Movement Disorders Flashcards Preview

Clinical Neuroscience weeks 1-3 > Movement Disorders > Flashcards

Flashcards in Movement Disorders Deck (57):
1

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

Hyperkinetic

2

Disorders that result in too little movement are labelled

Hypokinetic

3

Parkinson's disease is labeled what type of movement disorder

Hypokinetic

4

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

ok

5

prevalence of parkinsons

100 per 100,000

But 2% of ppl over 60.

6

Young onset parkinson disease likely to be genetic in origin

ok

7

Typical age of onset between

55-65

8

Motor features of PD are initially....

Asymmetric

9

4 cardinal motor features of parkinsons

- Tremor at rest
- Rigidity
- Bradykinesia
- Postural instability late

10

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

Not Parkinson dz most likely

11

Other PD features

- Masked face
- Diminished blink
- stooped posture
- small shuffling gait
- reduced armswing
hypokinetic dysarthria
micrographia

12

Behavior features of PD

-Depression
- Anxiety
- Cognitive impairment in PD is characterized by executive dysfunction...individuals have trouble making decisions and carrying out plans

13

Autonomic dysfunction and PD

- GI dysfunction in the form of constipation
- Urinary dysfunction
- Dysphagia (trouble swallowing)
- Erectile dysfunction
- Impaired BP regulation
- Orthostatic HTN

14

Other features of parkinsons

- 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

15

Classic pathologic abnormality is

- 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.

16

Neurochemical hallmark of PD=

Dopamine deficiency

17

Age of onset of Progressive Supranuclear Palsy?

50-60 (five years younger that PD

18

Life expectancy of pt with PSP

10 years

19

Parkinsonian features of PSP

- mostly rigidity and bradykinesia
Axial musculature is especially likely to be involved (may sometimes result in neck extensions)

20

What is the facial masking like in PSP

ASTONISHED

21

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

Early- thats how you differentiate from PD

22

Tremor is generally seen in PSP?

NO!!! unusual

23

What is the feature of PSP that ultimately permits diagnosis?

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

24

Neurofibrillary tangles?

PSP

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.