movement disorders

Question 1

benign essential tremor?

Answer 1

cause is unknown, often inherited in autosomal dominant manner (familial)

-causes minimal disability

Question 2

when can the B. Essential tremor begin?

Answer 2

at any age

Question 3

what can make the tremor worse?

Answer 3

emotional stress

Question 4

what can relieve a benign essential tremor?

Answer 4

small quantities of alcohol

Question 5

what are the Clinical features of BET?

Answer 5

rhythmic, 6-8 hz, to and fro motion, usually of the upper extremities, but sometimes of the head

-speech can also be affected if it involves the laryngeal muscles

Question 6

what is titubation?

Answer 6

the act of staggering or reeling; a staggering gait with shaking of the trunk and head, commonly seen in cerebellar disease.

Question 7

tx fo BET?

Answer 7

low doses of BB, usually propanolol]

-primidone if BB fails

Question 8

what is primidone?

Answer 8

MOA: barbituate, acts on GABA receptors, increasing synaptic inhibtion (metabolized to phenobarbital)

ADR: ataxia, vertigo, N/V, diplopia

Question 9

Parkinson’s disease?

Answer 9

-second most common neurodegenrative disorder

Question 10

idiopathic parkinson? epidemiology

Answer 10

occurs in all ethnic groups, equal sex distribution, and most often begins btw 45-65 years old +/- familial component

Question 11

what is the Pathophys of PD?

Answer 11

degenration of cells in the substantia nigra= deficiency of the neruotransmitter DA and an imbalance of DA and acetylcholine

Surviving substantia nigra neurons often contain Lewy bodies, which are intracytoplasmic proteinaceous inclusions (comprised of alpha-synuclein)

Question 12

what are some complaints that PD pts may have?

Answer 12

slowed movements, difficult arising from a sited position, climbing up and down stairs, trouble getting dressed and difficulty w/ handwriting *micropahgia

Question 13

What are the essential featrus that est a dx of PD?

Answer 13

resting tremor, bradykinesia, rigidity (check the muscle tone), and postural instability (shufflig gat)

Question 14

what is normally the presenting complaint or PD?

Answer 14

-tremor that is most noticeable at rest, at 4- cycles per sec, and may be only very slifht with voluntary effort

“pill rolling”

Question 15

how does the tremor of PD progress?

Answer 15

initially confied to one limb or the limbs on one side, but eventually, it may be present in all the limbs and the lips and mouth. bypasses the head

Question 16

what may bradykinesia look like in a PD pt?

Answer 16

generalized slowness of voluntary movements evident in the slow shuffling gait, reduced arm swing, slowed rapid alternating movements, infrequent blinking and mask like features

Question 17

what does rigidity look like in a PD pts?

Answer 17

found on passive ROM testing- think cogwheel rigidity

Question 18

what does postural instability look like in a PD pt?

Answer 18

difficulty in standing from a seated position, unsteadiness on turning, difficulty in stopping, and a tendency to fall

Question 19

what cognitive disorder/ sx can be seen in PD pts?

Answer 19

depression, dementia, cognitive impairment,

Question 20

diagnostic testing for PD?

Answer 20

none, blood tests and imaging studies can be done to r/o other causes

Question 21

what is the tx of PD?

Answer 21

balance acetylcholine w/ anticholinergic drugs and levodopa (precursor of dopamine)

Question 22

what can work in pts w/ advanced PD w/ dyskinesias?

Answer 22

amantadine

Question 23

what is the first line tx of parkinsons

Answer 23

levodopa-crosses bBBB and is converted to dopamine in body. can be used w/ carbidopa, that allows lower doses of levodopa and reduced side effects

Question 24

what does carbidopa do?

Answer 24

Inhibits dopa decarboxylase (DDC)
Prevents peripheral conversion of levodopa to dopamine
Allows more levodopa to cross BBB
Increases bioavailability

Question 25

what do you need to monitor in pts on levodopa?

Answer 25

LFTs

Question 26

what are adverse effects of levodopa?

Answer 26

Dyskinesias
On-off” Effect – fluctuations in clinical response to levodopa
End of dose wearing off
“Off” = marked akinesia
“On” = improved mobility but marked dyskinesia
Thought to be related to fluctuations in levodopa plasma concentrations
Fluctuations can be “smoothed out” by incorporating a dopamine receptor agonist into pharmacotherapy

Also: N, anorexia, hypotension, confusion, insomnia, nightmares, schizophrenic-like syndrome

Question 27

when are dopamine agonists used in PD?

Answer 27

for pts that have become refractory to levodopa and carbidopa

(bromocriptine, cabergoline)

Question 28

what other options that can be used for tx of PD?

Answer 28

Monoamine oxidase B inhbitors: selegiline

catecholamine-O-methytransferase inhibitors: tolcapone and entacopone

Question 29

how do MAO-B work?

Answer 29

inhibits breakdown of dopamine

Question 30

what are some ADR of dopamine agonists?

Answer 30

cardiac valvulopathy when used at PD dosing

Question 31

how do COMT inhibitors work/

Answer 31

reduce the metabolism of levodopa to #-O methyldopa and result in more stable plasma levels and more constant dopminergic stimulation of the briain

Question 32

what are non-drug tx for PD

Answer 32

PT, household modifications, special utensils

psychological support

Question 33

what are some anticholinergic drugs that can be used for PD?

Answer 33

benztropine, trihexyphenidyl, amantadine

Question 34

Huntington Disease

Answer 34

inherited, autosomal dominant disorder

-5/100,000

Question 35

what is the gene responsible fo r HD?

Answer 35

on the short arm of chromosome 4

expansion of CAG trinucleotide

*number of repeats determines the age of onset
> 40, sx will appear

Question 36

when do sx of HD develop?

Answer 36

after 30 yo

Question 37

CF of HD?

Answer 37

progressive chorea and dementia

usually fatal w/in 15-20 years

Question 38

what are the earliest mental changes of HD?

Answer 38

behavorial, with irritability, moodiness, and antisocial behavior that generally progresses to an obvious dementia

Question 39

what are the earliest physcial signs of HD?

Answer 39

mere restlessness of fidgetiness, but eventually severe choreiform movements and dystonic posturing

Question 40

what is chorea?

Answer 40

– rapid involuntary dance-like movements involving the face, trunk, and/or limbs

Question 41

diagnositic studies of HD

Answer 41

CT will show cerebral atrophy, and atrophy of the caudate nucleus

MRI and PEET scans show decreased glucose metabolsim in an anatomically normal caudate nucleus

Question 42

tx of HD

Answer 42

no cure, and progression can’t be halted

-symptomatic tx: phenothiazines to control dyskinesia and haloperidol or clazpine to control any behaviorl disturbances

Question 43

what is a drug that can help control chorea?

Answer 43

tetrabenazine, but it can worsen cognitive problems

mechanism of action involves blocking the transport of dopamine into vesicles in the presynaptic terminal. This depletes dopamine from the vesicles and reduces dopamine transmission