NCS EXAM QUESTIONS AND
ANSWERS
What is Huntington's Disease? - Correct Answers -inherited disorder, malfunction of
Huntington gene located on chromosome 4 - repeat of CAG triplets
What is the pathology of HD? - Correct Answers -severe loss of neurons in the caudate
and putamen with subsequent astrocytosis; head of caudate becomes shrunken and
there is dilatation of the anterior horns of lateral ventricles; also see neuronal
degeneration within the temporal and frontal lobes of the cerebral cortex, globus
pallidus, thalamus, subthalamic nucleus, substantial nigra, and cerebellum
Why does HD produce hyperkinetic movements in early stage? - Correct Answers -
Because of loss of projection neurons in D2 pathway, causing disinhibition of the
thalamus, which excites the cortex
Why does HD produce hypokinetic movements in the late stage? - Correct Answers -
Loss of striatal projection neurons of the direct D1 pathway as well as cortical neurons
When does juvenile onset of HD occur? - Correct Answers -Before age 20
What characterizes the difference between juvenile onset HD and typical HD? - Correct
Answers -Early phase involves bradykinesia, rigidity, tremor, and myoclonus similar to
PD; can have seizures and mental disability; faster disease progression (10 years vs.
19 years)
What are the two main classes of medications involved in managing HD? - Correct
Answers -Antichoreic/anti-psychotics and anti-depressants
What are some examples of antichoreic/anti-psychotics and anti-depressants used to
manage HD? - Correct Answers -Antichoreic/anti-psychotics: tetrabenazine, olanzpine,
risperdal, quetiapine/seroquel, ziiprasidone/geodon, aripiprazole/abilify, haldol,
fluphenazine/prolixin
What outcome measures are recommended for use with HD? - Correct Answers -
UHDRS
Total Functional Capacity Scale
Single Limb Stance
Reactive Pull Test
,6MWT, TUG, 10MWT, BBS, Tinetti
ABC, 4SST
5xSTS
How is the UHDRS motor assessment scored? - Correct Answers -Each item 0-4 where
0 = normal, 4 = severe
Total score possible 124
How are HD stages determined? (Using what scale?) - Correct Answers -Total
Functional Capacity Scale
How is the TFCS scored? - Correct Answers -Higher scores are better (more
functional); can score up to a 13
What TFCS equates to early stage HD? - Correct Answers -Stage 1
What TFCS equates to middle stage HD? - Correct Answers -Stage II and III
What TFCS equates to late stage HD? - Correct Answers -Stage IV and V
When should you use the FSST with HD patients? Why? - Correct Answers -Early
stages, because of cognitive impairments in later stages
What cutoff score for the Tinneti predicts falls in patients with HD? - Correct Answers -
Less than 21
How do you measure Dual Task Cost? - Correct Answers -(Dual task score - single task
score / single task score) x 100
What should treatment be focused on in the early stages of HD? - Correct Answers -
Aerobic exercise (walking), flexibility, strengthening postural muscles (quadruped
activities), dual tasking
What should treatment be focused on in the middle stages of HD? - Correct Answers -
Dual tasking if still having a response to treatment, functional task-specific training
(STS, stairs, fall recovery, etc.)
What is the best AD to use with HD? - Correct Answers -Rollator walker (4WW)
What should treatment be focused on in the late stages of HD? - Correct Answers -
Preventing things like skin breakdown, training caregiver on transfers, respiratory
issues, etc.
Can you use motor imagery to improve performance on motor tasks in HD? - Correct
Answers -Yes
, What is ALS? - Correct Answers -A progressive degeneration and loss of motor
neurons in the spinal cord, brain stem, and motor cortex
When does ALS onset? - Correct Answers -50s-60s
When does familial ALS onset? - Correct Answers -40s-50s
What is the average time from diagnosis to death in ALS? - Correct Answers -2-10
years
Men are more affected than women in all but what form of ALS? - Correct Answers -
Bulbar onset ALS is more common in older females
What are the types of ALS? - Correct Answers -Classic ALS, primary lateral sclerosis,
progressive spinal muscular atrophy, bulbar onset, pseudobulbar, ALS with
frontotemporal dementia
Which types of ALS present with UMN signs? - Correct Answers -classic ALS, primary
lateral sclerosis, pseudobulbar
Which types of ALS present with LMN signs? - Correct Answers -Classic ALS,
progressive spinal muscular atrophy, bulbar palsy
What is a disease-modifying drug available to treat ALS? - Correct Answers -Riluzole
What does Riluzole do? - Correct Answers -Decreases glutamate release
What are drugs to treat symptoms of ALS? - Correct Answers -Cramps (quinine,
Dilantin, Valium, baclofen)
Spasticity (baclofen, Valium, antrolene) Sialorrhea (elavil, Robinul, atropine,
scopolamine, Botox)
Sleep disturbance (elavil, Valium)
Depression (elavil, Prozac, Zoloft)
Excessive laughing and crying (elavil, Paxil, Zoloft)
What are some outcome measures specific to ALS? - Correct Answers -ALS Functional
Rating Scale
Schwab and England Scale of Capacity for Daily Living
ALS assessment questionnaire
ALS specific QoL instrument
Will ambulatory individuals with ALS show increased postural sway in static standing? -
Correct Answers -No because although they have muscular weakness, they have intact
sensation
ANSWERS
What is Huntington's Disease? - Correct Answers -inherited disorder, malfunction of
Huntington gene located on chromosome 4 - repeat of CAG triplets
What is the pathology of HD? - Correct Answers -severe loss of neurons in the caudate
and putamen with subsequent astrocytosis; head of caudate becomes shrunken and
there is dilatation of the anterior horns of lateral ventricles; also see neuronal
degeneration within the temporal and frontal lobes of the cerebral cortex, globus
pallidus, thalamus, subthalamic nucleus, substantial nigra, and cerebellum
Why does HD produce hyperkinetic movements in early stage? - Correct Answers -
Because of loss of projection neurons in D2 pathway, causing disinhibition of the
thalamus, which excites the cortex
Why does HD produce hypokinetic movements in the late stage? - Correct Answers -
Loss of striatal projection neurons of the direct D1 pathway as well as cortical neurons
When does juvenile onset of HD occur? - Correct Answers -Before age 20
What characterizes the difference between juvenile onset HD and typical HD? - Correct
Answers -Early phase involves bradykinesia, rigidity, tremor, and myoclonus similar to
PD; can have seizures and mental disability; faster disease progression (10 years vs.
19 years)
What are the two main classes of medications involved in managing HD? - Correct
Answers -Antichoreic/anti-psychotics and anti-depressants
What are some examples of antichoreic/anti-psychotics and anti-depressants used to
manage HD? - Correct Answers -Antichoreic/anti-psychotics: tetrabenazine, olanzpine,
risperdal, quetiapine/seroquel, ziiprasidone/geodon, aripiprazole/abilify, haldol,
fluphenazine/prolixin
What outcome measures are recommended for use with HD? - Correct Answers -
UHDRS
Total Functional Capacity Scale
Single Limb Stance
Reactive Pull Test
,6MWT, TUG, 10MWT, BBS, Tinetti
ABC, 4SST
5xSTS
How is the UHDRS motor assessment scored? - Correct Answers -Each item 0-4 where
0 = normal, 4 = severe
Total score possible 124
How are HD stages determined? (Using what scale?) - Correct Answers -Total
Functional Capacity Scale
How is the TFCS scored? - Correct Answers -Higher scores are better (more
functional); can score up to a 13
What TFCS equates to early stage HD? - Correct Answers -Stage 1
What TFCS equates to middle stage HD? - Correct Answers -Stage II and III
What TFCS equates to late stage HD? - Correct Answers -Stage IV and V
When should you use the FSST with HD patients? Why? - Correct Answers -Early
stages, because of cognitive impairments in later stages
What cutoff score for the Tinneti predicts falls in patients with HD? - Correct Answers -
Less than 21
How do you measure Dual Task Cost? - Correct Answers -(Dual task score - single task
score / single task score) x 100
What should treatment be focused on in the early stages of HD? - Correct Answers -
Aerobic exercise (walking), flexibility, strengthening postural muscles (quadruped
activities), dual tasking
What should treatment be focused on in the middle stages of HD? - Correct Answers -
Dual tasking if still having a response to treatment, functional task-specific training
(STS, stairs, fall recovery, etc.)
What is the best AD to use with HD? - Correct Answers -Rollator walker (4WW)
What should treatment be focused on in the late stages of HD? - Correct Answers -
Preventing things like skin breakdown, training caregiver on transfers, respiratory
issues, etc.
Can you use motor imagery to improve performance on motor tasks in HD? - Correct
Answers -Yes
, What is ALS? - Correct Answers -A progressive degeneration and loss of motor
neurons in the spinal cord, brain stem, and motor cortex
When does ALS onset? - Correct Answers -50s-60s
When does familial ALS onset? - Correct Answers -40s-50s
What is the average time from diagnosis to death in ALS? - Correct Answers -2-10
years
Men are more affected than women in all but what form of ALS? - Correct Answers -
Bulbar onset ALS is more common in older females
What are the types of ALS? - Correct Answers -Classic ALS, primary lateral sclerosis,
progressive spinal muscular atrophy, bulbar onset, pseudobulbar, ALS with
frontotemporal dementia
Which types of ALS present with UMN signs? - Correct Answers -classic ALS, primary
lateral sclerosis, pseudobulbar
Which types of ALS present with LMN signs? - Correct Answers -Classic ALS,
progressive spinal muscular atrophy, bulbar palsy
What is a disease-modifying drug available to treat ALS? - Correct Answers -Riluzole
What does Riluzole do? - Correct Answers -Decreases glutamate release
What are drugs to treat symptoms of ALS? - Correct Answers -Cramps (quinine,
Dilantin, Valium, baclofen)
Spasticity (baclofen, Valium, antrolene) Sialorrhea (elavil, Robinul, atropine,
scopolamine, Botox)
Sleep disturbance (elavil, Valium)
Depression (elavil, Prozac, Zoloft)
Excessive laughing and crying (elavil, Paxil, Zoloft)
What are some outcome measures specific to ALS? - Correct Answers -ALS Functional
Rating Scale
Schwab and England Scale of Capacity for Daily Living
ALS assessment questionnaire
ALS specific QoL instrument
Will ambulatory individuals with ALS show increased postural sway in static standing? -
Correct Answers -No because although they have muscular weakness, they have intact
sensation
