Progressive Neurodegenerative Diseases

Question 1

Multiple Sclerosis

Answer 1

• Immunological / neurodegenerative disease
• Immune system attacks myelin sheath around brain,
• Chronic inflammation / diffuse demyelination to white / gray
• Formation of plaques/scar tissue

Question 2

MS Etiology

Answer 2

• Unknown
• Hypothesis: genetics (polygenetic)
• Familial recurrence rate of 20%
• Smoking
• Vitamin D deficiency
• Heavy metal toxins in the environment
• History of viral infections

Question 3

MS Incidence and Prevalence

Answer 3

• Most common among people under age 40
• 10 to 18 per 100,000 people per year
• 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

Question 4

MS Signs and Symptoms

Answer 4

-location determines deficit
Motor, sensory, visual, and autonomic systems affected
* Visual deficits
* Urinary incontinence or retention
* Weakness
* Pain
* Spasticity
* Ataxia, dysphagia, dysarthria
* Vestibular dysfunction
* Cognitive or emotional disturbances
* Sensory Disturbances
* Dysesthesia / paresthesia
* Erectile dysfunction
* Gross / fine motor incoordination
* Fatigue

Question 5

MS Course and Prognosis*

Answer 5

• Dx between age 20 to 40 yrs old
• 30yrs old peak onset
• Not common in > 60 yrs old
• 4 patterns:
   Benign
   Relapsing -remitting-non-progressive
   Relapsing-remitting-progressive
   Primary progressive

Question 6

Relapsing-remitting-non-progressive

Answer 6

Person returns to the previous level of function after each exacerbation with no residual deficits.

Question 7

Benign

Answer 7

Person experiences one or two episodes of neurological deficits with no residual impairments

Question 8

Relapsing-remitting-progressive

Answer 8

Person has some residual impairment with each remission

Question 9

Primary progressive

Answer 9

Steady decline in function without remissions and exacerbations

Question 10

MS Medical / Surgical Management

Answer 10

• Disease-modifying drugs
  -Beta-blockers - Spasticity
• Anticholinergics - Dysphagia
• Antidepressants
• Acetylcholinesterase inhibitors - Intended for Alzheimer’s Disease but used to treat cognitive symptoms
• Steroids - Nerve Inflammation
• Cannabis extract
• Pain Killers
  -ot/pt

Question 11

Parkinson’s Disease Description and Definitions

Answer 11

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

Question 12

PD Etiology

Answer 12

• Some evidence; genetic + environmental factors
• Familial PD – genetic association
• Sporadic PD – environmental factors
   Hx of head trauma
   Dietary intake (too much iron)
   Exposure to environmental elements
   Beta-blockers and antipsychotics

Question 13

PD Incidence and Prevalence

Answer 13

• 2nd most common neurodegenerative disorder
• ~ 1million cases in US; age >60 yrs old
• More common in Europe, North America, South America
• 10% - 18% / 100,000 people / yr
• Affects males slightly more
• Greatest among Hispanics in US

Question 14

PD Signs and Primary Symptoms

Answer 14

• Muscle rigidity
• Bradykinesia
• Resting tremor
  Hyposmia (reduced olfactory sense)
• Fatigue
• Depression
• Rapid eye movement (REM)
• Sleep behavior disorder
• Constipation
  -all different

Question 15

PD Signs and secondary Symptoms

Answer 15

-Gait disturbances
-Micrographia (small handwriting)
-Dexterity and coordination difficulties
* Involuntary immobilization
-Cognitive impairments
-all different

Question 16

PD Course and Prognosis

Answer 16

• 1st dx older than 50 yrs
• Avg onset 60 yrs old
• Slow and progressive

Question 17

Preclinical period-

Answer 17

Neurons have begun to degenerate, but no symptoms are yet evident

Question 18

Prodromal period

Answer 18

Generalized symptoms such as depression, anxiety, constipation, REM,
behavior disorder, and fatigue may appear

Question 19

Symptomatic Period;

Answer 19

stages of classic motor PD symptoms are evident followed by
progressive nonmotor symptoms

Question 20

PD Stage I:

Answer 20

Signs of PD are strictly one-sided, affecting one side of the body only.

Question 21

PD Stage II

Answer 21

Signs of PD are bilateral and balance is not impaired

Question 22

PD Stage 3

Answer 22

Signs of PD are bilateral and balance is impaired

Question 23

PD stage 4

Answer 23

PD is functionally disabling.

Question 24

PD stage 5

Answer 24

The person is confined to bed or a wheelchair

Question 25

PD Medical / Surgical Management

Answer 25

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

Question 26

Amyotrophic Lateral Sclerosis Description and Definitions

Answer 26

* Lou Gehrig disease * Progressive / fatal * Motor neuron disease * Neurodegeneration * Lower motor neurons also affected * Inflammatory component in the process

Question 27

ALS Etiology

Answer 27

* No known cause, but it is speculated lead to this motor neuron disease * 2 ALS types:  Familial  Sporadic * Familial: 10% of cases, genetic mutation, family hx * Sporadic: most cases, speculation viral, retroviral, environmental causes, genetics * Exposure to chemicals / lead, electromagnetic fields

Question 28

ALS Incidence and Prevalence

Answer 28

* Most affected worldwide: white males older than 60 yrs * 3.9 cases / 100,000 persons in US * Sporadic ALS affects more males vs females * Familial ALS affects male / female relatively same

Question 29

ALS Signs and Symptoms

Answer 29

* Initial symptoms include weakness of FM muscles of the hand / asymmetrical foot drop (most common) * Night cramps (calf muscles) * Spasticity * Loss of emotional control * Difficulty speaking / swallowing * Cognitive deficits

Question 30

Corticospinal tract

Answer 30

Muscle spasticity, weakness, and hyperresponsive reflexes

Question 31

Corticobulbar tract dysfunction

Answer 31

Slow, labored, and distorted speech, often with a nasal quality, hyperactive gag reflex, tongue atrophy, and dysphasia

Question 32

ALS Course and Prognosis

Answer 32

* Age of onset between 16 and 77 yrs * ~ dx between ages 55 – 77 yrs * Sporadic peak age of onset 58 to 63 yrs * Familial peak age of onset 47 to 52 yrs * 1 to 5 yrs survival post dx * Death often cause of respiratory failure / pneumonia

Question 33

ALS Medical / Surgical Management

Answer 33

Medical treatments that are currently available do little to alter the fatal course of ALS * Riluzole only medication approved to tx * Meds for Relief from Symptoms -Gastrostomy

Question 34

PND Impact on Occupational Performance

Answer 34

all

Question 35

Demyelination

Answer 35

damage to the myelin sheath around nerves

Question 36

Familial

Answer 36

relating to or occurring in a family or its members:

Question 37

Lewy body pathology

Answer 37

Neuronal cytoplasmic inclusion bodies, type of demintia

Question 38

Motor neurons

Answer 38

a nerve cell forming part of a pathway along which impulses pass from the brain or spinal cord to a muscle or gland.

Question 39

Myelin sheath

Answer 39

protective layer of fat and protein that surrounds the main "body" section of a nerve cell called the axon . It increases the speed at which nerve impulses can travel along the axon1

Question 40

Non–tremor-dominant PD

Answer 40

a pattern with hypermetabolism in the pons, globus pallidus, and the thalamus can be seen

Question 41

Sporadic

Answer 41

refers to the sudden and unpredictable occurrence of a disease or infection

Question 42

Substantia nigra

Answer 42

part of your brain that helps control your movements.

Question 43

Symptomatic period

Answer 43

When a person has some symptoms associated with a disease or condition

Question 44

Tremor-dominant PD

Answer 44

characterized by initial prominent resting and action tremor, mild parkinsonism, unpredictable response to medication, and a better prognosis than idiopathic Parkinson's disease