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
Q

Restless Leg Syndrome: periodic leg movements of sleep (PLMS)

A

jerking movements of the legs during sleep

26
Q

Restless Leg Syndrome: periodic limb movement disorder (PLMD)

A

sleep fragmentation and daytime fatigue with PLMS

27
Q

Restless Leg Syndrome: treatment: iron replacement

A

if serum ferritin < 75mcg/L

agent: ferrous sulfate

take with vitamin C - enhances absorption

ADE: constipation, GI upset

28
Q

Restless Leg Syndrome: alpha 2 delta ligand: clinical factor indications

A

sleep disturbance disproportionate to other symptoms

comorbid insomnia

painful restless legs

hx/current impulse control disorder

comorbid generalized anxiety disorder

29
Q

Restless Leg Syndrome: dopamine agonist: clinical factor indications

A

increased risk for falls

severe symptoms of RLS

excess weight, metabolic syndrome, OSA

comorbid depression

30
Q

Restless Leg Syndrome: drugs to avoid with a history or current alcohol or substance abuse

A

hepatically metabolized (eg: ropinirole, rotigotine)

31
Q

Restless Leg Syndrome: drugs to avoid with pregnancy risk

A

dopaminergic agents

alpha 2 delta ligands

consider instead the use of iron

32
Q

Restless Leg Syndrome: drugs to avoid with impaired renal function

A

pramipexole

avoid/adjust dose in alpha 2 delta ligands

33
Q

Restless Leg Syndrome: drugs to avoid with hepatic impairment

A

ropinirole

use caution with rotigotine patch

34
Q

Intermittent Restless Leg Syndrome: Treatment: Dopaminergic Agents

A

carbidopa levidopa

useful for specific triggers (lengthy travel, events with prolonged sitting)

avoid exceeding 200mg/day

35
Q

Intermittent Restless Leg Syndrome: Treatment: Benzodiazepines

A

clonazepam (long acting), diazepam (erratic)

for younger patients

ADEs:

  • nocturnal unsteadiness
  • drowsiness
  • cognitive impairment in the morning
36
Q

Persistent Restless Leg Syndrome: Treatment: Dopamine Agonists

A

pramipexole, ropinirole

onset: ~90-120 minutes

ADEs:

  • nausea, lightheadedness, FATIGUE
  • NASAL STUFFINESS, constipation, insomnia, LEG EDEMA
  • RISK OF IMPULSE CONTROL DISORDERS
37
Q

Persistent Restless Leg Syndrome: Treatment: Alpha 2 Delta Ca Channel Ligands

A

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
Q

Refractory Restless Leg Syndrome: Treatment: Opioids

A

low potency: codeine, tramadol

high potency: methadone, oxycodone, hydrocodone

MOA: interaction bt spinal opioid and dopamine receptors

ADEs:
tolerance
constipation

39
Q

Restless Leg Syndrome: Augmentation

A

main complication of dopaminergic pathway

results in overall inc in symptom severity with increasing doses of medication

40
Q

Wilson’s Disease

A

recessive, inherited disorder of copper metabolism

impaired biliary copper exretion –> copper accumulates in organs (liver, brain, cornea)

41
Q

Wilson’s Disease: symptoms

A

kayser fleischer rings

N/V, fatigue, muscle cramps, jaundice

tremors, muscle stiffness, problems with speech swallowing and physical coordination

42
Q

Wilson’s Disease: treatment

A

chelating agents

oral zinc

43
Q

Wilson’s Disease: Treatment: Chelating Agents

A

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
Q

Wilson’s Disease: D-penicillamine

A

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
Q

Wilson’s Disease: Treatment: Trientine Copper Chelator

A

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
Q

Wilson’s Disease: Treatment: Oral Zinc

A

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
Q

Amyotrophic Lateral Sclerosis (ALS)

A

gradual degeneration and death of motor neurons (UMN, LMN)

rapidly progressive

spares sensory, autonomic, oculomotor function (usually cognitive function too)

48
Q

ALS: signs and symptoms

A
weakness
muscle atrophy
fasciculations 
spasticity
dysarthria
dysphagia
respiratory compromise
49
Q

ALS: etiology

A

abnormal glutamate uptake –> glutamate accumulation and excitotoxic injury

50
Q

ALS: Treatment: Riluzole

A

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
Q

ALS: Treatment: Riluzole: ADEs

A
HTN
abdominal pain
neuromuscular and arthralgia, tremor
dec lung function
elevated serum transaminases
52
Q

ALS: Treatment: Edaravone

A

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
Q

ALS: Treatment: Edaravone: ADEs

A

injection site contusion, gait disturbance, headache

contains sodium bisulfite

  • allergic reactions
  • asthmatic reactions
54
Q

ALS: Symptomatic Therapy: Spasticity: agents

A

baclofen
tizanidine
clonazepam

55
Q

ALS: Baclofen

A

GABA receptor agonist

acts to restore lost inhibition by stimulating postsynaptic GABA receptors

ADE: sedation

56
Q

ALS: Tizanidine

A

alpha 2 adrenergic receptor agonist in the CNS

presynaptically stimulates GABA release from spinal cord inhibitory interneuron

ADEs:
drowsiness
asthenia
dizziness

57
Q

ALS: Benzodiazepines

A

diazepam, clonazepam

antispasticity agents
contributes to respiratory depression in late stage

enhances inhibitory effect of GABA receptors

ADEs:
amnesia
confusion
drowsiness
slurred speech
58
Q

Myasthenia Gravis

A

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
Q

Myasthenia Gravis: Treatment: Anticholinesterase

A

pyridostigmine, neostigmine

inhibits acetylcholinesterase (metabolizing enzyme)

60
Q

Myasthenia Gravis: Anticholinesterase: ADEs

A
salivation
lacrimation
urination
defecation
GI upset
emesis

cardio
respiratory
pupil constriction