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BB Test 4 > Neurodegenerative Diseases SDL > Flashcards

Neurodegenerative Diseases SDL Flashcards

1
Q

What are the general features of neurodegenerative diseases?

A
  • Gray matter diseases (neuron cell bodies)
  • Usually an accumulation of protein due to problem with certain mechanism in cell
  • Selective injury (clinical findings determined from involved area)
  • Results in neuron loss and gliosis (scarring in response to injury)
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2
Q

What is the most common cause of dementia?

A

Alzheimer’s Disease (AD)

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3
Q

What is normally the cause of death from Alzheimer’s (AD)?

A
  • Pneumonia (infections)
  • Malnutrition
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4
Q

Describe the course of Alzheimer’s Disease (AD).

A
  1. Gradual impairment of cognitive function
  2. Later disorientation and memory loss
  3. Eventual immobilization and muteness
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5
Q

What is the histological findings in Alzheimer’s?

A
  • Neurofibrillary tangles and plaques (composed ot tau proteins)
  • Result from a cleavage error in β-APP and results in an accumulation of the β-amyloid which forms the plaques/tangles
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6
Q

What is another result of β-amyloid accumulation?

A

Cerebral Amyloid Angiopathy (CAA)

  • β-amyloid also accumulates in blood vessel walls which weakens them
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7
Q

What are some subsequent changes that occur in the neuron (both axon and body) as a result of Alzheimer’s?

A
  • Formation of paired helical filaments
  • Hyperphosphorylated tau
  • MAP2 (microtubule associated protein) problems
  • Ubiquination
  • Neuropil threads
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8
Q

What other disease shows neurofibrillary tangles?

A

Progressive Supranuclear Palsy (PSP) in the brainstem

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9
Q

In Alzheimer’s, where in the brain do neurofibrillary tangles start to form?

A
  • Start in hippocampus and spread along components of limbic system
  • Eventually involve neocortex
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10
Q

Which genes increase chances of developing familial or sporadic Alzheimer’s

A
  • Familial
    • β-APP (21)
    • Presenilin-1 (14)
    • Presenilin-2 (1)
  • Sporadic
    • ApoE4 (19)
    • IL-1A & B (2)
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11
Q

(T/F): Essentially all patients with DLB (Dementia with Lewy Bodies) have Parkinson’s.

A

True. They will have both cortical and substania nigra lewy bodies.

  • However, not all Parkinson’s patients have DLB (they might not have cortical LB)
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12
Q

What are the characteristics of Progressive Supranuclear Palsy (PSP)?

A
  • Symptoms:
    • Vertical gaze palsy
    • Truncal rigidity
  • Histology:
    • No tau mutations
    • Globose neurofibrillary tangles in globus pallidus, substantia nigra, and dentate nucleus
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13
Q

What are the characteristics of Corticobasal Degneration (CBD)?

A
  • Symptoms:
    • Extrapyramidal rigidity
    • Alien hand
    • Sensory cortical dysfunction
    • Cognitive decline
  • Histology:
    • Balooned neurons
    • Tau + astrocytes in motor and premotor cortex, basal ganglia, and substantia nigra
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14
Q

What are the symptoms of Parkinsons?

A
  • Tremor (at rest)
  • Rigidity (cogwheel)
  • Akinesia or bradykinesia (slow movements)
  • Postural instability (late)
  • Shuffling gate
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15
Q

What are the pathological findings in Parkinsons?

A
  • Loss of substantia nigra dopaminergic neurons
  • Lewy bodies in substantia nigra (α-synuclein +)
  • Loss of neuromelanin (pigment responsible for substania nigra color)
  • Associated with other Lewy body disorders
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16
Q

What are some other causes of Parkinsonism?

A
  • Drugs (especially neuroleptics)
  • Trauma (boxers)
  • Other basal ganglia and brainstem diseases
17
Q

Why might neuromelanin be seen microscopically even if a patient has Parkinsons?

A
  • Neuromelanin can remain for sometime in neuropil after neurons have degenerated
  • Neuromelanin can still be in the macrophages that are clearing it out
18
Q

What is Multiple System Atrophy (MSA)?

A
  • α-synuclein + neuronal inclusions
  • Involvement of cerebellum, pons, medulla, substantia nigra and striatum
  • Glial cytoplasmic inclusions, typically oligodendroglial
19
Q

What are the subtypes of Multiple System Atrophy (MSA)?

A
  • MSA-P: Parkinsonism-dominant (striatonigral degeneration)
  • MSA-C: Cerebellar dysfunction dominant (olivopontocerebellar atrophy)
  • MSA-A: Autonomic dysfunction dominant (Shy-Drager syndrome)
20
Q

What are the characteristics of Huntington’s Disease?

A
  • Large,involuntary movements (chorea)
  • Cortical neuron loss and dementia (usually late)
  • Trinucleotide repeats (CAG) in huntington gene (Chr 4)
  • Atrophy of striate nuclei (cuadate, putamen) and globus pallidus
21
Q

What the characteristics and subtypes of Spinocereballar Degeneration?

A
  • Involves degernation in the cerebellum, spinal cord, and peripheral nerves
  • Results in:
    • Cereballar and sensory ataxia, spasticity, sensorimotor peripheral neuropathy
    • It is inherited
    • There is neuron loss and gliosis
  • Subtypes: Spinocerebllar ataxias (SCA), Friedreich’s ataxia (FA), Ataxia telengiectasia (AT)
22
Q

(T/F): Motor Neuron Disease can involve upper motor neurons, spinal nerve lower motor neurons, or cranial nerve motor neurons.

A

True.

23
Q

What are the characteristics of ALS?

A
  • Affects middle-aged adults
  • Slowly progressive (approx 5 years)
  • Loss of anterior horn motor neurons
  • Degeneration of lateral corticospinal tracts
  • Muscle atrophy/weakness
  • Eventual respiratory muscle involvement and death
24
Q

What is Infantile Spinal Muscular Atrophy?

A
  • AKA Werdnig-Hoffman Disease
  • A degenerative motor neuron disease that affects spinal muscles in infants (more severe than chronic/adult form)
  • Death within one year
  • Loss of anterior horn motor neurons

BB Test 4 (14 decks)

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