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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?

A

PSP

25
Q

Neurochemical features of PSP

A

Striatal Dopamine Deficiency

26
Q

Multiple System Atrophy typical age of onset

A

50-55

27
Q

Life expectancy in MSA

A

5-10 years

28
Q

What does MSA look like initially?

A

A lot like PSP…rigidity and bradykinesia first, balance and postural instability come on fairly early as well, tremor is unusual

29
Q

Clinical hallmark of MSA

A

Progressive autonomic dysfunction

  • Orthostatic HTN most widely recognized
  • Urinary dysfunction
  • Impotence
30
Q

Glial cytoplasmic inclusion bodies positive for synuclein also seen in MSA

A

yeah

31
Q

Huntington’s dz is genetic or non-genetic?

A

Genetic

32
Q

What is the genetic mutation in huntington’s disease and where does it reside?

A

HTT gene, resides on the short arm of chromosome 4

33
Q

The mutation in huntington’s disease is a trinucleotide repeat (CAG)

A

ok

34
Q

What is the typical age of onset of Huntington’s dz?

A

35-45

35
Q

Life expectancy after symptom onset in huntingtons

A

10-20 years

36
Q

What is teh neurologic hallmark of huntingtons

A

CHorea— jerky dance like movement

37
Q

How do the symptoms of early onset huntingtons differ from late onset?

A

Early onset Huntington’s disease is characterized by the onset of parkinsonism rather than chorea right from the onset

38
Q

WHat type os behavioral changes occur in HD?

A
  • Personality changes (impulsiveness, irritability, obsessive behavior, aggression)
  • Depression
  • Dementia
39
Q

Tourette’s syndrome average age of onset and sexual disposition

A

male predominance, symptom onset between 2-15yoa

40
Q

Tourette’s syndromes often diminish when?

A

adulthood

41
Q

Diagnostic criteria of tourettes

A

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
Q

What are some of the behavioral features of Tourettes

A

ADHD in around half of those affected.

OCD in 30-50%

43
Q

What is dystonia

A

sustained muscle contraction that produces sustained and sometime repititious twisting movements that result in abnormal postures.

44
Q

Dystonia can be classified as?

A

Primary or secondary

Focal or generalized

45
Q

Primary Generalzed Dystonia cause

A

Mutation of DYT1 gene on chromosome 9, GAG deletion and altered Torsin A protein

46
Q

Primary Generalized dystonia is most common in families with what kind of heritage?

A

Ashkenazi jews

47
Q

Inheritance pattern of Primary Generalized Dystonia?

A

It is autosomal dominant but ht epenetrance is only 30-40% which means that most individuals with the disease may never develop dystonia

48
Q

Onset of primary genralized dystonia is usually focal

A

ok

49
Q

Dromedary gait?

A

Seen with PGD

50
Q

In contrast to generalized dystonia, when does focal dystonia appear

A

adulthood

51
Q

Focal dystonia generally affects muscles where

A

above the waist

52
Q

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.

A

Sensory tricks can sometimes alleviate the dystonia.

53
Q

Wilson’s disease mutation

A

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
Q

Most common neurological manifestation of Wilson;s disease is

A

Tremor

55
Q

Psychiatric features of Wilson’s disease

A

personality change, depression, mania, psychosis, etc..

56
Q

Opthalmologic abnormalities with WD

A

Kayser Fleischer rings and sunflower cataracts

57
Q

Oplaski cell

A

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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