and 77 years
• On average the diagnosis occurs between ages 55 – 77 years
• Sporadic peak age of onset 58 to 63 years
• Familial peak age of onset 47 to 52 years
• 1 to 5 years survival post diagnosis
• Death often caused by respiratory failure / pneumonia

Question 10

ALS Medical and Surgical Management

Answer 10

• Riluzole only medication approved for treatment
• Antispasmodic medications
• Nonsteroidal anti-inflammatory medications
• Gastrostomy
• Noninvasive positive-pressure ventilation
• Nebulizers

Question 11

MS Description and Definitions

Answer 11

• Immunological / neurodegenerative disease
• Immune system attacks myelin sheath around brain, spinal cord, and optic nerve (demyelination)
• Chronic inflammation / diffuse demyelination to white / gray matter and axons
• Formation of plaques

Question 12

MS Etiology

Answer 12

• Unknown
• Hypothesis: genetics (polygenetic) + environmental factors
• Viral infection
• Smoking

Question 13

MS Incidence and Prevalence

Answer 13

• Most common among people under age 40
• 1% of US population, rate increasing
• Less likely to have MS living by equator
• Affects females more
• Scandinavian / Scottish descent more susceptible
• 2x more common among Caucasians

Question 14

MS Signs and Symptoms

Answer 14

• Fatigue
• Motor, sensory, visual and automatic systems affected
• Diplopia
• Dysesthesia/Paresthesia
• Erectile Dysfunction
• Gross/Fine Motor Incoordination
• Cognitive Deficits
• Depression/Euphoria
• :Loss of Visual Acuity
• Spasticity
• Dysphagia
• Vestibular Dysfunction

Question 15

MS Course and Prognosis

Answer 15

-Diganosis between age 20 to 40 years old
-30 years old peak onset
-Not common in > 60 years old
-10 years post diagnosis 10% wheelchair bound / 50% unable to work
-Diagnosis in 20’s = prognosis approximately 46-60 years
-Diagnosis in 60’s = prognosis approximately 13-22 years
4 patterns:
-Benign
-Relapsing-remitting-nonprogressive
-Relapsing-remitting-progressive
-Primary progressive

Question 16

MS Medical and Surgical Management

Answer 16

• Disease-modifying drugs
• Beta-blockers
• Anticholinergics
• Antidepressants
• Steroids
• Cannabis extract
• Cognitive rehabilitation
• OT/PT
• Bone Marrow Transplants
• Low Impact Exercise
• Healthy Diets

Question 17

Parkinson’s Description and Definitions

Answer 17

• Death of dopaminergic neurons in substantia nigra
• Presence of Lewy bodies; abnormal formation of proteins within cell body / neurons
• Decrease dopamine = deficits in speed / quality of motor movements, cognitive skills, postural stability, affective expression
• Serotonergic denervation

Question 18

Parkinson’s Etiology

Answer 18

• Some evidence; genetic + environmental factors
• Familial PD
• Sporadic PD

Question 19

Familial PD

Answer 19

Genetic Association

Question 20

Sporadic PD

Answer 20

Environmental Factors

• History of head trauma
• Dietary intake
• Exposure to environmental elements

Question 21

Parkinson’s Incidence and Prevalence

Answer 21

• 2nd most common neurodegerative disorder
• Approx. 1 million cases in the US; age >60 yrs old
• More common in Europe, North/South America
• 10-18% of every 100,000 ppl/year
• Affects males slightly more
• Greatest among Hispanics in US

Question 22

Parkinson’s Signs and Symptoms

Answer 22

• Bradykinesia
• Resting tremor; “Pill Rolling”
• Muscle rigidity
• Progressive loss of facial expression
• infrequent eye blinking (early sign)
• Hyposmia
• Depression
• Constipation
• Sleep behavior disorder
• “Festinating Gait”
• Sexual dysfunction
• Anxiety

Question 23

Parkinson’s Course and Prognosis

Answer 23

-1st diagnosis in people older than 50 years
-Average onset 60 years old
-Slow and progressive
-15 – 20 years before onset of most severe stages
3 phases
-Preclinical period
-Prodromal period
-Symptomatic period; 5 stages`

Question 24

Parkinson’s Medical and Surgical Management

Answer 24

• Medication to increase uptake of dopamine
• Thalamotomy
• Deep brain stimulation
• Pallidotomy
• Continuous device-aided drug delivery
• Gene therapy
• Stem cell / fetal cell transplantation

Question 25

Neuro Conditions Impact on Occupational Performance

Answer 25

• Self-care skills
• Eating
• Mobility
• Family roles
• Formal education
• Employment
• Sexual expression
• Communication
• Mental health
• Loss of leisure engagement