NCS Exam
What is Huntington's Disease? - answer inherited disorder, malfunction of Huntington
gene located on chromosome 4 - repeat of CAG triplets
What is the pathology of HD? - answer 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? - answer 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? - answer Loss of
striatal projection neurons of the direct D1 pathway as well as cortical neurons
When does juvenile onset of HD occur? - answerBefore age 20
What characterizes the difference between juvenile onset HD and typical HD? -
answerEarly 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? -
answerAntichoreic/anti-psychotics and anti-depressants
What are some examples of antichoreic/anti-psychotics and anti-depressants used to
manage HD? - answerAntichoreic/anti-psychotics: tetrabenazine, olanzpine, risperdal,
quetiapine/seroquel, ziiprasidone/geodon, aripiprazole/abilify, haldol,
fluphenazine/prolixin
What outcome measures are recommended for use with HD? - answerUHDRS
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? - answerEach item 0-4 where 0 =
normal, 4 = severe
,Total score possible 124
How are HD stages determined? (Using what scale?) - answerTotal Functional Capacity
Scale
How is the TFCS scored? - answerHigher scores are better (more functional); can score
up to a 13
What TFCS equates to early stage HD? - answerStage 1
What TFCS equates to middle stage HD? - answerStage II and III
What TFCS equates to late stage HD? - answerStage IV and V
When should you use the FSST with HD patients? Why? - answerEarly stages,
because of cognitive impairments in later stages
What cutoff score for the Tinneti predicts falls in patients with HD? - answerLess than
21
How do you measure Dual Task Cost? - answer(Dual task score - single task score /
single task score) x 100
What should treatment be focused on in the early stages of HD? - answerAerobic
exercise (walking), flexibility, strengthening postural muscles (quadruped activities),
dual tasking
What should treatment be focused on in the middle stages of HD? - answerDual 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? - answerRollator walker (4WW)
What should treatment be focused on in the late stages of HD? - answerPreventing
things like skin breakdown, training caregiver on transfers, respiratory issues, etc.
Can you use motor imagery to improve performance on motor tasks in HD? -
answerYes
What is ALS? - answerA progressive degeneration and loss of motor neurons in the
spinal cord, brain stem, and motor cortex
When does ALS onset? - answer50s-60s
When does familial ALS onset? - answer40s-50s
, What is the average time from diagnosis to death in ALS? - answer2-10 years
Men are more affected than women in all but what form of ALS? - answerBulbar onset
ALS is more common in older females
What are the types of ALS? - answerClassic ALS, primary lateral sclerosis, progressive
spinal muscular atrophy, bulbar onset, pseudobulbar, ALS with frontotemporal dementia
Which types of ALS present with UMN signs? - answerclassic ALS, primary lateral
sclerosis, pseudobulbar
Which types of ALS present with LMN signs? - answerClassic ALS, progressive spinal
muscular atrophy, bulbar palsy
What is a disease-modifying drug available to treat ALS? - answerRiluzole
What does Riluzole do? - answerDecreases glutamate release
What are drugs to treat symptoms of ALS? - answerCramps (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? - answerALS 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? -
answerNo because although they have muscular weakness, they have intact sensation
What kind of strengthening should be emphasized in ALS? - answerConcentric at
moderate resistance and intensity in antigravity muscles
What are some general exercise guidelines for people with ALS? - answerModerate
intensity aerobic activity, encourage participation in sports
About 3 times per week with rest periods, especially if activity is longer than 15 minutes
What is the cutoff score on the TUG for predicting falls in individuals with ALS? -
answer14 seconds
What is Huntington's Disease? - answer inherited disorder, malfunction of Huntington
gene located on chromosome 4 - repeat of CAG triplets
What is the pathology of HD? - answer 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? - answer 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? - answer Loss of
striatal projection neurons of the direct D1 pathway as well as cortical neurons
When does juvenile onset of HD occur? - answerBefore age 20
What characterizes the difference between juvenile onset HD and typical HD? -
answerEarly 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? -
answerAntichoreic/anti-psychotics and anti-depressants
What are some examples of antichoreic/anti-psychotics and anti-depressants used to
manage HD? - answerAntichoreic/anti-psychotics: tetrabenazine, olanzpine, risperdal,
quetiapine/seroquel, ziiprasidone/geodon, aripiprazole/abilify, haldol,
fluphenazine/prolixin
What outcome measures are recommended for use with HD? - answerUHDRS
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? - answerEach item 0-4 where 0 =
normal, 4 = severe
,Total score possible 124
How are HD stages determined? (Using what scale?) - answerTotal Functional Capacity
Scale
How is the TFCS scored? - answerHigher scores are better (more functional); can score
up to a 13
What TFCS equates to early stage HD? - answerStage 1
What TFCS equates to middle stage HD? - answerStage II and III
What TFCS equates to late stage HD? - answerStage IV and V
When should you use the FSST with HD patients? Why? - answerEarly stages,
because of cognitive impairments in later stages
What cutoff score for the Tinneti predicts falls in patients with HD? - answerLess than
21
How do you measure Dual Task Cost? - answer(Dual task score - single task score /
single task score) x 100
What should treatment be focused on in the early stages of HD? - answerAerobic
exercise (walking), flexibility, strengthening postural muscles (quadruped activities),
dual tasking
What should treatment be focused on in the middle stages of HD? - answerDual 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? - answerRollator walker (4WW)
What should treatment be focused on in the late stages of HD? - answerPreventing
things like skin breakdown, training caregiver on transfers, respiratory issues, etc.
Can you use motor imagery to improve performance on motor tasks in HD? -
answerYes
What is ALS? - answerA progressive degeneration and loss of motor neurons in the
spinal cord, brain stem, and motor cortex
When does ALS onset? - answer50s-60s
When does familial ALS onset? - answer40s-50s
, What is the average time from diagnosis to death in ALS? - answer2-10 years
Men are more affected than women in all but what form of ALS? - answerBulbar onset
ALS is more common in older females
What are the types of ALS? - answerClassic ALS, primary lateral sclerosis, progressive
spinal muscular atrophy, bulbar onset, pseudobulbar, ALS with frontotemporal dementia
Which types of ALS present with UMN signs? - answerclassic ALS, primary lateral
sclerosis, pseudobulbar
Which types of ALS present with LMN signs? - answerClassic ALS, progressive spinal
muscular atrophy, bulbar palsy
What is a disease-modifying drug available to treat ALS? - answerRiluzole
What does Riluzole do? - answerDecreases glutamate release
What are drugs to treat symptoms of ALS? - answerCramps (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? - answerALS 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? -
answerNo because although they have muscular weakness, they have intact sensation
What kind of strengthening should be emphasized in ALS? - answerConcentric at
moderate resistance and intensity in antigravity muscles
What are some general exercise guidelines for people with ALS? - answerModerate
intensity aerobic activity, encourage participation in sports
About 3 times per week with rest periods, especially if activity is longer than 15 minutes
What is the cutoff score on the TUG for predicting falls in individuals with ALS? -
answer14 seconds
