Motor disorders: Pharmacology

Question 1

What is blepharospasm?

Answer 1

Involuntary forcible closure of the eyelids, affecting one or both eyes causing functional blindness
Eyes and visions are normal

Question 2

What is the treatment for blepharospasm?

Answer 2

Injection of botulinum toxin type A

Takes 3-5 days to work but lasts 3-6 months

Question 3

How does botox type A work for blepharospasm?

Answer 3

Acts on MNJ presynaptically by inhibiting release of AcH, resulting in local muscle weakening and reduction in spasms

Question 4

What are the side effects of botox type A for blepharospasm?

Answer 4

Eyebrow weakness or eyelid closure, blurry/double vision, lacrimation problems, asymmetric smile, and drooling (only last a few days/weeks)

Question 5

What is a hemifacial spasm?

Answer 5

Frequent involuntary contractions of the muscle on one side of the face

Question 6

What is a dystonic tremor?

Answer 6

Pt already has dystonia, muscular contractions are sustained in contraction

Question 7

What is cervical dystonia?

Answer 7

Spamodic torticollis and is the most common, abnormal positions of head and neck

Question 8

Botox type A has a high affinity for the _____.

Answer 8

Neuromuscular junction

Question 9

What does botox do with the light chain?

Answer 9

It cleaves SNARE proteins causing no released of AcH from the NMJ

Question 10

What are the characteristics of and essential tremor (ET)?

Answer 10

One of the most common tremor disorder
Kinetic postural tremor (a majority in the upper limbs)
Absence of other neurological signs, and possible isolated head tremor

Question 11

What are some other features of ET?

Answer 11

Tremor amplitude inc over time, inc difficulty w/ fine motor tasks, “benign” (doesn’t reduce life)

Question 12

What is the treatment for the essential tremor?

Answer 12

Beta blockers (works 50%) of the time, propanolol and primidone
Beta 2 more effective

Question 13

What is the surgical treatment for ET?

Answer 13

Thalamotomy (treats contralateral limb)
Deep brain stimulation in thalamus
Indications for bilateral surgery

Question 14

What is hemiballismus?

Answer 14

A loss of function of the subthalamus leading to jerking, ballistic motion

Question 15

When does Huntington’s disease appear and how?

Answer 15

Usually by age 40, generational (genetic)

Question 16

What is huntington’s?

Answer 16

Disease to the extrapyramidal system, disinhibition of dopaminergic nigro-striatal pathway
Excessive uncontrollable moments of head, face, trunk, limbs
Mood swings, depression, huntington’s dance, difficulty eating/driving

Question 17

What occurs with a loss of GABA inhibition?

Answer 17

Dopamine hyperactivity leading to involuntary, writhing movements

Question 18

What drugs should not be used for huntingtons?

Answer 18

Levadopa and bromcocriptine

Question 19

What drugs should be used for huntingtons?

Answer 19

Dopamine antagonists (clorpromazine)
GABA agnoists (baclofen)

Question 20

What is huntington’s disease characterized by?

Answer 20

Degeneration of the cerebral cortex and basal ganglia

Question 21

What drugs would you use to treat the depression and psychosis of huntington’s respectively?

Answer 21

Fluoxetine and antipsychotic drugs

Question 22

What drugs are used to treat the rigidity and seizures in huntington’s respectively?

Answer 22

Clozapine/quetiapine and clonasepam/valproic acid

Question 23

What is the treatment for the chorea (which causes falling) in huntington’s?

Answer 23

Tetrabenazine or reserpine

Question 24

What is tetrabenazine?

Answer 24

Non-competitive inhibitor of monoamine transport into synaptic vesicles

Question 25

What are the adverse reactions to tetrabenazine?

Answer 25

Allergic rxn, blue/purple skin patches, behavioral changes (hurting others or self), sweating, confusion, muscle stiffness, dec appetite, body aches

Question 26

What increases the symtpoms of tourette’s syndrome?

Answer 26

Stress, caffeine stimulants, fatigue, heat, steroids

Question 27

What decreases the symptoms of tourette’s syndrome?

Answer 27

THC, alcohol, nicotine and dec in mental activity

Question 28

What drug will reduce tourette’s by 50%?

Answer 28

Clonidine or guanfacine-alpha 2 adrenergic agonist

Question 29

What drugs should be used if the tics are severe?

Answer 29

Haloperidol or primozide

Question 30

What is the pathophysiology of ALS?

Answer 30

Degeneration motor neuron disease that affects UMN and LMN lying within the brain, spainl cord and peripheral nerves

Question 31

Describe riluzole.

Answer 31

Orally active, highly protein bound
MOA - enhances reuptake of glutmate by astrocytes and neurons
Blocks NMDA and kinate receptors and inhibits voltage gated Na channels

Question 32

What drug is used for spasticity?

Answer 32

Baclofen

Question 33

What are the characteristics of baclofen?

Answer 33

Orally active, plasma half life 3-4 hrs, GABA(B) agonist on nerves in spinal cord

Question 34

What are the adverse effects of baclofen?

Answer 34

Drowsiness, nausea, weakness, dec muscle tone, possible seizures on withdrawal

Question 35

What is restless leg syndrome (RLS)?

Answer 35

Leg dysthesias exacerbated by inactivity, relieved by movement; results in sleep onset delay

Question 36

What is periodic limb movement disorder (PLMD)?

Answer 36

Bried, repetitive jerking movements of lower extremities during stage 1 and 2 of sleep; results in arousals, sleep fragmentation

Question 37

What is the monoamine mechanism of RLS/PLMD?

Answer 37

Dopamine

Question 38

What are the diagnostic clues for RLS/PLMD?

Answer 38

Caffeine, TCAs, SSRIs, positive family history, association with Fe deficiency anemia (ferritin), neurological lesions

Question 39

Which age is the most common for RLS onset?

Answer 39

20-29

Question 40

Most drugs to treat RLS and PLDM are…..

Answer 40

Off label