ALS, MS, Parkinson's, Huntington's

Question 1

What are some S&S for ALS?

Answer 1

progressive weakness, atrophy
mm fasciculations in limbs and trunk
hyperreflexia, spasticity
(+) Babinski sign
difficulty speaking (bulbar mm)
difficulty swallowing
respiratory failure
inappropriate emotional responses

Question 2

What is clinically isolated syndrome MS?

Answer 2

a single attack of neurological symptoms though multiple brain lesions are likely observed - may or may not go on to develop progressive MS

Question 3

What is relapsing-remitting MS? (RRMS)

Answer 3

MS where there are clearly defined attacks and remissions

75%

Question 4

What is the most common form of MS?

Answer 4

relapsing-remitting (or attack-remitting)

75%

Question 5

What is benign MS? 10-15%

Answer 5

a subtype of RRMS where remission is almost complete - only minimal disability 10-15 years after onset

Question 6

In most cases of benign MS, what is affected mainly?

Answer 6

sight and or/touch (sensory)

Question 7

True or false: benign MS will always be benign MS.

Answer 7

false. it will no longer be MS the longer post-onset

Question 8

Describe progressive MS…

Answer 8

disability slowly and continuously increases with or without relapses

Question 9

What is primary-progressive MS? 10-15%

Who is usually affected?

Answer 9

nearly continuous worsening of MS from the beginning with NO clear relapses or remissions

people in their 40s - men and women equally (the only type that is equal)

Question 10

People with relapsing-remitting MS tend to eventually develop….

Answer 10

secondary progressive MS

Question 11

What does secondary-progressive MS refer to?

Answer 11

MS where there may or may not be relapses/remissions, but in remissions, the recovery is usually not complete - disability accumulates over time

Question 12

What is the average life expectancy after the onset of MS?

Answer 12

25-30 years

Question 13

What are the three symptom families for Huntington’s Disease?

Answer 13

abnormal movement, personality changes, dementia

Question 14

What is the risk of a child being born if one parent has Huntington’s?

Answer 14

50%

Question 15

Huntington’s destroys neurons mainly in the _______ and lowers levels of which neurotransmitters?

Answer 15

basal ganglia

GABA
ACh
Metenkephalin

Question 16

Huntington’s leaves HIGHER levels of what neurotransmitters?

Answer 16

dopamine and NE

Question 17

Huntington’s lowers levels of…

Answer 17

GABA, ACh, and metenkephalin

Question 18

Who are the basal ganglia?

Answer 18

caudate nucleus, putamen, globus pallidus, substantia nigra

Question 19

What causes the choreic movements in Huntington’s?

Answer 19

high levels of dopamine overstimulate the thalamocortical pathway

Question 20

In later stages of Huntington’s, what happens as a result of the loss of inhibition of thalamocortical output?

Answer 20

rigidity, bradykinesia

Question 21

What are some S&S of Huntington’s?

Answer 21

bradykinesia
chorea
dysarthria
dysphagia
cachexia
sleep disorders
incontinence
freezing phenomenon

Question 22

What is Parkinson’s?

Answer 22

a progressive disorder with diminishing basal ganglia function, leading to slow, difficult movement with resting tremors and muscular rigidity

Question 23

True or false: Parkinson’s S&S are always BL>

Answer 23

false. unilateral in stage 1!

Question 24

Differentiate the stages of Parkinson’s

Answer 24

1 - unilateral S&S
2 - bilateral S&S - moderate remor, rigidity and bradykinesia
3 - significant tremors, rigidity and bradykinesia; dystonia & freezing
4 - severe bradykinesia, impaired gait, assistance with ADLs required
5 - functional independence lost, no mobility

Question 25

What is cogwheel rigidity?

Answer 25

muscle rigidity with pROM (ratchet-like movement)

Question 26

What condition sees a pill rolling tremor?

Answer 26

Parkinsons

Question 27

What is festinating gait?

Answer 27

strides become quicker and shorter than normal, which can make it look like you’re hurrying
taking jerky steps.
moving your arms less when walking.
falling frequently

Question 28

What is retropulsion?

Answer 28

a loss of backward balance (can’t stop self from falling backward)

Question 29

What is a Katharine Hepburn tremor?

Answer 29

tremor in people who are older, have had head trauma, but don’t have Parkinson’s

Question 30

Give an example of ANS dysfunction in someone with Parkinson’s

Answer 30

very dry skin but very oily facial skin

Question 31

ACCORDING TO RATTRAY, life expectancy after Parksinons diagnosis is…

Answer 31

15-20 years from onset