Remaining Movement Disorders Flashcards

1
Q

Essential Tremor: first line agent

A

propanolol

  • beta 2 antagonist
  • depression of CNS activity
  • can cross BBB
  • prefer long acting
  • ADE: lightheadedness, fatigue, impotence, bradycardia
  • caution: heart block, asthma DM1

others:
sotalol
nadolol (less depression)

asthmatic patients:
atenolol
metropolol

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2
Q

Essenetial Tremor: other treatment options

A

alcohol
- ET related gait ataxia

nimodipine
-limb tremor associated w/ ET

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3
Q

Essential Tremor

A

rhythmic and oscillatory movement of a body part

  • constant frequency
  • variable amplitude

caused by alternating or synchronous contractions of antagonistic muscles

MC movement disorder

MC neurologic cause of postural/action tremor

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4
Q

Essential Tremor: Anticonvulsant: Primidone

A

off label

metabolite: phenobarbital

gradual inc in dose

ADEs:

  • SEDATION, drowsiness, confusion, fatigue, malaise
  • DEPRESSION
  • N/V
  • ataxia, dizziness, unsteadiness, vertigo
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5
Q

Essential Tremor: Anticonvulsant: Topiramate

A

enhances GABA
blocks voltage gated Na channel
antagonizes AMPA/kainite glutamate receptors

ADEs:

  • nausea
  • paresthesia
  • DIFFICULTY CONCENTRATING

causes weight loss

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6
Q

Essential Tremor: Anticonvulsant: Gabapentin

A

NOT GABA agonist

interacts w/ voltage gated Ca channels

ADEs:

  • sleepiness
  • dizziness
  • GAIT UNSTEADINESS
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7
Q

Essential Tremor: Botulinum Toxin Type A Injections

A

for limb tremor
associated w/ dose dependent hand weakness

may reduce head, voice tremor

prevents Ca dependent release of acetylcholine –> denervation
-only affects presynaptic membrane

ADEs:

  • breathiness
  • hoarseness
  • swallowing difficulty
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8
Q

Essential Tremor: Benzodiazepines

A

alprazolam, clonazepam

enhance GABA activity

limb tremor

dependence risk

ADEs:

  • mild sedation
  • fatigue
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9
Q

Huntington Disease

A

inherited progressive neurodegenerative disorder involving the basal ganglia

slow course

autosomal dominant - chromosome 4

GABAergic neuron is lost - acetylcholine or overactivity of dopaminergic neurons

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10
Q

Huntington Disease: symptoms

A

neurologic: chorea, dystonia, eye movement slowing, hyperreflexia, gait abnormality, myoclonus, parkinsonism
psychiatric: apathy, irritability, depression, delusions, aggression, anxiety, disinhibition, paranoia
cognitive: poor judgement, inflexibility of thought, loss of insight, dec concentration, memory loss, subcortical dementia

rigidity
bradykinesia

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11
Q

Huntington Disease: chorea

A

rapid involuntary nonrepetitive movement

involves face, trunk, limbs

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12
Q

Huntington Disease: treatment

A

supportive

chorea: tetrabenazine, deutetrabenazine

movement suppression: antipsychotic dopamine antagonist (halperidol, fluphenazine, olanzapine)

antidepressants

anticonvulsants (topiramate, levetiracetam)

amantadine

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13
Q

Huntington Disease: reduction of abnormal movements may be associated with…

A

iatrogenic parkinsonism

through low brain dopamine levels

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14
Q

Huntington Disease: Tetrabenazine (T), Deutetrabenazine (D)

A

deplete dopamine

inhibit presynaptic transporter (VMAT2)

reduce sx and severity

T: ADE: depression

D: advantage: prolonged half life (less frequent, lower dosing)

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15
Q

Huntington Disease: butyrophenone: haloperidol

A

blockade of D2 receptors&raquo_space; 5-HT2A (serotonin) receptors

clinical applications:
schizophrenia, bipolar, HUNTINGTON (chorea, dyskinesia, behavioral disturbances), TOURETTE’S

ADE: extrapyramidal dysfunction

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16
Q

Huntington Disease: Aripiprazole, Clozapine, Olanzapine, Quetiapine, Risperidone, Ziprasidone

A

blockade of 5-HT2A (serotonin) receptors > D2 receptors

clinical applications: schizophrenia, bipolar, major depression, agitation in alzheimer’s and parkinson, TOURETTE’S

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17
Q

Tourette Syndrome

A

neurologic disorder manifested by motor/phonic tics

onset: childhood

disturbance of striatal thalamic cortical (mesolimbic) spinal system –> disinhibition of motor and limbic system

comorbidities: ADHD, OCD

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18
Q

Tourette Syndrome: hallmark

A

Tics: sudden brief intermittent movements (motor) or utterances (vocal/phonic)

fluctuating severity
unpredictable pattern

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19
Q

Tourette Syndrome: treatment

A

education + counseling (habit reversal training)

Dopamine Depletion:
neuroleptics (pimozide, haloperidol)
fluphenazine
risperidone, aripirazole, ziprasidone 
tetrabenazine
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20
Q

Tourette Syndrome: haloperidol

A

D2 receptor blocker

reduce tic frequency, severity

ADE: extrapyramidal dysfunction

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21
Q

Tourette Syndrome: pimozide

A

dopamine receptor antagonist

for severe tics that failed standard treatment

ADEs: sedation, akathisia, akinesia, dec ocular accommodation

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22
Q

Tourette Syndrome: medications

A

Clonidine, guanfacine used w/ comorbid ADHD

Phenothiazines, atypical antipsychotics, clonazepam, carbamazepine: Sometimes of value

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23
Q

Restless Leg Syndrome

A

aka willis ekbom disease

overwhelming urge to move the legs

  • worse at night
  • relieved by movement
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24
Q

Restless Leg Syndrome: exacerbated by…

A
  • caffeine
  • stress
  • fatigue
  • antidepressants (not bupropion)
  • antipsychotics
  • dopamine blocking antiemetics (metoclopramide)
  • centrally acting antihistamines
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25
Restless Leg Syndrome: periodic leg movements of sleep (PLMS)
jerking movements of the legs during sleep
26
Restless Leg Syndrome: periodic limb movement disorder (PLMD)
sleep fragmentation and daytime fatigue with PLMS
27
Restless Leg Syndrome: treatment: iron replacement
if serum ferritin < 75mcg/L agent: ferrous sulfate take with vitamin C - enhances absorption ADE: constipation, GI upset
28
Restless Leg Syndrome: alpha 2 delta ligand: clinical factor indications
sleep disturbance disproportionate to other symptoms comorbid insomnia painful restless legs hx/current impulse control disorder comorbid generalized anxiety disorder
29
Restless Leg Syndrome: dopamine agonist: clinical factor indications
increased risk for falls severe symptoms of RLS excess weight, metabolic syndrome, OSA comorbid depression
30
Restless Leg Syndrome: drugs to avoid with a history or current alcohol or substance abuse
hepatically metabolized (eg: ropinirole, rotigotine)
31
Restless Leg Syndrome: drugs to avoid with pregnancy risk
dopaminergic agents alpha 2 delta ligands consider instead the use of iron
32
Restless Leg Syndrome: drugs to avoid with impaired renal function
pramipexole avoid/adjust dose in alpha 2 delta ligands
33
Restless Leg Syndrome: drugs to avoid with hepatic impairment
ropinirole use caution with rotigotine patch
34
Intermittent Restless Leg Syndrome: Treatment: Dopaminergic Agents
carbidopa levidopa useful for specific triggers (lengthy travel, events with prolonged sitting) avoid exceeding 200mg/day
35
Intermittent Restless Leg Syndrome: Treatment: Benzodiazepines
clonazepam (long acting), diazepam (erratic) for younger patients ADEs: - nocturnal unsteadiness - drowsiness - cognitive impairment in the morning
36
Persistent Restless Leg Syndrome: Treatment: Dopamine Agonists
pramipexole, ropinirole onset: ~90-120 minutes ADEs: - nausea, lightheadedness, FATIGUE - NASAL STUFFINESS, constipation, insomnia, LEG EDEMA - RISK OF IMPULSE CONTROL DISORDERS
37
Persistent Restless Leg Syndrome: Treatment: Alpha 2 Delta Ca Channel Ligands
gabapentin, pregabalin indications: - comorbid painful peripheral neuropathy - unrelated chronic pain syndrome - comorbid insomnia/sleep disturbances that is disproportionate - pts with impulse control disorder caution: inc risk of suicidal thoughts/behaviors ADE: dizziness
38
Refractory Restless Leg Syndrome: Treatment: Opioids
low potency: codeine, tramadol high potency: methadone, oxycodone, hydrocodone MOA: interaction bt spinal opioid and dopamine receptors ADEs: tolerance constipation
39
Restless Leg Syndrome: Augmentation
main complication of dopaminergic pathway results in overall inc in symptom severity with increasing doses of medication
40
Wilson's Disease
recessive, inherited disorder of copper metabolism impaired biliary copper exretion --> copper accumulates in organs (liver, brain, cornea)
41
Wilson's Disease: symptoms
kayser fleischer rings N/V, fatigue, muscle cramps, jaundice tremors, muscle stiffness, problems with speech swallowing and physical coordination
42
Wilson's Disease: treatment
chelating agents oral zinc
43
Wilson's Disease: Treatment: Chelating Agents
D penicillamine, trientine removes excess copper by releasing it from organs into bloodstream binds copper making it unavailable for activity ADEs: nervous system symptoms
44
Wilson's Disease: D-penicillamine
contains free sulfhydryl group that chelates copper depletes pyridoxine (B6) - supplement to prevent pyridoxal phosphate deficiency ``` ADEs: early onset: -fever -cutaneous eruptions -lymphadenopathy -neutropenia -thrombocytopenia -proteinuria ``` late onset: -proteinuria
45
Wilson's Disease: Treatment: Trientine Copper Chelator
lacks sulfhydryl groups removes copper and inc renal copper excretion poorly absorbed approved for children also chelates iron coadministration of iron should be avoided (nephrotoxocity) may lead iron overload in the liver ADEs: rare: hypersensitivity rxn, pancytopenia neurologic worsening
46
Wilson's Disease: Treatment: Oral Zinc
induces metallothionein to bind luminal copper - excreted in stool Forms: - zinc acetate (best absorption) - zinc gluconate (more tolerable) ADEs: GI upset elevation of serum amylase and lipase w/out clinical evidence of pancreatitis
47
Amyotrophic Lateral Sclerosis (ALS)
gradual degeneration and death of motor neurons (UMN, LMN) rapidly progressive spares sensory, autonomic, oculomotor function (usually cognitive function too)
48
ALS: signs and symptoms
``` weakness muscle atrophy fasciculations spasticity dysarthria dysphagia respiratory compromise ```
49
ALS: etiology
abnormal glutamate uptake --> glutamate accumulation and excitotoxic injury
50
ALS: Treatment: Riluzole
inhibits glutamate release inactivates voltage dependent Na channels interferes with intracellular events blocked by pertussis toxin (G protein interaction) highly protein bound (high fat meals dec absorption) metabolized by liver
51
ALS: Treatment: Riluzole: ADEs
``` HTN abdominal pain neuromuscular and arthralgia, tremor dec lung function elevated serum transaminases ```
52
ALS: Treatment: Edaravone
free radical scavenger prevents oxidative damage to cell membranes slows the decline of physical function indications: - ALS for 2yrs or less - living independently - FVC 80+%
53
ALS: Treatment: Edaravone: ADEs
injection site contusion, gait disturbance, headache contains sodium bisulfite - allergic reactions - asthmatic reactions
54
ALS: Symptomatic Therapy: Spasticity: agents
baclofen tizanidine clonazepam
55
ALS: Baclofen
GABA receptor agonist acts to restore lost inhibition by stimulating postsynaptic GABA receptors ADE: sedation
56
ALS: Tizanidine
alpha 2 adrenergic receptor agonist in the CNS presynaptically stimulates GABA release from spinal cord inhibitory interneuron ADEs: drowsiness asthenia dizziness
57
ALS: Benzodiazepines
diazepam, clonazepam antispasticity agents contributes to respiratory depression in late stage enhances inhibitory effect of GABA receptors ``` ADEs: amnesia confusion drowsiness slurred speech ```
58
Myasthenia Gravis
neuromuscular disease characterized by weakness and marked fatigability of skeletal muscle defect at the synapse - autoimmune response to ACh receptor at the end plate
59
Myasthenia Gravis: Treatment: Anticholinesterase
pyridostigmine, neostigmine inhibits acetylcholinesterase (metabolizing enzyme)
60
Myasthenia Gravis: Anticholinesterase: ADEs
``` salivation lacrimation urination defecation GI upset emesis ``` cardio respiratory pupil constriction