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Where is the lesion site in ataxic dysarthria, as found in Friedreich’s ataxia?

(a) Extrapyramidal system
(b) Bilateral upper motor neuron
(c) Cerebellum
(d) Multiple sites

Answer: (c)
Commentary: Types of dysarthria include spastic, most commonly found in cerebral palsy; hypokinetic, as in Parkinson’s disease; hyperkinetic, as in dystonia; ataxic, as in Friedrich’s ataxia; flaccid, as in bulbar palsy; and mixed, as in amyotrophic lateral sclerosis. The site of the causative lesion in spastic dysarthria is bilateral upper motor neuron; hypo- and hyperkinetic is extrapyramidal system; ataxic is cerebellum; flaccid is lower motor neuron; mixed is multiple sites.

Reference: Driver L, Ayyangar R, Van Tubbergen M. Language development in disorders of communication and oral motor function. In: Alexander MA, Matthews DJ, editors. Pediatric rehabilitation: principles and practice. 4th ed. New York: Demos Medical; 2010. p 58



. A negative prognostic sign in early multiple sclerosis is

(a) young age at onset.
(b) cerebellar signs.
(c) optic neuritis.
(d) monosymptomatic presentation.

Answer: (b)
Commentary: Early multiple sclerosis shows a more favorable outcome if presentation is at age
less than 35 years, monosymptomatic, with optic signs, and of sudden onset with long remission.

Reference: Kraft G, Brown T, Johnson S. Multiple sclerosis. In: Braddom RL, editor. Physical
medicine and rehabilitation. 4th ed. Philadelphia: Elsevier-Saunders; 2011. p 1238



Which statement is TRUE of pseudodementia?

(a) Usually a history of previous psychiatric problems exists.
(b) Onset is indistinct with a long history of problems before consultation.
(c) Memory loss of recent items is worse than for remote items.
(d) Nocturnal accentuation of dysfunction is common.

Answer: (a)
Commentary: Distinguishing dementia from pseudodementia (which is really depression) is
important, in order to provide appropriate treatment to your patient with memory problems.
Pseudodementia’s onset is fairly well demarcated with a short history and is rapidly progressive
in nature. These patients usually have a history of a previous psychiatric difficulty or a recent life
crisis. Their complaints of cognitive dysfunction are detailed and elaborate with an affective
change and the patients expend little effort on examination items. Nocturnal exacerbations are
rare and memory loss is inconsistent in recall of recent and remote items.
Dementia’s onset, in contrast, is indistinct with a history of problems long before they seek
clinical help and early deficits often go unnoticed. A history of previous psychiatric problems or emotional crisis is uncommon. These patients struggle with cognitive tasks but usually put forth
good effort. Nocturnal dysfunction is common. The memory loss on recent items is worse than
for remote items and there is a consistent impairment of performance.



A negative prognostic sign in early multiple sclerosis is
(a) young age at onset.
(b) cerebellar signs.
(c) optic neuritis.
(d) monosymptomatic presentation.

Answer: (b)
Commentary: Early multiple sclerosis shows a more favorable outcome if presentation is at age less than 35 years, monosymptomatic, with optic signs, and of sudden onset with long remission.



Where is the lesion site in ataxic dysarthria, as found in Friedreich’s ataxia?
(a) Extrapyramidal system
(b) Bilateral upper motor neuron
(c) Cerebellum
(d) Multiple sites

Answer: (c)
Commentary: Types of dysarthria include spastic, most commonly found in cerebral palsy; hypokinetic, as in Parkinson’s disease; hyperkinetic, as in dystonia; ataxic, as in Friedrich’s ataxia; flaccid, as in bulbar palsy; and mixed, as in amyotrophic lateral sclerosis. The site of the causative lesion in spastic dysarthria is bilateral upper motor neuron; hypo- and hyperkinetic is extrapyramidal system; ataxic is cerebellum; flaccid is lower motor neuron; mixed is multiple sites.



A 42-year-old woman with multiple sclerosis comes to you describing profound afternoon
fatigue. You recommend
(a) tizanidine (Zanaflex).
(b) amantadine (Symmetrel).
(c) azathioprine (Imuran).
(d) glatiramer acetate (Copaxone).

Answer: (b)
Commentary: Many medications are indicated for multiple issues relating to multiple sclerosis
(MS). Medications for fatigue include amantadine (Symmetrel) and modafinil (Provigil).
Medications for use in exacerbations include prednisone, ACTH and Solu-Medrol. These drugs
are supposed to decrease the length and severity of exacerbation. Disease altering medications
including the interferons beta 1A and beta 1B as well as glatiramer acetate (Copaxone) can
decrease the number of exacerbations. Medications for chronic MS include cyclophosphamide
(Cytoxan), azathioprine (Imuran), and cyclosporine (Sandimmune), which are supposed to slow
progression in chronic MS. Medications for spasticity include baclofen (Lioresal), dantrolene(Dantrium), tizanidine (Zanaflex), and diazepam (Valium). Medications for ataxia can include
clonazepam (Klonopin) as well as isoniazid (Nydrazid).



Your patient is having difficulty writing or performing tasks in certain positions. He has an
asymmetric tremor that is present only with movement. You suspect which type of tremor?
(a) Essential
(b) Primary orthostatic
(c) Cerebellar
(d) Dystonic

Answer: (d)
Commentary: Suspect a dystonic tremor if it is very asymmetrical and shows postural
dependence. There may be associated dystonia elsewhere in the body. The tremor may have task
specificity, such as writing or postural dependence. A cerebellar tremor will often have other
cerebellar signs present and often improves if the patient performs movement with eyes closed.
Essential tremor is symmetrical and best seen with an outstretch hand. Primary orthostatic tremor
presents as unsteadiness or tremor in the legs that increases with prolonged standing, and the
symptoms are relieved by sitting or walking.



62. A neurologist refers a patient to you with Parkinson disease and poor gait. What treatment
strategy is recommended to prevent frequent falls?
(a) Methylphenidate medication trial to increase attention and concentration
(b) Physical therapy with balance training and cueing strategies
(c) Referral to a neurosurgeon for implantation of a deep brain stimulator
(d) Maximized levodopa medication to improve balance control

Answer: (b)
Commentary: Physical therapy with cueing strategies, such as rhythmic auditory stimulation with
a metronome and balance and strength training are shown to be useful in improving gait and
decreasing falls. Treadmill training is still in its infancy and its role in improving gait is unclear,
although early studies are positive. The use of methylphenidate in initial trials was positive but a
recent randomized, double blinded study using methylphenidate showed no improvement in gait.
The use of deep brain stimulation is very inconsistent in its effect on balance and gait and further
study is needed to optimize type of stimulation and to define new targets for stimulation.
Levodopa can improve gait, but can also cause a worsening of gait and balance, possibly due to
drug-induced dyskinesias.



Personality changes and/or aphasia are typical of which dementia?
(a) Alzheimer
(b) Frontotemporal
(c) Parkinson’s disease with dementia
(d) Vascular

Answer: (b)
Commentary: Frontotemporal dementia is a neurodegenerative disease of unknown etiology with
atrophy and neuronal loss in the frontal and temporal lobes of the brain resulting in a gradual and
progressive decline in behavior and/or language. Overuse of stock phrases, lack of
conversational initiation and echolalia are more common in frontotemporal dementia. Alzheimer
disease is primarily associated with memory and visuospatial loss of function, and speech is more
fluent than in persons with frontotemporal dementia. Parkinson disease with dementia is
associated with symptoms of memory loss, fluctuating cognition, and visual hallucinations with
spontaneous parkinsonism motor features. Persons with vascular dementia usually have a history
of stroke or have focal neurologic deficits, early gait disturbance, changes in personality and
mood and a history of frequent falls or unsteadiness.



A significant improvement in quality of life for advanced ALS patients is attributed to
(a) having advanced directives.
(b) invasive ventilation to prolong life.
(c) earlier hospice transition.
(d) placement in long-term care.

Commentary: Improved quality-of-life for advanced ALS patients is attributed to optimizing inhome
care and early hospice transition. Hospice can ease the burden of care by the family
members. Hospice organizations have guidelines for early entry into hospice during advanced
stages. Most patients who undergo invasive mechanical ventilation do so emergently and often
against their wishes. Invasive ventilation is not preferred in over 92% of ALS patients surveyed.
Often, there is a lack of advanced directives to guide the treating team. Lack of physician
communication with the patient about advanced directives is the major barrier to patients
developing their advanced directives.



You perform an extraocular muscle exam of a patient with multiple sclerosis. You note
that when looking to the right the left eye will not cross midline and she complains of diplopia
when looking to the right but not straight ahead or to the left. You classify her findings as

(a) Parinaud syndrome .
(b) Horner syndrome.
(c) Internuclear ophthalmoplegia.
(d) Millard-Gubler syndrome

Answer: C
Commentary: Internuclear ophthalmoplegia (INO) is caused by a lesion in the medial longitudinal
fasciculus (MLF) in the paramedian brainstem. It is characterized by impaired adduction of the
contralateral eye with gaze toward the side of the lesion. In practice, an isolated case of INO is
rare since the 2 sides of the MLF are very near the midline of the brain stem. The two most
common causes of INO are multiple sclerosis and paramedian brain stem infarct. The Horner
syndrome is due to a superior cervical sympathetic ganglion lesion and causes miosis, ptosis and
anhydrosis. Parinaud syndrome causes impaired upward gaze with dilated and nonreactive pupils
and is the result of a lesion in the midbrain, usually a pineal tumor. Millard-Gubler syndrome is
due to an ipsilateral pons lesion causing ipsilateral palsy of cranial nerve (CN) 6 and CN 7 and a
contralateral hemiparesis



A 45-year-old woman with paraparesis due to relapse-remitting multiple sclerosis diagnosed 5 years ago presents to your outpatient clinic. She started using a cane for ambulation about 3 months ago. She previously did not want to take any medications for her disease, but now asks you whether there are any that can improve her ability to walk. The most appropriate option for her is

A dalfampridine ER (Ampyra).
B Glatiramer Acetate (Copaxone)
C Interferon B1a (Avonex)
D Natalizumab (Tysabri)

Dalfampridine ER (Ampyra), also known as 4-aminopyridine, has been shown to improve walking speed in patients with all forms of multiple sclerosis. This agent is a potassium-channel blocker that increases acetylcholine release at the neuromuscular junction and in the central nervous system. The benefits may be related to improved nerve conduction and the frequency response in demyelinated nerve fibers via prolongation of the repolarization phase of the action potential. The other 3 medications listed are all shown to reduce relapse rate and new lesions on MRI, but not to improve function.



A patient with Parkinson disease is referred to you for rehabilitation recommendations, but you question the diagnosis. Which clinical feature is typical of parkinsonism and therefore increases the likelihood of a correct diagnosis?

a. Poor vertical eye movement
b. asymmetric onset of tremor
c. rapid progression of symptoms
d hyperreflexia

Option b is correct.

The typical individual afflicted with Parkinson disease presents with symptoms on 1 side of the body, a pattern referred to as asymmetric onset. Progressive supranuclear palsy is characterized by the inability to exercise voluntary movement of the eyes. Parkinson-plus syndromes include several diseases, including multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and diffuse Lewy body disease (DLBD). The symptoms of Parkinson-plus syndromes progress within the first 3 to 5 years, while it usually takes 10 to 15 years to see these symptoms in Parkinson disease. People with Parkinson-plus syndromes also have a poor response to dopamine replacement. Rigidity but not hyperreflexia is seen in Parkinson disease.



Which medication used in amyotrophic lateral sclerosis has a neuroprotective effect by inhibiting glutaminergic neurotransmission in the spinal cord?

A. Riluzole (Rilutek)
B Gabapentin (Neurontin)
C Cannabidiol (Cannabis)
D Prednisone (Deltasone

Option a is correct.

Riluzole is a neuroprotective agent that appears to inhibit glutaminergic neurotransmission in the spinal cord. It is modestly effective in improving the life expectancy in the patient with ALS.



A 52-year-old man presents with slowly progressive leg weakness. He also complains of clumsiness with his right hand, which creates difficulties with buttons or turning keys. Examination reveals mild bilateral foot drop and leg weakness. Fasciculations and mild wasting are observed in the calf muscles. There is no spasticity or impaired sensation. His speech is normal, but tongue fasciculations are appreciated. Respiration, pulse and temperature are normal. Electrodiagnostic studies show evidence of denervation with reinnervation. The most likely diagnosis is?

A amyotrophic lateral sclerosis
B Charcot-Marie-Tooth disease
C Guillain-Barré syndrome
D myasthenia gravis

Option a is correct.

Flaccid paresis involving the lower extremities, foot drop, hand clumsiness, muscle wasting, and especially fasciculation in a middle-aged person are highly suggestive of amyotrophic lateral sclerosis (ALS). These symptoms result from degeneration of the motor neurons in the spinal cord (lower motor neuron) and lead to denervation of skeletal muscle. His tongue fasciculations result from degeneration of motor neurons of cranial nerve nuclei. Surviving neurons may reinnervate the denervated myofibers by axon sprouting. The finding of denervation/reinnervation on needle EMG examination is confirmatory of the clinical diagnosis. The patient will later develop evidence of corticospinal and corticobulbar (upper motor neuron) degeneration as his disease progresses.



The best prognostic indicator for non-invasive ventilation in patients with amyotrophic lateral sclerosis (ALS) is

a. functional vital capacity.
b. residual volume.
c.tidal volume.
d total lung capacity.

Option a is correct.

Functional vital capacity is the best prognostic indicator for non invasive ventilation in patients with ALS. Patients may lose vital capacity at a rate of 1,000 mL or more per year. When vital capacity falls to 25 mL/kg of body weight, the ability to cough is impaired, increasing the risk of aspiration pneumonia.



Which clinical factor offers a favorable prognosis in multiple sclerosis?

(a) Male sex
(b) Older age at onset
(c) Normal MRI at presentation
(d) Early development of mild disability

Answer: C
Commentary: Favorable clinical factors with prognostic value in multiple sclerosis include
younger age at onset, female, normal MRI at presentation, complete recovery from first relapse, low relapse rate, long interval to second relapse, and low disability at 2 and 4 years.



Your patient has a tremor of 5-8Hz, which is made worse with activity, and there is little or no
tremor at rest. This finding best describes which type of tremor?

(a) Parkinsonian
(b) Essential
(c) Enhanced physiological
(d) Cerebellar

Answer: B
Commentary: An essential tremor is more prominent with activity (5-8Hz) and is diminished at rest. Stress exacerbates the tremor. The essential tremor can be confused with parkinsonian
tremor. Essential tremor is more prominent with activity, while parkinsonian tremor is more
prominent at rest, with a 4-5Hz frequency. There is also an absence of the other symptoms of parkinsonism such as loss of postural reflexes, rigidity and bradykinesia. Enhanced physiologic tremor is a high-frequency tremor that is most prominent with posture and action. It is
exacerbated by anxiety, fatigue and many drugs. It can be seen with alcohol withdrawal. The
cerebellar tremor has a frequency of about 3Hz and is mainly in a horizontal plane. It is most prominent with fine repetitive action of the extremities and is associated with other signs of
cerebellar ataxia



The most common clinical manifestation of Lyme disease is

(a) monoarticular or oligoarticular arthritis.
(b) facial-nerve palsy.
(c) atrioventricular block.
(d) erythema migrans.

Erythema migrans (EM) is a skin lesion that is erythematous, and may be round or oval, flat or raised, and possibly have central clearing. Of persons with untreated EM, sixty percent will have monoarticular or oligoarticular arthritis. Ten percent will have a neurologic presentation, such as facial-nerve palsy. Approximately 5 percent will have a cardiac manifestation such as atrioventricular block.



A 67-year-old man with Parkinson disease is experiencing more falls. These falls usually occur shortly after getting up in the morning, or after a large meal. You suspect his falls are due to
(a) vestibular dysfunction.
(b) orthostatic hypotension.
(c) increased lower extremity weakness.
(d) increased rigidity.

The majority of patients with Parkinson disease experience orthostatic hypotension (OH) as the disease progresses. The patient’s history suggests falls related to postural changes and situations that lower blood pressure. Educating your patient to avoid or mitigate these situations (slow postural changes, small meals, and avoidance of high heat exposure and alcohol) is the best initial treatment.



In Lyme disease, beyond the initial erythema migrans lesion from infection with the spirochete
Borrelia burgdorfer what other findings may be seen later on?
(a) Facial nerve palsy
(b) Renal insufficiency
(c) Pleural effusion
(d) Cardiomyopathy

Commentary: Lyme disease is the result of a bite from a tick infected with Borrelia burgdorferi. Erythema migrans lesion is typically the initial skin lesion seen. Other findings from systemic infection include mono-/polyarticular arthritis, facial nerve palsy, aseptic meningitis, radiculopathy, or heart block. Renal insufficiency and pleural effusion are not seen.



The risk of an exacerbation of multiple sclerosis is reduced
a) during pregnancy.
b) in the first 3 months after delivery of a baby.
c) in the postoperative period after a surgery.
d) during a respiratory infection.

Answer: (a)
Commentary: During pregnancy multiple sclerosis (MS) exacerbations decrease to about half of
what they would be otherwise. During the first 3 months postpartum, the relapse rate is higher
than normal. The net effect of pregnancy on the course of MS is neutral, and women need not
make decisions about pregnancy based on fear that it will worsen their disease. There is no
documented effect on MS due to surgery or with a respiratory infection.



Which pulmonary parameter is most commonly followed in a patient with amyotrophic lateral sclerosis (ALS)?
(a) Arterial blood gas (ABG)
(b) Oxygen saturation (O2 sat)
(c) Forced expiratory volume in 1 second (FEV1)
(d) Vital capacity (VC)

(d) Vital capacity should be monitored in patients with neuromuscular disease such as ALS. The forced vital capacity is convenient to follow disease progression, and it correlates with disability. FEV1 is normal. Blood gases remain normal until the patient is in near respiratory arrest. Hypercapnia precedes hypoxia, so monitoring oxygen saturation is not helpful.



A negative prognosticator for successful surgical nerve repair after trauma is
(a) partial transection of the nerve.
(b) distal nerve injury.
(c) prior radiation therapy.
(d) nerve repair within 4 months of injury.

(c) Negative prognosticators for successful nerve repair include advanced age, nerve injury resulting from dislocation (stretch), delay of repair beyond 5 months, prior radiation therapy, nerve discontinuity (gap) exceeding 2.5cm, proximal nerve injury and poor condition of nerve endings



Rehabilitation strategies for addressing rigidity associated with Parkinson’s disease include
(a) relaxation techniques with gentle stretching.
(b) a strengthening program.
(c) botulinium toxin injections.
(d) oral baclofen.

(a) Relaxation techniques with slow, rhythmic rotational movements starting with passive range of motion distally and progressing proximally and then adding active range of motion is effective in decreasing rigidity.



Your patient with Parkinson’s disease has sialorrhea. Initial treatment recommendations would
(a) a behavior modification program with speech therapy.
(b) Robinul (glycopyrrolate) with meals.
(c) Botox (botulinum toxin type A) to the salivary glands.
(d) low dose Elavil (amitriptyline) at bedtime

(a) A defective swallowing mechanism rather than excessive saliva production is the primary cause of
drooling and the complaints of excessive drooling in Parkinson’s disease. A behavior modification
program with frequent reinforcement may be effective in reducing drooling in Parkinson’s disease
and should be used prior to medication or botulinum toxin therapy



Falls are common and often disabling in individuals with Parkinson’s disease. The risk of falls is
increased in patients who
(a) are taking nonsteroidal anti-inflammatory medication for pain.
(b) are using a walker for ambulation.
(c) have erratic arm swing with gait.
(d) are taking benzodiazepines

(d) Recurrent falls are more common in individuals with Parkinson’s disease who are taking benzodiazepines. Other risk factors are: history of falls, severe disease, poor balance, depression,
and loss of arm swing with gait.



A 59-year-old man with Parkinson’s disease is evaluated in your clinic. He complains of problems
with rigidity, bradykinesia, tremor, and a functional decline in his activities of daily living. You
(a) Mysoline (primidone) orally to decrease tremor and rigidity.
(b) botulinum toxin injections to decrease rigidity followed by occupational therapy with
stretching program.
(c) occupational therapy with fluidotherapy to improve hand function and a stretching program.
(d) physical therapy with repetitive exercise and a home exercise program.

(d) A systematic program of physical therapy for Parkinson’s disease with repetitive exercise for 1 hour daily, 3 times a week for 4 weeks can significantly improve rigidity, bradykinesia, and activities of daily living, but not tremor. The improvements were not sustained if a regular home program of exercise was not continued



A benefit of marijuana (cannabis) when used in the management of motor neuron disorders such as
amyotrophic lateral sclerosis is
(a) decreased daytime sleepiness.
(b) appetite reduction.
(c) saliva production stimulation.
(d) bronchodilation

(d) The benefits of using cannabinoid medications in patients with motor neuron disorders include analgesia, muscle relaxation, bronchodilation, saliva reduction, appetite stimulation, and sleepinduction.



Which medication has demonstrated positive effects in treating fatigue in patients with amyotrophic
lateral sclerosis?
(a) Modafinil (Provigil)
(b) Baclofen (Lioresal)
(c) Bromocriptine (Parlodel)
(d) Gabapentin (Neurontin)

(a) Modafinil (Provigil) is well tolerated and may reduce the symptoms of fatigue in amyotrophic
lateral sclerosis