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Neurology > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

most likely diagnosis - 65 yo man with 20 yr history of tremor predominantly limited to activities; there is also head tremor. Family history is positive for similar findings and alcohol use decreases its severity

A

essential tremor

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2
Q

next diagnostic step for essential tremor

A

MRI of brain and spine

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3
Q

next step in therapy for essential tremor

A

primidone or propranolol

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4
Q

Froment sign

A

cogwheel effect when testing muscle tone (esp in arms) without increased tone (rigidity)

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5
Q

cogwheel rigidity

A

feeling of periodic resistance to passive movement felt by the examiner in a limb

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6
Q

bradykinesia

A

slowed ability to start and continue movements; impaired ability to adjust the body’s position

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7
Q

lead pipe rigidity

A

hypertonicity felt in parkinsonian limb throughout the range of movements of a joint; indicative of increased tone in all sets of muscles around a joint

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8
Q

physiological tremor

A

very low amplitude, fine tremor (bw 6-12 Hz) that is barely visible to naked eye; present in every normal individual when maintaining a posture or movement

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9
Q

main CF of essential tremor

A
  • bilateral tremor of upper extremities (usually)
  • begins in mid-late life
  • postural and kinetic
  • may have mild impairment of balance
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10
Q

resting tremor

A

occurs when body part is at complete rest against gravity, seen in PD

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11
Q

intention/terminal tremor

A

marked increase in tremor amplitude during a terminal portion of targeted movement i.e. MS, cerebellar disease

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12
Q

40 yo man in seen in ER for inappropriate behavior and confusion; he has appeared fidgety for recent years and recently, has developed jerky movements of entire body. He is alert, easily distracted and tangential. His balance is slightly altered. He has bilaterally increased reflexes and bilateral ankle clonus - most likely dx?

A

Huntington’s disease

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13
Q

next diagnostic step for Huntington’s?

A

genetic counselling and genetic testing for the disease

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14
Q

distal hand movements and long history of fidgeting are typical for…

A

Huntington disease

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15
Q

chorea

A

sudden jerky irregular movements with muscle contractions that are not repetitive or rhythmic but flow from one muscle to the next

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16
Q

athetosis

A

writhing and twisting movements often assoc. with chorea

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17
Q

dystonia

A

sustained muscle contractions causing twisting and repetitive movements or abnormal posture

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18
Q

tardive dyskinesia

A

caused by long term or high dose use of typical antipsychotics; impairment of voluntary movements that is irreversible

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19
Q

young child comes in with symptoms resembling parkinsonism, namely bradykinesia, rigidity, dystonia, myoclonus and seizures - what should you consider?

A

Westphal variant of Huntington’s dz with childhood onset

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20
Q

a young patient comes in with involuntary movements that gradually become more severe, affect all motor activities including gait, arm movements and speech; the patient has symptoms resembling rheumatic fever - dx?

A

syndenham chorea

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21
Q

21 yr old man with history of progressive dystonia beginning in left UE and spreading his back and left LE; his abnormal movements are complex, involving dystonia, myoclonus and tremor that limit his gait, posture and extremity use - dx?

A

primary generalized dystonia - DYT1

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22
Q

next diagnostic step for primary generalized dystonia

A

MRI of brain

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23
Q

next step in therapy for primary generalized dystonia

A

deep brain stimulation of globus pallidus, pars interna

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24
Q

dystonia

A

syndrome characterized by sustained muscle contractions, which provokes twisting and repetitive movements or abnormal postures

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25
Q

myoclonus

A

sudden, involuntary jerking of a muscle or group of muscles

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26
Q

primary dystonia

A

condition with no etiology that can be identified and dystonia is the sole/major symptom

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27
Q

secondary dystonia

A

dystonia in the context of a neurological disease in which dystonia is only one of several symptoms or in which dystonia is the result of an environmental insult

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28
Q

clues that dystonia is secondary

A
  • history of trauma, drugs, infections
  • dystonia at rest
  • atypical site for age of onset
  • early onset speech abnormality
  • hemidystonia
  • abnormal brain imaging or lab tests
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29
Q

which areas of the brain are through to be affected in primary dystonias

A

low or generally abnormal patterns of activity of basal ganglia output structures: internal segment of globus pallidus and substantia nigra pars reticulata

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30
Q

pharmacologic tx. of dystonia

A

levodopa
blockers of central muscarinic cholinergic receptors
benzodiazepines
baclofen

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31
Q

middle aged man presents with asymmetric onset of tremor at rest; he has mild poverty of movement (akinesia of face and body) and increased tone - dx?

A

Parkinson’s

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32
Q

next step in diagnosis of suspected Parkinson’s

A

MRI of brain to evaluate other disorders in ddx

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33
Q

next step in therapy in newly diagnosed Parkinsons

A

either DA agonist or MAO-B inhibitor

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34
Q

substantia nigra

A

consists of pars compacta, pars reticulata and pars lateralis

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35
Q

Lewy body

A

eosinophilic, round inclusion found in cell cytoplasm of substantia nigra, nucleus basalis of Meynert, locus ceruleus, dorsal raphe and dorsal motor nucleus of CN X; made of alpha-synuclein

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36
Q

multiple system atrophy (MSA) - type P

A

resembles parkinson’s except that tremor is less prominent and disorder is symmetric

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37
Q

pt presents with symptoms of parkinsonism, but they also have autonomic insufficiency, notably orthostatic hypotension and impotence – what dx. should you be considering?

A

multiple system atrophy

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38
Q

pt presents with early onset dementia, delusions, hallucinations, fluctuations in consciousness, myoclonus and signs of parkinsonism - dx/

A

Lewy body dementia

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39
Q

unilateral, coarse tremor, rigidity, increased reflexes as well as limb apraxia, limb dystonia and alien limb phenomenon; asymmetric appearance

A

corticobasalganglionic degeneration

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40
Q

progressive supranuclear gaze palsy

A

supranuclear downgaze palsy (inability to voluntarily look down) and square wave jerks on extraocular motion testing; upright rather than flexed posture and frequent falls early on; presence of pseudobulbar emotionality

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41
Q

ataxia

A

unsteady, clumsy motion of limbs or torso caused by a failure of gross coordination of muscle movements

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42
Q

AD cerebellar ataxia - I

A

cerebellar ataxia assoc. with additional features related to optic nerve extrapyramidal system, cerebral cortex and peripheral nerves

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43
Q

AD cerebellar ataxia - II

A

assoc. with pigmentary macular dystrophy

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44
Q

AD cerebellar ataxia - III

A

pure late onset cerebellar syndrome

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45
Q

dx. of spinocerebellar ataxia

A

DNA testing

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46
Q

dz. with ataxia, dysarthria, pyramidal signs, peripheral neuropathy, hyperreflexia, cognitive impairment, nystagmus, hypermetric saccades, opthalmoparesis

A

SCA type 1

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47
Q

dz. with ataxia, dysarthria, peripheral neuropathy, hyporeflexia, dementia and myoclonus, slow saccades and opthalmoplegia

A

SCA type 2

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48
Q

dz with ataxia, dysarthria, spasticity, parkinsonism, amyotrophy, lid retraction, nystagmus, saccade dysmetria, square wave jerks and opthalmoparesis

A

SCA type 3

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49
Q

dz with ataxia, dysathria, nystamus (down beating) and saccadic pursuit with very slow progression and lack of family history

A

SCA type 6

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50
Q

dz with ataxia, dysarthria, retinopathy, peripheral neuropathy, pyramidal signs, infantile phenotypes, saccadic smooth pursuit and slow saccades

A

SCA type 7

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51
Q

dz with ataxia, dysarthria, mild sensory neuropathy, nystagmus and saccidic pursuit

A

SCA type 8

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52
Q

pt presents with pure cerebellar ataxia, familial parkinsonism, hereditary spastic paraplegia, hereditary neuropathy and restless legs sydnrome; impaired temperature distinction in all limbs, even trunk and face - dx?

A

SCA type 3 (i.e. Machado-Joseph syndrome)

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53
Q

radiological feature of SCA

A

high T2 signal in cerebellar cortex

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54
Q

radiological features of multiple system atrophy (MSA)

A

high signal lateral to striatum and high signal hot cross bun sign in brainstem

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55
Q

MCC of tardive dyskinesia

A

chronic exposure to central DA antagonists ex. neuroleptics, metoclopramide

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56
Q

risk factors for developing tardive dyskinesia

A

advanced age
female
co-existent brain damage

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57
Q

tx. of tardive dyskinesia

A

benzodiazepines
baclofen
vit. E
tetrabenazine (not in USA)

58
Q

arching spasms of back and neck are characteristic for…

A

tardive dyskinesia (vs. dystonia which occurs in segmental form involving muscles of face and neck)

59
Q

what type of drugs often make tardive dyskinesia symptoms worse?

A

anticholinergics (which help dystonia)

60
Q

what time period must elapse for it to be considered tardive dyskinesia?

A

3 months (vs. dystonia is usually quicker)

61
Q

what kinds of drugs MC cause tardive dyskinesia?

A

DA- receptor antagonists i.e. neuroleptics (DA depleting agents such as reserpine or tetrabenazine that do not block R. have not been shown to cause TD)

62
Q

what structures make up the basal ganglia

A 
caudate nucleus
putamen
globus pallidus
substantia nigra
subthalamus
63
Q

cardinal features of PD

A

tremor (resting)
rigidity (cogwheel)
akinesia/bradykinesia
postural instability

64
Q

does PD target men or women?

A

Men = Women

65
Q

CF of tremor in PD

A

rhythmical tremor (slow, 4-6 Hz) that is most prominent with limb at rest, affects distal arm more and is worse with anxiety or excitement as well as walking with arm hanging by side

66
Q

rigidity in PD

A

increased resistance to passive movement; first evident in neck and shoulders
- pt perceives it as stiffness and sense of effort to move limbs

67
Q

bradykinesia in PD

A

decreased spontaneous and automatic movements with hypomimia, decreased blinking and impaired fine motor movements

68
Q

postural instability in PD

A

stooped posture, impaired righting reflexes and festinating gait

69
Q

atypical sx of PD that should point you to alternate dx

A
  • early onset or rapidly progressing dementia
  • rapidly progressive course
  • supranuclear gaze palsy
  • UMN or cerebellar signs
  • urinary incontinence
  • postural hypotension
  • early falls
70
Q

what drugs can induce Parkinsonism

A

phenothiazines - metoclopramide
haloperidol & other neuroleptics
reserpine

71
Q

toxins that can induce parkinsonism

A

MPTP
carbon monoxide
manganese

72
Q

when neuronal system degenerative conditions present with Parkinsonism

A 
multiple system atrophy
Shy-Drager syndrome
progressive supranuclear palsy
striato-nigral degeneration
olivopontocerebellar atrophy
corticobasal ganglionic degeneration
73
Q

how can you tell MSA from PD?

A

MSA has less prominent tremor and symmetric, early ANS dysfunction, cerebellar signs, vocal cord paresis and stimulus sensitive myoclonus

74
Q

features of cortical basal ganglionic degeneration

A

unilateral coarse tremor with apraxia, unilateral rigidity, cortical sensory impairment, alien limb, increased reflexes and stimulus sensitive myoclonus

75
Q

progressive supranuclear palsy

A

limited vertical gaze with inability to look down, axial rigidity and neck extension, impaired reflexes and visual changes lead to early falls

76
Q

vascular parkinsonism

A

lower half rigidity with only legs involved and marked gait impairment

77
Q

pathophys of PD

A

loss of DA-containing neurons in substantia nigra as well as gliosis and Lewy bodies on microscopic examination

78
Q

loss of DA-containing neurons in substantia correlates best with what symptom?

A

bradykinesia (not tremor)

79
Q

side effects of Levo-dopa

A
  • dose dependent response
  • wearing-off of effects within hours
  • painful dystonias
  • on-off phenomena and freezing
  • postural hypotension
  • confusions
  • hallucinations
80
Q

how do you eliminate the wearing-off effects of levodopa?

A

combine with DA agonist or add anticholinergic / COMT inhibitors

81
Q

ex. of DA agonists used in PD

A

pramipexole, ropinirole, bromocriptine, pergolide

82
Q

use of DA agonists in PD

A

can be started for mild/early PD to delay onset of dyskinesias with levodopa OR can be used in conjunction with Sinemet to smooth out dose response curve

83
Q

s/e of DA agonists

A

hallucinations
psychosis
NV
hypotension

84
Q

what type of drugs are good for tremor-predominant PD

A

anti-cholinergics

  • benztropine
  • benzhexol
  • diphenhydramine
  • trihexyphenidyl
85
Q

use of anti-cholinergics in PD

A
  • used to decred ease the relative imbalance in Ach-DA activity in striatum; typically used in early stages of dz
86
Q

side effects of anticholinergics

A

urinary retention
constipation
confusion

87
Q

MAO-B inhibitors in PD

A

selegeline, rasagiline, L-deprenyl

  • inhibit enzymes which break down DA
  • reduces symptoms in mild forms of PD or in pts on levodopa
88
Q

what do you do if a patient develops dyskinesias on levodopa?

A
  • decrease dose of levodopa
  • add/increase dose of DA agonist
  • discontinue selegeline
  • add amantadine
  • consider surgery
89
Q

what is amantadine particularly useful for

A

alleviates levodopa induced dyskinesias

- MOA: blocks NMDA-R

90
Q

who is a good candidate for deep brain stimulation for PD?

A

pt with PD without cognitive or psych symptoms who has medically intractable motor fluctuations, intractable tremor and intolerance of medications

91
Q

which areas are stimulated in deep brain stimulation for PD

A

thalamus (VL)
globus paladus (internal segment)
subthalamic nucleus

92
Q

disorder with muscle rigidity, fever, autonomic lability, altered LOC, elevated CK level and leukocytosis

A

neuroleptic malignant syndrome

93
Q

Tx of neuroleptic malignant syndrome

A

d/c drug
antipyretics/rehydration
bromocriptine - bc NMS develops due to chronic DA blockade (tx includes giving DA)

94
Q

akathisia

A

subjective desire to be in constant motion and is assoc. with inability to sit or stand still

95
Q

tx. akathisia

A

anticholinergics or BB

96
Q

tardive dyskinesia

A

MC movement disorder complicating neuroleptic use; includes chewing movements, lip smacking and rolling of tongue inside mouth
- d/c drug immediately

97
Q

stiff-man syndrome

A

fluctuating and progressive muscle rigidity with spasms; usually begins with stiffness of axial and trunk muscles with spread to proximal limb muscles; pts develop lumbar hyperlordosis with restricted movements at hips and spine AND paroxysmal painful muscle spasms often provoked by sudden movement or startle

98
Q

dx of stiff man syndrome

A

demonstration of continuous motor unit activity w/o evidence of neuromyotonia, pyramidal or extrapyramidal dysfunction or structural spinal cord disease

99
Q

what abs may be present in stiff man syndrome

A

anti GAD ab

ampiphysin ab

100
Q

tx of stiff man syndrome

A

baclofen, BDZs - antispastic agents

101
Q

what kind of a tremor is essential tremor

A

bilateral, postural and kinetic tremor involving mainly the upper extremities; responsive to alcohol

102
Q

pathology of essential tremor

A

not known, PET scans show increased activity in thalamus

103
Q

type of inheritance in essential tremor

A

autosomal dominant

- but may occur sporadically

104
Q

DOC for tx of essential tremor

A

propranolol

- if no response to BB can try primidone (limited by sedation), topiramate or gabapentin

105
Q

chorea

A

involuntary, abrupt and irregular movements that flow as if randomly from one body part to another

106
Q

symptoms that usually accompany chorea

A
  • inability to sustain muscle contraction (motor impersistence) ie. cant hold tongue protruded or inability to maintain tight handgrip
107
Q

hereditary causes of chorea

A

Huntingtons
Wilsons dz
Neuroacanthocytosis

108
Q

drugs that cause chorea

A

neuroleptics

antiparkinsonian meds

109
Q

toxins that cause chorea

A

alcohol, anoxia, CO

110
Q

metabolic conditions that cause chorea

A

hyperthyroidism

hyper/hypoglycemia

111
Q

what vascular lesion can cause chorea

A

infarction/ hemorrhage of caudate

112
Q

tx of chorea

A

haloperidol

113
Q

ballism

A

large-amplitude and poorly patterned flinging or flailing movements of limbs (frequently unilateral as in hemiballismus)

114
Q

what lesion causes hemiballismus

A

contralateral lesion in caudate, putamen or subthalamic nucleus

115
Q

Tx of hemiballismus

A

DA-depleting and blocking agents

- if severe, thalamotomy or pallidotomy

116
Q

dystonia

A

sustained muscle contraction leading to repetitive twisting movements or abnormal postures

117
Q

geste antagoniste

A

gently touching the affected body part can diminish dystonia

118
Q

CF of dystonia

A
  • worse with fatigue, stress or emotions, better with relaxation or sleep
  • worsens during voluntary mvmt
  • usually present only during specific movement at time of onset and progresses to rest
119
Q

idiopathic torsion dystonia

A

primary dystonia - broad spectrum disorder including blepharospasm, torticollis, spasmodic dysphonia, writer’s cramp

120
Q

myoclonus

A

sudden-lightning like movement produced by abrupt and brief muscle contraction or inhibition

121
Q

essential myoclonus

A

nonphysiologic variety that occurs in isolation w/o evidence of other neurologic symptoms and signs; may improve with small quantities of alcohol

122
Q

tx of myoclonus

A

clonazepam

valproate

123
Q

tics

A

abrupt, stereotyped, coordinated movements or vocalization

124
Q

PANDAS

A

pediatric autoimmune neurologic disorders assoc with streptococcal infection
- associated with exacerbation of tics, OCD or both; strep infection triggers an autoantibody response that cross-reacts with components of basal ganglia in susceptible individuals

125
Q

tx. of Tics

A

DA antagonists (haloperidol) but due to potent s/e drugs such as clonazepam or clonidine should be tried first

126
Q

Wilsons dz

A

AR disorder with mutation of copper-binding protein (Ceruloplasmin) leading to impaired conjugation and entry of copper to biliary excretory pathway

127
Q

neurologic manifestations of Wilson’s dz

A 
tremor 
ataxia
dysarthria
dyskinesia
parkinsonism
cognitive dysfunction
128
Q

tx of wilsons dz

A

traditionally, penicillamine

now: trientine + zinc (less toxic)

129
Q

dx of wilsons dz

A

increased serum copper, decreased serum ceruloplasmin
increased 24 hr copper excretion test
Kayser Fliescher rings on eye exam

130
Q

paroxysmal dyskinesia

A

rare group of movement disorders characterized by recurrent attacks of hyperkinesias with preserved consciousness

131
Q

paroxysmal kinesigenic choreoathetosis

A

episodes of chorea, athetosis or dystonia are triggered by sudden movements and last for seconds to minutes

132
Q

tx for PKC

A

carbamazepine

133
Q

juvenile onset huntingtons

A

dystonia and parkinsonian features, 90% from affected father

134
Q

four cardinal symptoms of Huntingtons

A
  1. choreoathetosis
  2. intellectual decline
  3. behavioral - personality changes, depression, psychosis
  4. functional decline - dementia
135
Q

movement problems in Huntingtons

A
  • chorea –> dystonia –> choreoathetosis
  • severe dysarthria, recurrent aspirations
  • impaired saccades and loss of smooth pursuit (early sign)
136
Q

what kind of intellectual decline is seen in Huntingtons dz?

A

impaired memory and performance skills; mostly subcortical features i.e. problems with executive function

137
Q

what kind of behavioral changes are seen in Huntingtons?

A

personality changes
depression w/ biologic signs
psychosis with hallucinations, paranoia and thought disorders

138
Q

Westphal variant of Huntingtons

A

develops in childhood – looks more like parkinsonism w/ bradykinesia and rigidity; also have dystonia, myoclonus and seizures
- involvement of neostriatum with atrophy of head of caudate/putamen

139
Q

dx of Huntingtons

A

best = genetic testing
imaging shows caudate atrophy
PET shows decreased metabolism in basal ganglia

140
Q

neurochemistry of Huntingtons

A

decreased GABA transmission
hyperactivity of DA systems
excitotoxic effect of glutamate

141
Q

tx of movement symptoms in Huntingtons

A

anti-DA agents i.e. phenothiazines, haloperidol, reserpine

- only transiently effective before bradykinesia develops with LOTS of side effects

Neurology (15 decks)

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