Remaining Movement Disorders Flashcards

1
Q

What are some movement disorders?

A

essential tremors, huntington disease, tourette syndrome, RLS, wilson disease, amyotrophic lateral sclerosis (ALS), myasthenia gravis

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2
Q

What are the first line agents for essential tremor with persistent disability due to tremor?

A

propranolol

primidone

propranolol + primidone

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3
Q

What can you add to first line agents (Propranolol or Primidone) in essential tremor pts with persistent disability due to tremor?

A

Gabapentin

Topiramate

Nimodipine

if that still doesn’t work:

limb tremor- DBS

head/voice tremor- botox injections

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4
Q

What is an essential tremor?

A

A rhythmic and oscillatory movement of a body part

relatively constant freq., varying amp.

Caused by either alternating or synchronous contractions of antagonistic muscles

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5
Q

What is the most common movement disorder?

A

essential tremor

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6
Q

Propranolol MOA?

A

non selective BB

-depression of CNS

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7
Q

Dosage of propranolol?

A

Long acting form preferred

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8
Q

ADEs of propranolol?

A

lightheadedness, fatigue, impotence, and bradycardia

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9
Q

Contraindication for propranolol?

A

heart block, asthma, or type 1 diabetes mellitus

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10
Q

What BBs can you give to asthmatics with essential tremor?

A

Atenolol or Metoprolol

non-selective

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11
Q

Which BBs causes less depression?

A

Sotalol, Nadolol

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12
Q

What does off label drug use mean?

A

not approved by food and drug administration but has been showed to be effective and have become typical standard of care

-be careful with this

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13
Q

off label use for Primidone?

A

anticonvulsant

also used for anesthesia

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14
Q

Dosage of primidone?

A

start low, gradually increase

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15
Q

ADEs of Primidone?

A

Sedation!

drowsiness, confusion, fatigue, depression, N/V, ataxia, dizziness, unsteadiness

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16
Q

What is another second line option for tremor with inadequate response to propranolol?

A

alcohol

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17
Q

What can you use for tremor if Primidone isn;t effective? assumed MOA?

A

Topiramate

enhances GABA activity

Blocks voltage gated Na channel

Antagonizes AMPA/kainite glutamate receptors (excitatory)

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18
Q

ADEs of topiramate?

A

nausea, paresthesia, concentration difficulty*

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19
Q

Gabapentin MOA?

A

NOT GABA agonist

possibly: interacts w/ an auxiliary subunit of voltage sensitive ca2 channels

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20
Q

ADEs of gabapentin

A

sleepiness, dizziness, gait unsteadiness*, brain fog

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21
Q

How are botox injections used for tremor?

A

Modest benefit for limb tremor associated with ET

may also help head tremor/voice tremor

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22
Q

MOA of botox injections?

A

prevents Ca dependent release of acetylcholine and produces a state of denervation

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23
Q

ADEs of botox injection to tx voice tremor?

A

breathliness, hoarseness, swallowing difficulty

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24
Q

MOA of Alprazolam* or Clonazepam? (Benzodiazepines)

A

enhance GABA activity

limb tremor if it is aggravated by anxiety

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25
Q

ADEs of Alprazolam?

A

mild sedation, fatigue

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26
Q

What are some options if you are concerned for Benzo dependance?

A

Alcohol

Nimodipine (CCB)

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27
Q

What is huntington disease?

A

Inherited progressive neurodegenerative disorder involving the basal ganglia

  • movement abn. +/- cognitive and psychiatric func. abn.
  • slow course of progression
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28
Q

How is huntington’s inherited?

A

autosomal dominant- chromosome 4

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29
Q

What is chorea?

A

Assoc. with huntington’s

rapid, involuntary, non-repeative movement involving the face, trunk and limbs

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30
Q

Pathophys in Huntington’s ?

A

GABAnergic neuron is lost; acetylcholine or overactivity of dopaminergic neurons

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31
Q

Tx for huntington’s?

A

Supportive care!

Chorea- Tetrabenzaine and Deubetrabenazine

Suppressing movement- antipsychotics (dopamine antagonists)

+/- antidepressants, anticonvulsants, Amatadine

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32
Q

Why do we need to be careful with use of antipsychotics in huntington’s pts?

A

Dopamine antagonist, so need to make sure we’re not causing parkinson’s

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33
Q

Tetrabenazine MOA?

A

Tetrabenazine: deplete amines, especially dopamine, from nerve endings; acts by inhibiting presynaptic vesicular monoamine transporter type 2

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34
Q

Clinical application of Tetrabenazeine?

A

reduce sxs severity i.e. chorea

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35
Q

ADEs of Tetrabenazine?

A

Depression*, hypotension, sedation

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36
Q

Haloperidol MOA?

A

Blockade of D2 receptors&raquo_space; 5-HT2A receptors

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37
Q

Haloperidol effects?

A

Some α blockade, but minimal M-receptor blockade, less sedation than phenothiazines

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38
Q

Haloperidol is a…

A

Butyrophenone

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39
Q

Haloperidol clinical application?

A

Huntington’s chorea*

Tourette’s syndrome*

Schizophrenia

Bipolar diseoder

Behavioral disturbances

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40
Q

ADEs of Haloperidol?

A

extrapyramidal dysfunc.

  • big movements and posture

(can resemble parkinson’s)

41
Q

What are some atypical antipsychotics?

A
Aripiprazole
 Clozapine
    Olanzapine
 Quetiapine
 Risperidone
 Ziprasidone
42
Q

Atypical antipsychotics MOA?

A

Blockade of 5-HT2A receptors

>

blockade of D2 receptors

43
Q

What are Atypical antipsychotics used for?

A

Huntington’s

schizophrenia

bipolar disorder

Tourette’s syndrome *

Agitation in Alzheimer’s, Parkinson’s*

44
Q

ADEs of aytpical antipsychotics?

A

Agranulocytosis, DM, hypercholesterolemia, hyperprolactinemia, QT prolongation, weight gain

45
Q

What is tourette syndrome?

A

Neurological disorder manifested by motor and phonic tics with onset during childhood

Tics are clinical hallmark

46
Q

What are tics?

A

sudden, brief, intermittent movements (motor tics) or utterances (vocal or phonic tics)

47
Q

Most pts with tourette syndrome also have..

A

ADHD or OCD

48
Q

Tx of tourette’s syndrome?

A

Education and counseling for mild sxs

For more bothersome:
-Neuroleptics pimozide (Orap) or haloperidol

off label:

  • Fluphenazine (dopamine antagonist)
  • Risperidone
  • Tetrabenazine
49
Q

when can you use botox for TS?

A

if focal or phonic tics

50
Q

Clinical application of Haloperidol in TS?

A

reduce vocal and motor tic freq. and severity

51
Q

Pimozide MOA?

A

Dopamine receptor antagonist

52
Q

Pimozoide clinical application?

A

Severe motor and phonic tics who failed standard treatment

53
Q

Pimozoide ADEs?

A

Sedation, akathisia, akinesia

Ocular-accommodation decreased

54
Q

What is restless leg syndrome?

A

overwhelming urge to move the legs, assoc. with unpleasant paresthesias

worse at night and at night and relieved by movement

55
Q

RLS is commonly assoc. with?

A

sleep disturbances, caffeine, stress, alcohol, and fatigue

56
Q

What drugs are assoc. with RLS?

A

Antidepressants (except bupropion)

Antipsychotics

Dopamine-blocking antiemetics (eg, metoclopramide)

Centrally-acting antihistamines

57
Q

What is Periodic leg movements of sleep (PLMS)?

A

jerking movements of the leg during sleep

58
Q

What is Periodic limb movement disorder (PLMD)

A

when sleep fragmentation and daytime fatigue coexist with PLMS

59
Q

Tx for RLS?

A

iron replacement
-if serum ferritin lower than 75

Ferrous sulfate

60
Q

What can you give for RLS if iron replacement doesn’t work?

A

alpha 2 delta ligand (gabapentin, pregablin)

dopamine agonist (pramipexole)

61
Q

When should you give alpha 2 delta ligand for RLS?

A

sleep disturbance, insomnia, painful RLS, comorbid pain syndrome, hx of current impulses control disorder, anxiety

62
Q

When should you give dopamine agonist for RLS?

A

increased risk for falls, severe sxs of RLS, excess weight, metabolic syndrome, OSA, depression

63
Q

What can be used for the tx of intermittent RLS?

A

Dopaminergic agents: Levodopa

-Carbidopa-levodopa

Benzodiazepines

  • mild cases of RLS, particularly in younger pts
  • Clonazepam, Diazepam
64
Q

ADEs of benzodiazepines?

A

nocturnal unsteadiness and drowsiness or cognitive impairment in the morning*

65
Q

Name 2 dopamine agonists. What are they used for?

A

Pramipexole, Ropinirole

persistent RLS

66
Q

ADEs of Dopamine agonists

A

Common–nausea, lightheadedness, and fatigue*

Less common–nasal stuffiness, constipation, insomnia, and leg edema

Concerning-increased risk of impulse control disorders*

67
Q

Name 2 Alpha 2 delta calcium channel ligands. What are they used for

A

gabapentin, Pregabalin

persistent RLS
-also: peripheral neuropathy, chronic pain syndrome, insomnia/sleep disturbances*

68
Q

When are Alpha 2 delta calcium channel ligands preferred for RLS?

A

in pts with impulse control disorder

BUT increases risk of suicidal thoughts and behavior

69
Q

When are opioids use for RLS?

A

for refractory RLS

codeine, tramadol, methadone, oxycodone

70
Q

Opioid MOA

A

interaction btwn. spinal opioid and dopamine receptors

71
Q

What is augmentation?

A

increased sxs with increased doses of meds

-main comp of dopaminergic therapy in RLS

72
Q

What is Wilson’s disease?

A

Recessively inherited disorder of copper metabolism

impaired biliary copper excretion leads to accumulation of copper in several organs

73
Q

Sxs of wilson’s disease?

A

Liver: N/V, fatigue, jaundice

CNS: tremors or uncontrolled movements, muscle stiffness, probs with speech, swallowing or coordination

74
Q

Tx for Wilson’s disease?

A

Chelating agents: D- Penicillamine, Trientine

oral zinc

75
Q

What are chelating agents? MOA?

A

remove extra copper from the body by releasing it from organs into the bloodstream

76
Q

D penicillamine MOA?

A

Contains a free sulfhydryl group that functions as a copper chelating moiety

77
Q

D penicillamine ADEs?

A

fever, cutaneous eruptions, lymphadenopathy, neutropenia, thrombocytopenia, proteinuria

Give with Pyridoxine 25mg/day to prevent pyridoxal phosphate deficiency

78
Q

Trientine MOA ?

A

Functions principally by removing copper from less strongly bound sites on proteins and membranes, and it increases renal copper excretion

79
Q

Can chelating agents be used in conjunction with iron replacement?

A

NO

80
Q

Trientine ADEs?

A

hypersensitivity rxn and pancytopenia-rare

neuro sxs

81
Q

What should you monitor in a pt taking Trientine?

A

24 hours copper excretion- to check for effectiveness

82
Q

MOA of oral zinc?

A

interferes with the absorption of copper

83
Q

Oral zinc ADEs?

A

GI upset

elevation in serum amylase and lipase without evidence of pancreatitis

84
Q

What is amyotrophic lateral sclerosis (ALS)

A

aka Lou Gehrig’s disease

disorder of the motor neurons of the ventral horn of the spinal cord (lower motor neurons) and the cortical neurons that provide their afferent input (upper motor neurons)

gradual deterioration

85
Q

Sxs of ALS?

A

weakness, muscle atrophy, fasciculation, spasicity, dysarthria, dysphagia, res. compromise

SPARES sensory, autonomic and oculomotor func.

86
Q

Tx of ALS?

A

Riluzole

Free radial scavanger: Edaravone

87
Q

Riluzole PK?

A

absorbed orally, high protein bound

high fat meals decrease absorption

88
Q

Riluzole ADEs?

A

HTN, abn pain, neuromuscular and arthralgia, tremor, decrease lung func., elevates LFTs

89
Q

Endaravone ADEs?

A

injection site contusion, gait disturbance, HA

sodium bisulfite- pos. allergic rxn

90
Q

What can you use for sxs relief of ALS: spasticity?

A

Baclofen

Tizanidine

Clonazepam

91
Q

Baclofen MOA?

A

GABA B receptor agonist

92
Q

Baclofen ADEs?

A

sedation

93
Q

Tizanidine MOA?

A

agonists of alpha adrenergic receptors in the CNS

94
Q

Tizanidine ADEs?

A

Drowsiness, asthenia, dizziness

95
Q

Benzodiazepines MOA?

A

enhances the inhibitor effect of GABA A receptors

96
Q

ADEs of benzodiazepines?

A

amnesia, confusion, drowsiness, slurred speech

97
Q

What is myasthenia gravis?

A

Neuromuscular disease characterized by weakness and marked fatiguability of skeletal muscle

defect in the synaptic transmission at the neuromuscular junction

98
Q

Tx of MG?

A

standard anticholinesterase drugs:

  • Pyridostigmine
  • Neostigmine
99
Q

MOA of standard anticholinesterase drugs?

A

inhibit the action of the metabolizing enzyme acetylcholinesterase