Progressive Neurodegenerative Diseases Flashcards
Multiple Sclerosis
- Immunological / neurodegenerative disease
- Immune system attacks myelin sheath around brain,
- Chronic inflammation / diffuse demyelination to white / gray
- Formation of plaques/scar tissue
MS Etiology
- Unknown
- Hypothesis: genetics (polygenetic)
- Familial recurrence rate of 20%
- Smoking
- Vitamin D deficiency
- Heavy metal toxins in the environment
- History of viral infections
MS Incidence and Prevalence
- 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
MS Signs and Symptoms
-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
MS Course and Prognosis*
- 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
Relapsing-remitting-non-progressive
Person returns to the previous level of function after each exacerbation with no residual deficits.
Benign
Person experiences one or two episodes of neurological deficits with no residual impairments
Relapsing-remitting-progressive
Person has some residual impairment with each remission
Primary progressive
Steady decline in function without remissions and exacerbations
MS Medical / Surgical Management
- 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
Parkinson’s Disease Description and Definitions
- 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
PD Etiology
- 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
PD Incidence and Prevalence
- 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
PD Signs and Primary Symptoms
- Muscle rigidity
- Bradykinesia
- Resting tremor
Hyposmia (reduced olfactory sense) - Fatigue
- Depression
- Rapid eye movement (REM)
- Sleep behavior disorder
- Constipation
-all different
PD Signs and secondary Symptoms
-Gait disturbances
-Micrographia (small handwriting)
-Dexterity and coordination difficulties
* Involuntary immobilization
-Cognitive impairments
-all different
PD Course and Prognosis
- 1st dx older than 50 yrs
- Avg onset 60 yrs old
- Slow and progressive
Preclinical period-
Neurons have begun to degenerate, but no symptoms are yet evident
Prodromal period
Generalized symptoms such as depression, anxiety, constipation, REM,
behavior disorder, and fatigue may appear
Symptomatic Period;
stages of classic motor PD symptoms are evident followed by
progressive nonmotor symptoms
PD Stage I:
Signs of PD are strictly one-sided, affecting one side of the body only.
PD Stage II
Signs of PD are bilateral and balance is not impaired
PD Stage 3
Signs of PD are bilateral and balance is impaired
PD stage 4
PD is functionally disabling.
PD stage 5
The person is confined to bed or a wheelchair
PD Medical / Surgical Management
- 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
Amyotrophic Lateral Sclerosis Description and Definitions
- Lou Gehrig disease
- Progressive / fatal
- Motor neuron disease
- Neurodegeneration
- Lower motor neurons also affected
- Inflammatory component in the process
ALS Etiology
- 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
ALS Incidence and Prevalence
- 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
ALS Signs and Symptoms
- 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
Corticospinal tract
Muscle spasticity, weakness, and hyperresponsive reflexes
Corticobulbar tract dysfunction
Slow, labored, and distorted speech, often with a nasal quality, hyperactive
gag reflex, tongue atrophy, and dysphasia
ALS Course and Prognosis
- 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
ALS Medical / Surgical Management
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
PND Impact on Occupational Performance
all
Demyelination
damage to the myelin sheath around nerves
Familial
relating to or occurring in a family or its members:
Lewy body pathology
Neuronal cytoplasmic inclusion bodies,
type of demintia
Motor neurons
a nerve cell forming part of a pathway along which impulses pass from the brain or spinal cord to a muscle or gland.
Myelin sheath
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
Non–tremor-dominant PD
a pattern with hypermetabolism in the pons, globus pallidus, and the thalamus can be seen
Sporadic
refers to the sudden and unpredictable occurrence of a disease or infection
Substantia nigra
part of your brain that helps control your movements.
Symptomatic period
When a person has some symptoms associated with a disease or condition
Tremor-dominant PD
characterized by initial prominent resting and action tremor, mild parkinsonism, unpredictable response to medication, and a better prognosis than idiopathic Parkinson’s disease
