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From Flashcardlet > Neuro 67: Motor system pathology > Flashcards

Neuro 67: Motor system pathology Flashcards

1
Q

Causes/types of Parkinson’s

A
  1. Idiopathic
  2. Hereditary
  3. Postencephalic
  4. toxic (pesticides, herbicides, cycad beans, etc)
  5. head trauma (esp. boxers)
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2
Q

Alpha-synuclein (park 1)

A
  • early onset
  • dominant inheritance
  • numerous mutations in the gene that makes alpha-synclein
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3
Q

Parkinson’s a mito disease?

A
  • increased reactive oxygen species (ROS)–> increase mito permeability –> causes apop
  • ROS also inhibit ubiquitin-proteolysis system –> causes an increase in misfolded proteins –> lewy body formation Increase
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4
Q

Ubiquitone-proteolysis system

A
  • gets rid of misfolded proteins

- may be abnormal in PD –> leads to the buildup of Lewy bodies

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5
Q

Misfolding of proteins

A
  • Forms inclusion bodies that can aggregate and can cause degredation or apoptosis
  • misfolded proteins can have abnormal fctn
  • mutations, chaperone abnormalities or free radicals cause misfolding
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6
Q

6 Clinical features of classic PD

A
  1. Onset 50-70 yrs in the classic form
  2. Tremor at rest, Esp pill rolling
  3. Rigidity
  4. Bradykinesia (slow moving)
  5. Dementia late in some cases
  6. Disability in 10-15 hrs
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7
Q

Substantia nigra in PD

A
  • loss of the pigmented neurons in the substantia nigra

- this causes an injury to the dopaminergic system

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8
Q

Lewy bodies

A
  • seen in PD
  • cytoplasmic inclusions in the neurons
  • usually 2
  • have darker center with a clearer halo
  • sometimes have multiple rings
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9
Q

Inclusions of lewy bodies

A

-made of various filaments, including alpha-synuclein

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10
Q

MPTP

A

-toxin that inhibits complex I –> leads to ROS accumulation –> mt membrane permeability –> apoptosis OR the inhibition of the u-p system –> lewy bodies

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11
Q

Theoretical Tx of PD

A
  1. Anti-oxidants to combat the ROS –> not successful
  2. Co-enzyme Q –> to promote mito integrity
  3. Dopamine agonists –> to prevent apoptosis )Pramipexole is in trials right now)

**aim to prevent the cause of the disease

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12
Q

Diffuse lewy body disease

A
  • Dementia
  • Relatively common
  • Parkinsonian features, but more rapid course than classic PD
  • find lewy bodies in cerebral cortex and brainstem
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13
Q

Progressive supranuclear palsy

A
  • sometimes called atypical PD
  • onset 40-60 yrs
  • more common in men
  • Progressive
  • fatal in 5 years
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14
Q

Progressive supranuclear palsy: sx

A
  1. Vacant stare
  2. Eye mvmnt disorders = vertical gaze palsy
  3. Rigidity, tremors, weak, spastic
  4. Dysarthria & dysphasia
  5. Dimentia
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15
Q

Psp pathology

A
  • widespread loss of neurons in the: cerebral cortex, globus pallidus,sub nigra, colliculi, periaqueductal grey, &brainstem nuclei
  • NFT found in neurons and glia = Huge, round
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16
Q

Globose inclusions

A
  • seen in Psp
  • large, bulbous
  • neurofibulary tangles
  • seen mainly in the brainstem
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17
Q

Huntington’s disease: inheritance & genetic

A
  • dominantly inherited
  • on chromosome 4
  • the gene codes for a polyglutamine region of the protein –> normally there are less than 30 CAG repeats,
  • in the disease use will get an increase in the number of glutamine segments –> the more repeats you have = the more severe the disease will be and the earlier the disease onset
  • this abnormal protein will, disrupt normal gene functions (ex. Transcription, transport, and transmission) –> the fctn of the normal gene is unknown
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18
Q

Huntingtons disease onset and sx

A
  • age 30-50 yrs
  • choreiform mvmnts
  • personality changes
  • dementia later
  • average survival is 15 yrs after Dx
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19
Q

Huntington disease pathology

A
  • widened ventricles

- atrophy of the BG, ESP caudate nucleus & putamen + cerebral cortex too

20
Q

Huntington and imaging

A

-can see effects of the disease on imaging, usually the disease is very advanced by the time the disease can be seen

21
Q

Huntington histology

A
  • see atrophy of the small GABA-ergic neurons

- replaced by glial cells = gliosis

22
Q

Huntingtin inclusions

A
  • mutant Huntingtin protein accumulates
  • seen within neurons intranuclearly!
  • can be seen ONLY with an immuno stain
22
Q

Cerebellar ataxias

A
  • invol tremor

- initiated or made worse by intentional mvmnt

23
Q

Friedreichs ataxia: onset, sx, & prognosis

A
  • onset in older children and young adults
  • staggering & ataxic gait
  • dysarthria & nystagmus
  • decreased vib sensory and prop is diminished
  • babinski responses and wkness
  • more prevalent in diabetics
  • 15 yr survival after Dx
24
Q

Friedreichs ataxia: genetics

A
  • recessive familial
  • mutation that causes frataxin production regulation to be abnormal
  • get abnormal synthesis of frataxin, there will be less frataxin
  • frataxin is on the mito membrane –> so this is prob a mito disease
25
Q

Fried ataxia: histology

A

-Cerebellar cortical atrophy of All three layers

26
Q

Spinal cord disease in fried

A
  • see long tract disease
  • damage to post and lat columns
  • this is where the decreaesed prop and vib sense come from
27
Q

Fried myocarditis

A

-pts get arrhythmias and CHF

28
Q

Spinocerebellar degenerations

A
  • there is a variety of these that effect both the cerebellum and spinal cord
  • usually Dom. Inherited
  • usually caused by increased CAG repeats
  • the genes involved code for polyglutamine portions of different proteins
29
Q

Motor diseases primary feature

A

-weakness

30
Q

Motor neuron diseases

A
  • see atrophy in the muscles –> distal first
  • atrophy is neurogenic –> due to a loss in trophic influence
  • most cases are idiopathic
31
Q

Sporadic ALS

A
  • sclerosis = gliosis
  • usually don’t know the cause
  • see TDP-43 inclusions in the cytoplasm
32
Q

TDP-43

A
  • plays a role in sporadic ALS
  • this protein probably plays a role in preventing apop, so when it is abnormal there will be more apop of neurons and more neurons will be lost!
  • TDP-43 inclusions seen as Truncated, hyperphosphorylated fragments in the cytoplasm of sporadic ALS pts
33
Q

ALS: onset, clinical features, and prognosis

A
  • onset 30-60 yrs
  • involves both UMN &LMN
  • have muscular wkness that usually begins distally w/ atrophy and fasiculations –> BUT facial m. are spared from atrophy!!
  • have cortical and spinal cord involvement
  • Progressive course with 3-5 year survival
34
Q

ALS and the spinal cord

A
  • the anterior roots are targeted and become smaller

- pyramidal tracts are diminished = wallerian degeneration

35
Q

ALS histology

A
  • central chromatolysis = reactive change, pre-necrotic change
  • loss of motor neurons
  • see ALS-Bunina bodies on the mn.s
36
Q

Bunina bodies

A
  • cyto inclusions
  • round
  • red
  • well defined
  • autophagic vacuoles
  • suggests that thaese nare markers for the degenrations of the neurons
37
Q

Poliomyelitis: sx

A

-acute paralysis: Legs>arms, Sometimes bulbar or respiratory muscles are involved

38
Q

Polio histology

A

-looks like other viral CNS diseases
-see lots of inflam
-see:
Neuronophagia, lymphocytic cuffing

39
Q

Polio prognosis

A
  • someyptimes do not fully recover becuase there was extensive neuron destruction
  • Most (80%) patients recover within 6 months –> but pt is usually left with leg wkness
40
Q

Degenerative diseases: what are they and what are they characterized by?

A
  • many are hereditary/genetic
  • often begin as nerve cell disease
  • Characterized by:
    1. atrophy
    2. neuronal loss
    3. gliosis
41
Q

Extrapyramidal diseases: 3 sx

A
  1. Random, involuntary, purposeless movements
  2. Tremors
  3. Rigidity
42
Q

Neuroimaging and PD

A
  • PET scanning shows diagnostic findings
  • Use 18 fluorodopa and measure brain uptake
  • Find signal loss in putamen in PD
  • Test is based on loss of substantia nigra (dopaminergic) projections to corpus striatum–> but these changes are not usually seen until later in the disease, so these tests are not really for Dx
43
Q

Involuntary movements/ataxia: what is it & causes

A
  • Cerebellum is Involved
  • Causes:
    1. Friedrich’s ataxia
    2. Spinocerebellar ataxias (atrophies)
  • Often hereditary
  • Slowly progressive disease
44
Q

Neutrophic Poliomyelitis: onset and cause

A
  • caused by picornavirus
  • onset=older children and young adults –> This is when virus is neurotropic!
  • get damage to the LMN b/c the picornavirus gets to the Motor neurons, kills them and causes an encephalitis that involves the sc

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