Remaining Movement Disorders Flashcards

(99 cards)

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
ADEs of Alprazolam?
mild sedation, fatigue
26
What are some options if you are concerned for Benzo dependance?
Alcohol Nimodipine (CCB)
27
What is huntington disease?
Inherited progressive neurodegenerative disorder involving the basal ganglia - movement abn. +/- cognitive and psychiatric func. abn. - slow course of progression
28
How is huntington's inherited?
autosomal dominant- chromosome 4
29
What is chorea?
Assoc. with huntington's rapid, involuntary, non-repeative movement involving the face, trunk and limbs
30
Pathophys in Huntington's ?
GABAnergic neuron is lost; acetylcholine or overactivity of dopaminergic neurons
31
Tx for huntington's?
Supportive care! Chorea- Tetrabenzaine and Deubetrabenazine Suppressing movement- antipsychotics (dopamine antagonists) +/- antidepressants, anticonvulsants, Amatadine
32
Why do we need to be careful with use of antipsychotics in huntington's pts?
Dopamine antagonist, so need to make sure we're not causing parkinson's
33
Tetrabenazine MOA?
Tetrabenazine: deplete amines, especially dopamine, from nerve endings; acts by inhibiting presynaptic vesicular monoamine transporter type 2
34
Clinical application of Tetrabenazeine?
reduce sxs severity i.e. chorea
35
ADEs of Tetrabenazine?
Depression*, hypotension, sedation
36
Haloperidol MOA?
Blockade of D2 receptors >> 5-HT2A receptors
37
Haloperidol effects?
Some α blockade, but minimal M-receptor blockade, less sedation than phenothiazines
38
Haloperidol is a...
Butyrophenone
39
Haloperidol clinical application?
Huntington's chorea* Tourette's syndrome* Schizophrenia Bipolar diseoder Behavioral disturbances
40
ADEs of Haloperidol?
extrapyramidal dysfunc. - big movements and posture (can resemble parkinson's)
41
What are some atypical antipsychotics?
``` Aripiprazole  Clozapine Olanzapine  Quetiapine  Risperidone  Ziprasidone ```
42
Atypical antipsychotics MOA?
Blockade of 5-HT2A receptors > blockade of D2 receptors
43
What are Atypical antipsychotics used for?
Huntington's schizophrenia bipolar disorder Tourette's syndrome * Agitation in Alzheimer's, Parkinson's*
44
ADEs of aytpical antipsychotics?
Agranulocytosis, DM, hypercholesterolemia, hyperprolactinemia, QT prolongation, weight gain
45
What is tourette syndrome?
Neurological disorder manifested by motor and phonic tics with onset during childhood Tics are clinical hallmark
46
What are tics?
sudden, brief, intermittent movements (motor tics) or utterances (vocal or phonic tics)
47
Most pts with tourette syndrome also have..
ADHD or OCD
48
Tx of tourette's syndrome?
Education and counseling for mild sxs For more bothersome: -Neuroleptics pimozide (Orap) or haloperidol off label: - Fluphenazine (dopamine antagonist) - Risperidone - Tetrabenazine
49
when can you use botox for TS?
if focal or phonic tics
50
Clinical application of Haloperidol in TS?
reduce vocal and motor tic freq. and severity
51
Pimozide MOA?
Dopamine receptor antagonist
52
Pimozoide clinical application?
Severe motor and phonic tics who failed standard treatment
53
Pimozoide ADEs?
Sedation, akathisia, akinesia | Ocular-accommodation decreased
54
What is restless leg syndrome?
overwhelming urge to move the legs, assoc. with unpleasant paresthesias worse at night and at night and relieved by movement
55
RLS is commonly assoc. with?
sleep disturbances, caffeine, stress, alcohol, and fatigue
56
What drugs are assoc. with RLS?
Antidepressants (except bupropion) Antipsychotics Dopamine-blocking antiemetics (eg, metoclopramide) Centrally-acting antihistamines
57
What is Periodic leg movements of sleep (PLMS)?
jerking movements of the leg during sleep
58
What is Periodic limb movement disorder (PLMD)
when sleep fragmentation and daytime fatigue coexist with PLMS
59
Tx for RLS?
iron replacement -if serum ferritin lower than 75 Ferrous sulfate
60
What can you give for RLS if iron replacement doesn't work?
alpha 2 delta ligand (gabapentin, pregablin) dopamine agonist (pramipexole)
61
When should you give alpha 2 delta ligand for RLS?
sleep disturbance, insomnia, painful RLS, comorbid pain syndrome, hx of current impulses control disorder, anxiety
62
When should you give dopamine agonist for RLS?
increased risk for falls, severe sxs of RLS, excess weight, metabolic syndrome, OSA, depression
63
What can be used for the tx of intermittent RLS?
Dopaminergic agents: Levodopa -Carbidopa-levodopa Benzodiazepines - mild cases of RLS, particularly in younger pts - Clonazepam, Diazepam
64
ADEs of benzodiazepines?
nocturnal unsteadiness and drowsiness or cognitive impairment in the morning*
65
Name 2 dopamine agonists. What are they used for?
Pramipexole, Ropinirole persistent RLS
66
ADEs of Dopamine agonists
Common--nausea, lightheadedness, and fatigue* Less common--nasal stuffiness*, constipation, insomnia, and leg edema* Concerning-increased risk of impulse control disorders*
67
Name 2 Alpha 2 delta calcium channel ligands. What are they used for
gabapentin, Pregabalin persistent RLS -also: peripheral neuropathy, chronic pain syndrome, insomnia/sleep disturbances*
68
When are Alpha 2 delta calcium channel ligands preferred for RLS?
in pts with impulse control disorder BUT increases risk of suicidal thoughts and behavior
69
When are opioids use for RLS?
for refractory RLS codeine, tramadol, methadone, oxycodone
70
Opioid MOA
interaction btwn. spinal opioid and dopamine receptors
71
What is augmentation?
increased sxs with increased doses of meds -main comp of dopaminergic therapy in RLS
72
What is Wilson's disease?
Recessively inherited disorder of copper metabolism impaired biliary copper excretion leads to accumulation of copper in several organs
73
Sxs of wilson's disease?
Liver: N/V, fatigue, jaundice CNS: tremors or uncontrolled movements, muscle stiffness, probs with speech, swallowing or coordination
74
Tx for Wilson's disease?
Chelating agents: D- Penicillamine, Trientine oral zinc
75
What are chelating agents? MOA?
remove extra copper from the body by releasing it from organs into the bloodstream
76
D penicillamine MOA?
Contains a free sulfhydryl group that functions as a copper chelating moiety
77
D penicillamine ADEs?
fever, cutaneous eruptions, lymphadenopathy, neutropenia, thrombocytopenia, proteinuria Give with Pyridoxine 25mg/day to prevent pyridoxal phosphate deficiency
78
Trientine MOA ?
Functions principally by removing copper from less strongly bound sites on proteins and membranes, and it increases renal copper excretion
79
Can chelating agents be used in conjunction with iron replacement?
NO
80
Trientine ADEs?
hypersensitivity rxn and pancytopenia-rare neuro sxs
81
What should you monitor in a pt taking Trientine?
24 hours copper excretion- to check for effectiveness
82
MOA of oral zinc?
interferes with the absorption of copper
83
Oral zinc ADEs?
GI upset elevation in serum amylase and lipase without evidence of pancreatitis
84
What is amyotrophic lateral sclerosis (ALS)
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
Sxs of ALS?
weakness, muscle atrophy, fasciculation, spasicity, dysarthria, dysphagia, res. compromise SPARES sensory, autonomic and oculomotor func.
86
Tx of ALS?
Riluzole Free radial scavanger: Edaravone
87
Riluzole PK?
absorbed orally, high protein bound high fat meals decrease absorption
88
Riluzole ADEs?
HTN, abn pain, neuromuscular and arthralgia, tremor, decrease lung func., elevates LFTs
89
Endaravone ADEs?
injection site contusion, gait disturbance, HA sodium bisulfite- pos. allergic rxn
90
What can you use for sxs relief of ALS: spasticity?
Baclofen Tizanidine Clonazepam
91
Baclofen MOA?
GABA B receptor agonist
92
Baclofen ADEs?
sedation
93
Tizanidine MOA?
agonists of alpha adrenergic receptors in the CNS
94
Tizanidine ADEs?
Drowsiness, asthenia, dizziness
95
Benzodiazepines MOA?
enhances the inhibitor effect of GABA A receptors
96
ADEs of benzodiazepines?
amnesia, confusion, drowsiness, slurred speech
97
What is myasthenia gravis?
Neuromuscular disease characterized by weakness and marked fatiguability of skeletal muscle defect in the synaptic transmission at the neuromuscular junction
98
Tx of MG?
standard anticholinesterase drugs: - Pyridostigmine - Neostigmine
99
MOA of standard anticholinesterase drugs?
inhibit the action of the metabolizing enzyme acetylcholinesterase