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Medical Conditions > Progressive Neurodegenerative Diseases > Flashcards

Progressive Neurodegenerative Diseases Flashcards

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USMLE® Step 1 flashcards
1
Q

Multiple Sclerosis

A
  • Immunological / neurodegenerative disease
  • Immune system attacks myelin sheath around brain,
  • Chronic inflammation / diffuse demyelination to white / gray
  • Formation of plaques/scar tissue
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2
Q

MS Etiology

A
  • Unknown
  • Hypothesis: genetics (polygenetic)
  • Familial recurrence rate of 20%
  • Smoking
  • Vitamin D deficiency
  • Heavy metal toxins in the environment
  • History of viral infections
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3
Q

MS Incidence and Prevalence

A
  • 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
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4
Q

MS Signs and Symptoms

A

-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

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5
Q

MS Course and Prognosis*

A
  • 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
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6
Q

Relapsing-remitting-non-progressive

A

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

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7
Q

Benign

A

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

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8
Q

Relapsing-remitting-progressive

A

Person has some residual impairment with each remission

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9
Q

Primary progressive

A

Steady decline in function without remissions and exacerbations

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10
Q

MS Medical / Surgical Management

A
  • 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
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11
Q

Parkinson’s Disease Description and Definitions

A
  • 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
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12
Q

PD Etiology

A
  • 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
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13
Q

PD Incidence and Prevalence

A
  • 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
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14
Q

PD Signs and Primary Symptoms

A
  • Muscle rigidity
  • Bradykinesia
  • Resting tremor
    Hyposmia (reduced olfactory sense)
  • Fatigue
  • Depression
  • Rapid eye movement (REM)
  • Sleep behavior disorder
  • Constipation
    -all different
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15
Q

PD Signs and secondary Symptoms

A

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

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16
Q

PD Course and Prognosis

A
  • 1st dx older than 50 yrs
  • Avg onset 60 yrs old
  • Slow and progressive
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17
Q

Preclinical period-

A

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

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18
Q

Prodromal period

A

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

19
Q

Symptomatic Period;

A

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

20
Q

PD Stage I:

A

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

21
Q

PD Stage II

A

Signs of PD are bilateral and balance is not impaired

22
Q

PD Stage 3

A

Signs of PD are bilateral and balance is impaired

23
Q

PD stage 4

A

PD is functionally disabling.

24
Q

PD stage 5

A

The person is confined to bed or a wheelchair

25
Q

PD Medical / Surgical Management

A
  • 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
26
Q

Amyotrophic Lateral Sclerosis Description and Definitions

A
  • Lou Gehrig disease
  • Progressive / fatal
  • Motor neuron disease
  • Neurodegeneration
  • Lower motor neurons also affected
  • Inflammatory component in the process
27
Q

ALS Etiology

A
  • 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
28
Q

ALS Incidence and Prevalence

A
  • 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
29
Q

ALS Signs and Symptoms

A
  • 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
30
Q

Corticospinal tract

A

Muscle spasticity, weakness, and hyperresponsive reflexes

31
Q

Corticobulbar tract dysfunction

A

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

32
Q

ALS Course and Prognosis

A
  • 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
33
Q

ALS Medical / Surgical Management

A

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

34
Q

PND Impact on Occupational Performance

A

all

35
Q

Demyelination

A

damage to the myelin sheath around nerves

36
Q

Familial

A

relating to or occurring in a family or its members:

37
Q

Lewy body pathology

A

Neuronal cytoplasmic inclusion bodies,
type of demintia

38
Q

Motor neurons

A

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

39
Q

Myelin sheath

A

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

40
Q

Non–tremor-dominant PD

A

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

41
Q

Sporadic

A

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

42
Q

Substantia nigra

A

part of your brain that helps control your movements.

43
Q

Symptomatic period

A

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

44
Q

Tremor-dominant PD

A

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

Medical Conditions (18 decks)

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