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Clinical Neuroscience weeks 1-3 > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

Disorders that result in too much, or unwanted/distorted, movement are labelled

A

Hyperkinetic

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2
Q

Disorders that result in too little movement are labelled

A

Hypokinetic

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3
Q

Parkinson’s disease is labeled what type of movement disorder

A

Hypokinetic

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4
Q

Parkinson’s is the second most common neurodegenerative disorder behind alzheimers

A

ok

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5
Q

prevalence of parkinsons

A

100 per 100,000

But 2% of ppl over 60.

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6
Q

Young onset parkinson disease likely to be genetic in origin

A

ok

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7
Q

Typical age of onset between

A

55-65

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8
Q

Motor features of PD are initially….

A

Asymmetric

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9
Q

4 cardinal motor features of parkinsons

A
  • Tremor at rest
  • Rigidity
  • Bradykinesia
  • Postural instability late
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10
Q

If you see an individual with parkinsonism develop postural instability early, what do you think…

A

Not Parkinson dz most likely

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11
Q

Other PD features

A
  • Masked face
  • Diminished blink
  • stooped posture
  • small shuffling gait
  • reduced armswing
    hypokinetic dysarthria
    micrographia
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12
Q

Behavior features of PD

A
  • Depression
  • Anxiety
  • Cognitive impairment in PD is characterized by executive dysfunction…individuals have trouble making decisions and carrying out plans
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13
Q

Autonomic dysfunction and PD

A
  • GI dysfunction in the form of constipation
  • Urinary dysfunction
  • Dysphagia (trouble swallowing)
  • Erectile dysfunction
  • Impaired BP regulation
  • Orthostatic HTN
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14
Q

Other features of parkinsons

A
  • Impairment of olfaction
  • Blurred Vision
  • Pain in the shoulder
  • Sleep fragmentation
  • REM sleep behavior disorder in which individuals retain the ability to move during dreaming
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15
Q

Classic pathologic abnormality is

A
  • Degeneration of Pigmented Neurons which have their cell body in the substantia nigra pars compacta
  • microscopically, cytoplasmic inclusion bodies called Lewy bodies can be seen in the surviving pigmented neurons within the substantia nigra and elsewhere.
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16
Q

Neurochemical hallmark of PD=

A

Dopamine deficiency

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17
Q

Age of onset of Progressive Supranuclear Palsy?

A

50-60 (five years younger that PD

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18
Q

Life expectancy of pt with PSP

A

10 years

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19
Q

Parkinsonian features of PSP

A
  • mostly rigidity and bradykinesia

Axial musculature is especially likely to be involved (may sometimes result in neck extensions)

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20
Q

What is the facial masking like in PSP

A

ASTONISHED

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21
Q

Gait and balance disturbances along with postural instability appear when in PSP

A

Early- thats how you differentiate from PD

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22
Q

Tremor is generally seen in PSP?

A

NO!!! unusual

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23
Q

What is the feature of PSP that ultimately permits diagnosis?

A

Impairment of downgaze, then up, then over….PD usually shows impairment of upgaze.

24
Q

Neurofibrillary tangles?

25
Neurochemical features of PSP
Striatal Dopamine Deficiency
26
Multiple System Atrophy typical age of onset
50-55
27
Life expectancy in MSA
5-10 years
28
What does MSA look like initially?
A lot like PSP...rigidity and bradykinesia first, balance and postural instability come on fairly early as well, tremor is unusual
29
Clinical hallmark of MSA
Progressive autonomic dysfunction - Orthostatic HTN most widely recognized - Urinary dysfunction - Impotence
30
Glial cytoplasmic inclusion bodies positive for synuclein also seen in MSA
yeah
31
Huntington's dz is genetic or non-genetic?
Genetic
32
What is the genetic mutation in huntington's disease and where does it reside?
HTT gene, resides on the short arm of chromosome 4
33
The mutation in huntington's disease is a trinucleotide repeat (CAG)
ok
34
What is the typical age of onset of Huntington's dz?
35-45
35
Life expectancy after symptom onset in huntingtons
10-20 years
36
What is teh neurologic hallmark of huntingtons
CHorea--- jerky dance like movement
37
How do the symptoms of early onset huntingtons differ from late onset?
Early onset Huntington's disease is characterized by the onset of parkinsonism rather than chorea right from the onset
38
WHat type os behavioral changes occur in HD?
- Personality changes (impulsiveness, irritability, obsessive behavior, aggression) - Depression - Dementia
39
Tourette's syndrome average age of onset and sexual disposition
male predominance, symptom onset between 2-15yoa
40
Tourette's syndromes often diminish when?
adulthood
41
Diagnostic criteria of tourettes
must have multiple motor tics and at least one vocal tic present at some point throughout the day. Tics must occur many times a day, every day, or intermittently over the course of a year, Must have no tic free period of greater than 3 mos. Onset prior to 18
42
What are some of the behavioral features of Tourettes
ADHD in around half of those affected. | OCD in 30-50%
43
What is dystonia
sustained muscle contraction that produces sustained and sometime repititious twisting movements that result in abnormal postures.
44
Dystonia can be classified as?
Primary or secondary | Focal or generalized
45
Primary Generalzed Dystonia cause
Mutation of DYT1 gene on chromosome 9, GAG deletion and altered Torsin A protein
46
Primary Generalized dystonia is most common in families with what kind of heritage?
Ashkenazi jews
47
Inheritance pattern of Primary Generalized Dystonia?
It is autosomal dominant but ht epenetrance is only 30-40% which means that most individuals with the disease may never develop dystonia
48
Onset of primary genralized dystonia is usually focal
ok
49
Dromedary gait?
Seen with PGD
50
In contrast to generalized dystonia, when does focal dystonia appear
adulthood
51
Focal dystonia generally affects muscles where
above the waist
52
Some focal dystonias are task specific in that they only become active when the affected individual is performing a certain motor action or task such as writing or playing the guitar.
Sensory tricks can sometimes alleviate the dystonia.
53
Wilson's disease mutation
long arm of chromosome 13, ATP7B gene ( the product of this gene is a copper transporting ATPase that generally helps form ceruloplasmin in the liver). Copper accumulates in the liver and ultimately is deposited in organs like the brain.
54
Most common neurological manifestation of Wilson;s disease is
Tremor
55
Psychiatric features of Wilson's disease
personality change, depression, mania, psychosis, etc..
56
Opthalmologic abnormalities with WD
Kayser Fleischer rings and sunflower cataracts
57
Oplaski cell
ALtered glial cell that can be found in basal gangla of individuals with WD.

Clinical Neuroscience weeks 1-3 (26 decks)

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