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194 Cards in this Set
- Front
- Back
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What is the area of the brain predominantly involved in movement disorders?
|
basal ganglia
|
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Is the basal ganglia facilatory or inhibitory?
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inhibitory
|
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What are brief, rapid, forceful and dysrhythmic flinging of the limbs?
|
chorea
|
|
What are slow, writhing, involunatary movements?
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athetosis
|
|
What two areas of the body are usually involved in athetosis?
|
face, and distal upper extremities
|
|
What is the side of hemiballismus in relation to the lesion?
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unilateral and contralateral to the lesion
|
|
Where is the lesion in hemiballismus?
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contralateral subthalamic nuclei
|
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What structure is controlled by the subthalamic nuclei?
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globus pallidus
|
|
What is akathisia?
|
reversible motor restlessness accompanied by a sensation of inner tension or anxiety
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What is the typical cause of akathisia?
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antipsychotic medications
|
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What is the most important sign of cerebellar disease?
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ataxia
|
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What are the three types of ataxia?
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sensory
vestibular cerebellar |
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Sensory ataxia is due to a loss of what function?
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propioception
|
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What are three symptoms of vestibular ataxia?
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vertigo, nausea, and vomiting
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What are three neurologic deficits associated with cerebellar ataxia?
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dysmetria
asynergia dysdiadochokinesis |
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What is dysmetria?
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disturbance in the trajectory or placement of a body part during active movement resulting in hypometria (undershooting) or hypermetria (overshooting) of the target
|
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What is a disturbance in the trajectory or placement of a body part during active movement resulting in hypometria (undershooting) or hypermetria (overshooting) of the target
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dysmetria
|
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Where is the lesion most commonly in dysmetria?
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cerebellum
|
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What refers to the difficulties within the sequence and speed of components of movement?
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asynergia
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What is asynergia?
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The difficulties within the sequence and speed of components of movement
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What is dysdiadochokinesis?
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difficulty with repetitive or fine movement
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What is difficult with repetitive or fine movement?
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dysdiadochokinesis
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What type of tremor is seen with Parkinson's disease?
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resting tremor
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What is the tremor that occurs during sustained contraction of muscles to maintain the position of a body part against gravity?
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essential tremor
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What is an essential tremor?
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a tremor that occurs during sustained contraction of muscles to maintain the position of a body part against gravity
|
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What is an intention tremor?
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a tremor that occurs during voluntary movement from one point to another
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What is a tremor that occurs during voluntary movement from one point to another?
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intention tremor
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Which tremor is often a sign of cerebellar dysfunction?
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intention tremor
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What is the most common type of tremor?
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essential tremor
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Late onsent of an essential tremor is called what?
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senile tremor
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Is an essential tremor benign or serious?
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benign
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What is the inheritence pattern of an essential tremor?
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autosomal dominant
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What substance intake attenuates or resolves an essential tremor?
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alcohol
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What is a pharmacological agent used to treat essential tremor?
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propanalol
|
|
What class of medication is propanolol?
|
beta-blocker
|
|
What class other than beta blockers is used to treat essential tremor?
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anticholinergics
|
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What does an EMG show in essential tremor?
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simultaneous bursts produced from simultaneous contractures of agonist and antagonist muscles
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Simultaneous bursts produced from simultaneous contractures of agonist and antagonist muscles on EMG is characterstic of what type of tremor?
|
essential tremor
|
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What movement disorder is characterized by sudden, jerky, or irregular contractions of a muscle or groups of muscles?
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myoclonus
|
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What are three treatments for myoclonus?
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valproic acid
botulinum toxin clonazepam |
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What is sustained muscle contraction that causes repetitive, twisting movements of variable speed and leads to abnormal posture?
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dystonia
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What is the most common focal dystonia?
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cervial dystonia - spasmodic torticollis
|
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What muscle is involved in spasmodic torticollis?
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sternocleidomastoid
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What is the most common form of generalized dystonia?
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primary dystonia
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What is the inheritence pattern of primary dystonia?
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autosomal dominant
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What medicine may helpful in a small subset of the population with dystonia?
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levodopa
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What age group is most likely to progress to a generalized disabiling dystonia?
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younger
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What is the most effective treatment of focal dystonias?
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intra-muscular injection of botulinum toxin
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What is the second most common focal dystonia?
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blepharospasm
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What are two primary muscles involved in spasmodic torticollis?
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ipsilateral splenius capitus
contralateral sternocleidomastoid |
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What is dystonia of a head tilt called
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laterocollis
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What is the dystonia of neck extension called?
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retrocollis
|
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What is the dystonia of neck flexion called?
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anterocollis
|
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What bilateral muscles are involved in antercollis?
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bilateral sternocleidomastoid
|
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What bilateral muscle is involved in retrocollis?
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bilateral splenius capitus
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What is the sensation of restless leg syndrome?
|
dysethestic
|
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What percentage of the population has restless leg syndrome?
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15%
|
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What are two causes of secondary restless leg syndrome?
|
iron deficiency
peripheral neuropathy |
|
What are 4 dopaminergic agents used to treat restless leg syndrome?
|
Sinemet
Requip Parlodel Mirapex |
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What benzodiazepine is used to treat restless leg syndrome?
|
klonipin
|
|
What is the most common movement disorder?
|
Parkinson's
|
|
What percentage over 50 years old is affected by Parkinson's?
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1%
|
|
What 5 agents can cause drug induced parkinsonism?
|
haloperidol (Haldol)
metoclopramide (Reglan) reserpine amiodarone lithium |
|
What 2 toxins can cause toxin-induced parkinsonism?
|
manganse
carbon monoxide |
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What is the parkinsonism associated with repeated trauma to the head?
|
dementia pugilistica
|
|
What type of stroke can cause parkinsonism?
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lacunar strokes
|
|
Parkinson's is a disease of what structure in the brain?
|
basal ganglia
|
|
What two areas of the basal ganglia suffer cell loss in Parksinson's?
|
substantia nigra
locus ceruleus |
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What pathway in the brain degenerates in Parkinson's?
|
nigrostriatal pathway
|
|
The nigrostriatal pathway connects what two areas?
|
substantia nigra to the corpus striatum
|
|
What two structures in the brain produce dopamine?
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substantia niagra
locus ceruleus |
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In what structure is there a decrease in dopamine content?
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corpus striatum
|
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What pathological structures are found in damaged basal ganglia cells?
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Lewy bodies
|
|
What is the stain affinity of Lewy bodies?
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eosinophilic
|
|
In what part of the cell are Lewy bodies found?
|
intracytoplasmic
|
|
What is dopamine's effect on the cholinergic system?
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inhibitory
|
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What is the annual incidence of Parkinson's per 100,000?
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20/100,000
|
|
What is the male:female ratio of Parkinson's?
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3:2
|
|
What is the prevelence of Parkinson's?
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160/100,000
|
|
What the most common Parkinson's symptom?
|
Pin-rolling resting tremor
|
|
What is the frequency range (Hz) of a Parkinson's resting tremor?
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3-5 Hz
|
|
What is the EMG pattern of a Parkinson's resting tremor?
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rhythmic alternating bursts in agonist and antagonist muscles
|
|
What are the 6 most prominent signs/symptoms of Parkinson's?
|
resting tremor
bradykinesia cogwheel rigidity masked facies fenestrating gait postural instability |
|
What is masked facies called?
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hypomimia
|
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What is tremor superimposed on muscular rigidity?
|
cogwheel rigidity
|
|
What is a circulatory manifestation of Parkinson's?
|
orthostatic hypotension
|
|
How common are depression and dementia in Parkinson's? (1/x)
|
1/3 of patients
|
|
How common is resting tremor in Parkinson's (1/x)?
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2/3
|
|
What are the two goals of Parkinson's treatment?
|
reduce cholinergic activity
increase dopaminergic activity |
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What are the two component agent of Sinemet?
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L-Dopa and Carbidopa
|
|
What is Carbidopa?
|
a dopa-decarboxylase inhibitor
|
|
What is the function of Carbidopa?
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prevents systemic metabolism of L-Dopa
|
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What is L-dopa?
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a metabolic precursor of dopamine
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What agent stimulates dopamine D2 receptors?
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bromocriptine
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Why was pergolide removed from the market in 2007?
|
heart valve damage
|
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What class of medication is bromocriptine?
|
ergot derivatives
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What specific dopamine receptor does bromocriptine stimulate?
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D2
|
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Name 2 nonergot derivatives used to stimulate dopamine receptors?
|
ropinirole - Requip
pramipexole - Mirapex |
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What anti-viral agent acts by potentiating the release of endogenous dopamine?
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amantadine
|
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How does amantadine work?
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It potentiates the release of endogenous dopamine
|
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What are 3 other actions of amantadine?
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mild anticholinergic
inhibits dopamine reuptake blocks glutamate receptors |
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What is the brand name of amantadine?
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Symmetrel
|
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What class of medications used in Parkinson's is useful to treat tremors?
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anticholinergic
|
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Name 4 anticholinergics used in Parkinson's?
|
trihexphenidyl - Artane
benzotropine - Cogenitin procyclidine - Kemadrin Orphanedrine - Disipal |
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What class of medications used in Parkinson's reduce oxidative damage in the substantia niagra?
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selective monoamine oxidase B inhibitors
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How do selective monoamine oxidase B inhibitors work in Parkinson's?
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reduce oxidative damage in the substantia niagra
|
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Name 2 MAO-B inhibitors used in Parkinson's?
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Selegiline - Eldepryl
Rasagiline - Azilect |
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What is the predominant isoenzyme in the corpus striatum responsible for the majority of the oxidative metabolism of dopmaine?
|
MAO-B
|
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Name 2 catechol-o-methyltransferase inhibitors used in Parkinson's?
|
Tolcapone - Tasmar
Enacapone - Comtan |
|
What is the mechanism of action of catechol-o-methyltransferase?
|
inhibits the metabolism of dopamine and its precursor in the liver, and GI tract
|
|
A reduction of tremors is experience on the ipsi or contra-lateral side of a thalamotomy?
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contralateral
|
|
What structure is partially destroyed in a pallidotomy?
|
globus pallidus
|
|
What 3 symptoms does a pallidotomy treat best?
|
dyskinesias
stiffness freezing |
|
What symtoms is treated with a thalmotomy?
|
tremor
|
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What 3 structures can be treated in deep brain stimulation?
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globus pallidus internus
thalamus subthalamic nucleus |
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What 3 Parkinson's symptoms are treated effectively with deep brain stimulation of the subthalamic nucleus?
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tremor
rigidity bradykinesia |
|
What is the most common surgical for Parkinson's disease?
|
subthalamic nucleus deep brain stimulation
|
|
Thalamic deep brain stimulation worsens what 3 Parkinson's symptoms?
|
bradykinesia
gait rigidity |
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What Parkinson's surgical procedure allows for a reduction of medication by half?
|
subthalamic nucleus deep brain stimulation
|
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What Parkinson's procedure controls all the cardinal signs/symptoms of Parkinson's?
|
palladial deep brain stimulation
|
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Into what structure are the leads placed in a palladial deep brain stimulation?
|
globus pallidus interna
|
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What study is done to evaluate oropharyngeal dysphagia in Parkinson's?
|
swallowing evaluation
|
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What has a worse prognosis - Parkinson's or Parkinson-plus syndrome?
|
Parkinson-plus
|
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What is most common of the parkinson-plus syndromes?
|
progressive supranuclear palsy
|
|
What is the ocular symptom in progressive supranuclear palsy?
|
vertical gaze palsy
|
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In what direction is a patient unable to look in progressive supranuclear palsy?
|
down
|
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What are the two types of multisystem atrophy?
|
parkinsonian type
cerebellar type |
|
What are the 3 parkinsonian types of multisystem atrophy?
|
Shy-Drager
Olivopontocerebellar atrophy Striatonigral degeneration |
|
In additon to parkinson's symptoms, what is the main autonomic dysfunction of Shy-Drager?
|
progressive dysautonomia
|
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In addition to parkinsonian features what are the two main autonomic dysfunctions seen in Olivopontocerebellar atrophy?
|
ataxia
dysarthria |
|
What Parkinsonian symptom is uncommon in Striatonigral degeneration?
|
tremors
|
|
What autonomic dysfunction is seen in stritonigral degeneration, in addition to Parkinson features?
|
cervical dystonia - anterocollis
|
|
What is the inheritence pattern of Huntington's?
|
autosomal dominant
|
|
What receptors have heightened sensitivity in Huntington's?
|
striatal dopamine receptors
|
|
What area of the brain is affected in Huntington's?
|
corpus striatum
|
|
Atrophy of what brain structure can be seen on MRI or CT in Huntington's?
|
caudate nucleus
|
|
What neurotransmitter is deficient, and in what brain structure in Huntington's?
|
deficiency of GABA in basal ganglia
|
|
In Huntington's in addition to deficiency of GABA there is a deficiency of what 2 other neurotransmitters?
|
substance P
enkephalins |
|
What is the chromosome involved in Huntington's?
|
4p16.3
|
|
What is the DNA nucleotide pattern seen in Huntington's?
|
trinucleotide CAG repeat
|
|
What is the prevalence range per 100,000 of Huntington's?
|
4-8/100,000
|
|
What is the cause of death in Huntington's?
|
aspiration pneumonia
|
|
What is the classic triad in Huntington's?
|
chorea/choreoathetosis
dementia family history |
|
What are 5 signs/symptoms of a family history in Huntington's?
|
dysarthria
dysphagia depression teeth grinding facial grimacing |
|
What is the survival range (in years) after diagnosis in Huntington's?
|
15-20 years
|
|
What are the hyperkinetic, involuntary, jerky movements seen in Huntington's?
|
choreoathetosis
|
|
What is the most effective agent used to supress the movement disorder in Huntington's?
|
haloperidol
|
|
What two phenothiazines are used to supress the movement disorder in Huntington's?
|
fluphenazine
perphenazine |
|
What is primary side effect risk of haloperidol or the phenothiazine medications?
|
tardive dyskinesia
|
|
What is the goal of Huntington's pharma treatment?
|
reduce dopamine action
|
|
Name two presynaptic dopamine depleters used in Huntington's?
|
reserpine
tetrabenazine |
|
Reserpine and tetrabenzaine belong to what class of meds used in the treatment of chorea in Huntington's?
|
presynaptic dopamine depleters
|
|
What are two side effects of haloperidol and the phenothiaizines?
|
extrapyramidal and anticholinergic side effects
|
|
What is the mechanism of action of fluphenazine and perphenazine?
|
dopamine receptor blockers
|
|
What is the Huntington's variant featuring the Parkinson's-like symtoms of hypokinesia and rigidity?
|
Westphal variant
|
|
Who introduced the classification system for ataxias?
|
Harding
|
|
What is the inheritance pattern of Friedrich ataxia?
|
autosomal recessive
|
|
What is the inheritance pattern of early onset cerebellar ataxia?
|
autosomal recessive
|
|
What is the inheritance pattern of ataxia-telangectasia?
|
autosomal recessive
|
|
What is the inheritance pattern of ataxia with isolated vitamin E deficiency?
|
autosomal recessive
|
|
What is the inheritance pattern of spinocerebellar ataxia?
|
autosomal dominant
|
|
What is the inheritance pattern of dentatorubral-pallidoluysian atrophy?
|
autosomal dominant
|
|
What is the inheritance pattern of episodic ataxias?
|
autosomal dominant
|
|
What are two physical causes of ataxia?
|
heat stroke
hyperthermia |
|
What is an endocrine cause of ataxia?
|
hypothyroidism
|
|
What is a GI cause of ataxia?
|
malabsorption of vitamin E
|
|
What are 3 drug/toxin associated ataxias?
|
antiepileptics
lithium solvents |
|
What malignancy is associated with ataxia?
|
paraneoplastic cerebellar degeneration
|
|
What alcohol induced condtion is a cause of ataxia?
|
alcoholic cerebellar degeneration
|
|
What is the etiology of Friedrich's ataxia?
|
low production of messenger RNA from gene X25
|
|
What is the most common age range presentation of Friedrich's?
|
10-15 years old
|
|
What are the 3 presenting signs/symptoms of Friedrich's?
|
ataxia
areflexia of the lower limbs impaired vibration and proprioception |
|
What primitive reflex is seen in most patients with Friedrich's?
|
extensor plantar reflex (Babinski)
|
|
Name 3 non-neurologic signs seen in Friedrich's?
|
scoliosis
pes cavus hypertrophic obstructive cardiomyopathy |
|
When does ataxia-telangectasia begin?
|
childhood
|
|
What is the difference between early onset cerebellar ataxia and Friedrich's?
|
retained deep tendon reflexes in early onset cerebellar ataxia
|
|
What hereditary ataxia has a high incidence of neoplasia?
|
ataxia-telangectasia
|
|
What is the nucleotide repeat pattern seen in spinocerebellar ataxia?
|
CAG
|
|
In what country is there a high incidence of dentatorubral-pallidoluysian atrophy?
|
Japan
|
|
What are the 3 signs/symptoms of dentatorubral-pallidoluysian atrophy?
|
ataxia
dysarthria dementia |
|
What is the etiology of episodic ataxia type I?
|
potassium channel gene
|
|
What are three signs/symptoms of episodic ataxia type I?
|
brief attacks of ataxia and dysarthria associated with interictal myokymia
|
|
What is the chromosome for episodic ataxia type I?
|
12p
|
|
In what country is there a high incidence of dentatorubral-pallidoluysian atrophy?
|
Japan
|
|
What are the 3 signs/symptoms of dentatorubral-pallidoluysian atrophy?
|
ataxia
dysarthria dementia |
|
What is the etiology of episodic ataxia type I?
|
potassium channel gene
|
|
What are three signs/symptoms of episodic ataxia type I?
|
brief attacks of ataxia and dysarthria associated with interictal myokymia
|
|
What is the chromosome for episodic ataxia type I?
|
12p
|
|
What is the chromosome for epidodic ataxia type II?
|
19p
|
|
What is the difference between episodic ataxia type I and type II?
|
type II attacks are of longer duration
|
|
What is the most common inherited form of mental retardation?
|
fragile x syndrome
|
|
What are the two signs/symptoms of fragile x ataxia syndrome?
|
progressive action tremor
cerebellar ataxia |
|
What is average age of onset of multiple system atrophy-cerebellar?
|
55 year old
|
|
What are 3 signs/symptoms of multiple system atrophy-cerebellar?
|
ataxia
dysarthria cerebellar oculomotor abnormalities |
