NCS Exam || with Complete Solutions.
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
What kind of strengthening should be emphasized in ALS? correct answers Concentric at
moderate resistance and intensity in antigravity muscles
What are some general exercise guidelines for people with ALS? correct answers Moderate
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? correct
answers 14 seconds
What is a special consideration in individuals with ALS? What should be assessed? correct
answers Respiratory capacity - assess forced vital capacity (FVC)
What percentage of PD cases are idiopathic? correct answers 78%
What can cause secondary Parkinsonism? correct answers Brain injury, infections, metabolic
abnormalities, drug-induced
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
What kind of strengthening should be emphasized in ALS? correct answers Concentric at
moderate resistance and intensity in antigravity muscles
What are some general exercise guidelines for people with ALS? correct answers Moderate
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? correct
answers 14 seconds
What is a special consideration in individuals with ALS? What should be assessed? correct
answers Respiratory capacity - assess forced vital capacity (FVC)
What percentage of PD cases are idiopathic? correct answers 78%
What can cause secondary Parkinsonism? correct answers Brain injury, infections, metabolic
abnormalities, drug-induced
