0:29

Hello and welcome back to the Neurotransmitters

0:31

, your source for everything regarding clinical neurology

0:34

. Today we are continuing

0:36

our Neurology IM Board

0:38

Reviews series , part 6 today

0:40

. And today we are hitting movement

0:43

disorders . As always , I want to

0:45

remind everyone that this is not an exhaustive

0:47

review on all of these entities . This

0:49

is an overview intended to help people

0:51

preparing for their internal medicine , in-service

0:54

exams or

1:00

board reviews . With that being said , one of the main ways to differentiate different kinds of

1:02

movement disorders is whether you have too much or too little movement

1:04

, that is to say , hyper versus hypokinetic

1:07

. So let's start with some of our hypokinetics

1:10

, so people who have some reduced movements

1:12

. The classic example of this is Parkinsonism

1:15

, and you'll notice I say ism at the end there because

1:18

there are things that can have Parkinsonian

1:20

features that are not Parkinson's

1:22

disease . So Parkinsonism

1:25

we generally define as kind of slowing

1:27

of movements , rigidity , resting

1:30

tremors , postural instability , and

1:32

since this is Parkinsonism , let's start

1:34

off with Parkinson's disease . So

1:36

the main acronym that people usually learn

1:38

is TRAP T for

1:40

tremor at rest . Now , this

1:43

is a resting tremor . Obviously , this tends to be

1:45

a low amplitude , high frequency

1:47

tremor that we typically see when

1:49

people are sitting arms relaxed at

1:51

their side , but you can also see

1:53

it when people outstretch their arms in a postural

1:56

kind of position and this tremor

1:58

may re-emerge after a few moments . The

2:01

R is for rigidity , so

2:03

a lot of people will have heard the term lead pipe

2:05

rigidity . Now , I don't know about you

2:07

, but I don't recall the last time I tried

2:09

to passively bend a lead pipe with my own two hands . I tried to passively bend a

2:11

lead pipe with my own two hands . But

2:14

, that being said , the idea is

2:16

that rigidity is not velocity

2:19

dependent . It doesn't matter how fast

2:21

or how slow you try and bend that lead pipe

2:23

. You're going to get the same amount of resistance . And

2:26

this is in contrast to spasticity

2:28

, or what you'll sometimes see in older

2:30

books as clasp knife spasticity

2:32

. So if you've ever tried to open a pocket knife , many

2:39

of them will have a little resistance to them and then they suddenly pop open the

2:41

rest of the way and stay open . Similarly , spasticity is a velocity dependent

2:43

resistance to passive movement

2:45

. So the faster you move a limb , the

2:47

more resistance you tend to get . Next

2:49

is for akinesia , or bradykinesia

2:52

. This is a reduction not

2:54

just in the speed of a movement but also

2:56

in the amplitude of a movement . Some tests

2:58

you might do during your exam . To evaluate this would

3:00

be finger tapping having people open

3:02

or close their hands , tapping their toe and

3:05

you do want to try and do this to a rhythm , so either tapping

3:07

your foot or clapping your hands , or

3:09

giving them some kind of metronome to

3:11

which you want them to adhere so you can tell

3:14

if they're slowing their movements a little

3:16

more subtly . And lastly , we have

3:18

P for postural instability

3:20

, and this can manifest in several

3:22

different ways . One of the classic ones

3:24

is gait freezing . People will be walking

3:26

and all of a sudden , their feet feel like they're stuck to the ground

3:28

. They just can't lift them off , and this can result in

3:31

falls . Obviously , some situations that

3:33

may trigger this are when someone is passing through a doorway

3:35

or when they're walking across a floor and

3:37

it goes from carpet to a tile or vice versa

3:39

. You will also see what's called

3:41

on-block turning , so they will take many

3:44

steps and they don't really turn their shoulders in

3:46

advance of the rest of their body . You may also

3:48

see a stooped posture . Sometimes this

3:50

is called camptochormia , and this is

3:52

a very stooped . Sometimes this is called camptochormia , and this is a very

3:54

stooped . Forward-flexed posture of the trunk , and again

3:56

, that acronym is TRAP , tremor

3:59

at rest rigidity , akinesia , slash

4:01

bradykinesia and postural instability

4:03

. Now there are other symptoms , of course

4:05

. Some of those ones may include a masked

4:07

face , so reduced facial expression

4:09

. Hypophonia , so their voice

4:11

will become very quiet . Micrographia

4:14

, so their voice will become very quiet . Micrographia , so

4:16

their writing may become very small . Now with Parkinson's disease

4:19

, a lot of people end up fixated on the motor symptoms because

4:21

that's what's easy to see . But there

4:23

are many non-motor symptoms as

4:25

well , things that can come along with

4:27

Parkinson's disease as it progresses . You

4:29

can develop dementia . There can be

4:31

associated depression or anxiety , pseudobulbar

4:34

affect or emotional incontinence . You

4:36

can get sleep disorders , a common one

4:38

being REM sleep behavior disorder , where people

4:41

kind of act out their dreams . We'll talk more about that

4:43

later . Autonomic dysfunction

4:45

also very common . Constipation

4:47

, gut motility issues , sialorrhea

4:50

so drooling can become an issue hyposmia

4:52

, loss of smell , musculoskeletal

4:54

issues like I mentioned , the camptochormia . Sometimes

4:57

that can lead to back pain , poor posture

4:59

. You can also develop dystonias

5:01

, especially when we start getting on higher doses

5:03

of treatments for Parkinson's disease

5:05

. You can also get pain from rigidity

5:07

, visceral pain , again related to that , constipation

5:09

. So many things that you need to be aware of

5:11

with these patients , especially as the disease

5:13

progresses . In particular I want to

5:15

emphasize two big risks that you want

5:17

to assess for in these patients . So one is depression

5:20

screening so making sure you're using a validated tool

5:22

to screen for depression in these patients

5:24

is appropriate . And the second are falls

5:26

. We want to make sure that we're assessing gait on a regular

5:29

basis in these patients . Now

5:31

, one of the main tests that maybe not everyone

5:33

does is something called a pull test

5:35

. So you are going to stand

5:37

behind the patient . I recommend

5:39

that you have your back to a wall and

5:41

you're going to tell the patient what you're

5:43

doing . You're going to place both hands on their shoulders

5:45

and you're going to give them a brisk pull , and

5:48

if they take more than just a few steps

5:50

backwards , that's considered a positive pull

5:53

test and that suggests that they are at

5:55

increased risk of falls . Now I want to emphasize

5:58

having your own back to the wall

6:00

, especially if you are a smaller person and your

6:02

patient is on the larger side , because

6:04

sometimes with Parkinson's , the patient may

6:06

fall right over like a tree going down , and

6:08

so if they are larger than you , you

6:10

will be going down with them . So having the back

6:13

to your wall means that you just get a little bump against

6:15

your back , as opposed to both of you hitting the ground . Now

6:17

there are obviously other components to

6:19

the gait evaluation , but this is one

6:22

that not everyone is probably thinking about

6:24

if you are mostly working in the internal medicine

6:26

or primary care clinics . So

6:28

let's talk a little bit about treatment

6:30

, particularly pharmacologic treatment for

6:32

Parkinson's disease . So most of our treatments

6:35

revolve around dopamine therapy

6:37

, right ? The idea is that we have loss

6:40

of dopamine producing neurons and

6:42

we need to replace that dopamine

6:44

. So the main medication is

6:46

carbidopa , levodopa , or brand name

6:48

, is Sinemet . So there are different formulations

6:51

of carbidopa , levodopa there's immediate

6:53

release , extended release , continued

6:55

release , enteral gel . So our

6:58

main one that we're going to be using most of the time

7:00

is this immediate release formulation and that's

7:02

usually going to be dosed at least three

7:05

times daily and it is in a combination

7:07

tablet , right ? Carbidopa helps prevent peripheral

7:09

metabolism of the levodopa . We

7:12

found that levodopa by itself tends to cause

7:14

lots of emesis , orthostatic

7:23

hypotension , hence the name Sinemet , sin without emet , emesis vomiting so without vomiting is kind

7:25

of the idea there , one of those clever brand names that you come across from time to

7:27

time . This is my own personal experience

7:29

and not an official recommendation . I usually

7:31

start with the 25-100 combination

7:34

tablets . I'll usually start those three

7:36

times daily and I tend to increase

7:38

in frequency more so than in the

7:40

single dose . Or , as one of my instructors

7:43

told me years ago , you are doing fence

7:45

posts , not telephone posts , in terms of

7:47

your dosing . So you want lots

7:49

of small , frequent doses rather than

7:51

a few big high doses

7:53

. Now , some of these extended releases out

7:55

there can help alleviate the need

7:57

for frequent medication dosing , as sometimes

8:00

the regimens can become quite cumbersome

8:02

in terms of how often someone's supposed to be taking their

8:04

medication . One of the theories that it is important

8:06

to keep in mind is the narrowing therapeutic

8:09

window over time with Parkinson's

8:11

disease . So when we say the therapeutic window

8:13

is narrowing as the disease advances

8:15

, what we mean is that normally you

8:18

take a dose of carbidopa , levodopa , you

8:20

have a period of effect right

8:22

the on time , and this is

8:24

where maybe rigidity is reduced or

8:26

tremors are reduced , what have you and

8:29

Then it wears off , the symptoms come

8:31

back . So as we go on

8:33

, we start seeing more off time

8:35

and we also start seeing periods

8:37

of too much dopamine activity or

8:40

peak dose dyskinesias or

8:42

other peak dose type side effects , and we'll talk

8:44

about some of the management strategies for that . So

8:46

that is why people tend to lean towards these

8:48

frequent small doses rather than fewer

8:51

high doses , because that

8:53

window over time starts to narrow

8:55

and so you end up overshooting and undershooting

8:58

more often with less frequent dosing

9:00

. At least , that is one of the theories of treatment

9:02

. Other medications that we'll sometimes

9:04

use include dopamine agonists , so Pramopexol

9:07

, rapinrol . There is often a debate going

9:09

back and forth about which should you start

9:11

first carbidopa , levodopa or

9:13

dopamine agonists . Some

9:15

of the thought was that if you start carbidopa

9:17

levodopa too early , you are more likely to

9:20

lead to dyskinesias earlier

9:22

. At the time that I'm recording this , in April 2024

9:24

, the trend is more towards starting carbidopa

9:26

levodopa over dopamine agonists , partially

9:29

related to some of the side effects , and that

9:31

there isn't really that much difference in dyskinesia

9:33

development . So something to keep in mind with

9:35

all of our dopaminergic therapies is

9:37

that as you increase dopamine , the risk for certain

9:39

kinds of side effects does increase

9:42

, in particular impulsivity , behavior

9:44

changes , hallucinations , things of that

9:46

nature . With carbidopa levodopa

9:48

we also see some risk for peripheral

9:50

edema and orthostatic hypotension

9:52

creep in as the dose goes up . Also

9:55

, another class of medications that are

9:57

sometimes used are the catechol-O-methyltransferase

10:00

inhibitors , or COMT , and

10:02

these are Entocopone and Tolcopone

10:04

Entocopone the brand name is Compton

10:07

, right ? Another one of those clever brand names there

10:09

, and this helps to prolong

10:12

the levodopa effect , right ? The idea is that it's

10:14

kind of blocking that peripheral metabolism again

10:16

. Another class are the monoamine

10:18

oxidase type B inhibitors

10:20

, or MAOI-Bs , and

10:23

selagiline and resagiline are the ones here

10:25

. Now I mentioned peak dose

10:27

dyskinesias , that is to say , dyskinesias

10:29

are excessive movements that tend to occur

10:31

as the dopamine overshoots the

10:33

therapeutic window , and one

10:36

medication that is used are these glutamate

10:38

NMDA antagonists or amantadine . So

10:40

amantadine can sometimes help with these

10:42

side effects as well . As we mentioned

10:44

earlier , hallucinations , particularly visual

10:47

in nature , can develop with

10:49

Parkinson's disease . Usually later

10:51

, if it's early , we start thinking about one of our Parkinson's

10:53

plus quote-unquote syndromes and we'll

10:55

talk about those later . So as

10:58

far as treatment of these hallucinations , quetiapine

11:01

is very commonly used . We usually

11:03

are talking about low doses , right ? If we're using

11:05

dopamine blocking therapy in

11:07

someone whom we're also giving dopaminergic

11:09

therapies . So we don't want to really be

11:11

self-defeating and giving medications that

11:13

counteract what we're trying to do in the first place

11:15

. So that is why we tend to use lower

11:18

doses of quetiapine . You'll

11:20

also see a lot of movement disorder specialists

11:22

using clozapine , which is

11:25

also very well tolerated . As far as the Parkinson's

11:27

side of things , but you do have to be very careful

11:29

in terms of the other side effects with clozapine

11:32

, particularly our white blood cell count . There

11:34

is a relatively new medication on the

11:36

market for Parkinson's psychosis

11:38

specifically , and that is pimivanserin

11:41

. Now , this is a different mechanism . This is a

11:43

selective serotonin 5-HT2B

11:46

receptor inverse agonist

11:48

. That is a lot to remember , but

11:51

this medication is not supposed to have the same dopamine

11:53

blockade issues that other things

11:55

for Parkinson's related hallucinations

11:57

or psychosis may have . So it's

11:59

definitely something to keep in mind , although cost may

12:02

be a limiting issue in some situations . As

12:04

we mentioned earlier , you can get some autonomic

12:06

issues . In particular , orthostatic hypotension

12:09

another fall contributor can be very

12:11

common in Parkinson's disease . So

12:14

one of the medications that we'll sometimes use is a norepinephrine

12:16

precursor and it's droxidopa , and

12:19

so that can sometimes be used in people who are having

12:21

issues with blood pressure management , as

12:23

well as more traditional blood pressure elevating

12:25

medications . It's also important to remember the

12:27

non-pharmacologic options as far as compression

12:29

stockings , a high salt-containing diet

12:31

and things of that nature , and

12:38

I also want to mention deep brain stimulation . So this is primarily for patients who are

12:41

having severe motor fluctuations or they're having refractory tremor . You also tend

12:43

to avoid DBS in patients who are already

12:45

starting to develop some signs of dementia , as

12:47

they don't tend to have as good of outcomes as

12:49

those who don't when they're undergoing this kind of surgery

12:52

. The targets for DBS there

12:54

are various ones , but the classic ones

12:56

are the subthalamic nucleus and the globus

12:58

pallidus interna . Now we did

13:00

spend a fair amount of time just talking about the

13:02

pharmacologic management of Parkinson's

13:05

disease , but just as important

13:07

, you need to make sure that these patients are undergoing

13:09

exercise regularly . There are

13:11

certain kinds of physical therapy protocols

13:13

the big and loud protocol , where

13:16

we focus a lot on the hypophonia , on

13:18

exaggerated movements , the kinds of activities

13:20

that people with Parkinson's tend to have

13:22

more difficulty with . So it is important to

13:24

take a multidisciplinary approach to

13:26

these patients and focus on the basic

13:29

things as well . And again , don't forget about those

13:31

non-motor symptoms of constipation

13:33

, sialorrhea or hypophonia , as

13:35

these things can be quite disturbing to people's

13:37

quality of life . Let's change gears slightly

13:39

and talk about the Parkinson's Plus syndromes

13:42

. These are often called Parkinson's Plus

13:44

as they have Parkinsonism plus

13:47

an additional finding on exam

13:49

or by history that makes you think this is just

13:51

a little different than it should be . Now

13:53

there are a lot of different things that can go into

13:55

the diagnosis of these different entities . But

13:58

again , right , this is a more superficial look

14:00

and we're going to focus on just a couple salient

14:02

points on each one of these that will

14:04

hopefully identify them in the question

14:06

stem or maybe even in real life for

14:08

you . So first of all , we have progressive

14:11

supernuclear palsy , or PSP

14:13

. Now the two things that you'll see sometimes

14:15

in a question stem that should make you think PSP

14:17

if someone has had a relatively

14:20

short duration of Parkinsonism

14:22

and they are falling a lot

14:24

, so early falls is a big one . Now

14:26

, another thing they may also mention is that there is

14:28

impairment of vertical gaze . Right

14:31

, they supernuclear , can't move their eyes

14:33

that well , so early falls , vertical

14:35

eye movements , think PSP . Next

14:38

up , corticobasal degeneration or corticobasal

14:41

syndrome . So this tends to

14:43

be more unilateral and

14:45

you tend to see on imaging you might have atrophy

14:47

of one hemisphere or one basal ganglia

14:50

and sometimes you'll see that they

14:52

have the alien hand syndrome . So one

14:54

hand is kind of doing its own thing and it feels like the patient

14:56

can't control that hand . So it tends

14:58

to be very unilateral as compared to

15:01

a lot of these other syndromes , although

15:03

in the early stages of Parkinson's you

15:05

may have a more unilateral resting

15:07

tremor , but this tends

15:09

to be much more marked . You may also

15:12

see something like associated neglect or

15:14

sensory disturbances on the side that is

15:16

affected as well in corticobasal

15:18

degeneration , multiple system atrophy

15:20

or MSA . This is characterized

15:23

classically by a very prominent orthostatic

15:25

hypotension . Lots of dysautonomia

15:27

is the classic form . So MSA

15:30

does come in three different subtypes MSA-A

15:33

for autonomic , msa-c for

15:35

cerebellar and MSA-P for Parkinsonian

15:37

. So the autonomic type obviously

15:40

has more autonomics involved

15:42

. They all do to an extent . The

15:44

MSA-C might have some more ataxia

15:46

and the MSA-P has a lot

15:49

of Parkinsonism . But in a question

15:51

vignette I would expect to hear a lot about

15:53

very early orthostatic hypotension

15:55

, erectile dysfunction , urinary

15:58

retention , a lot of these autonomic

16:00

issues . Lastly , we have dementia with

16:02

Lewy bodies or DLB . Now

16:04

we did mention DLB a bit in our

16:06

dementia podcast earlier in this series

16:08

, so do look back at that if you want

16:10

more about Lewy body . But the main things

16:13

that you want to think about with Lewy body

16:15

versus Parkinson's disease is

16:17

that the cognitive changes and the visual hallucinations

16:20

will happen earlier with DLB as

16:22

opposed to Parkinson's disease . So

16:24

typically the rule of thumb is if you

16:26

have cognitive changes before motor think

16:29

Lewy body , if you have motor before cognitive

16:31

think Parkinson's disease . This is

16:34

, of course , an oversimplification and it is a

16:36

spectrum , but on the test that's the

16:38

best way to think about it . So hopefully by now you're

16:40

getting the picture that just because someone may have Parkinsonism

16:43

does not mean they have Parkinson's disease

16:45

. So we've mentioned several different disorders

16:47

that can have Parkinsonian features to

16:49

them , but there are others , so

16:51

a couple again that we mentioned in our dementia podcast

16:54

normal pressure hydrocephalus may develop

16:56

some Parkinsonian features and vascular

16:58

dementia may also develop some Parkinsonian

17:00

features . So , again , always

17:03

important to get the MRIs and make sure

17:05

that you don't see signs that are suggestive of

17:07

NPH or old strokes that might

17:09

point you in a different direction . You can

17:11

still try dopamine therapy in these patients

17:14

who may have Parkinsonism , as opposed

17:16

to traditional Parkinson's disease , and

17:18

you may get some effective results in some people

17:20

. However , you should not expect

17:22

all people to respond as if they had

17:24

Parkinson's disease . A last category

17:26

of Parkinsonism I want to mention is drug-induced

17:29

Parkinsonism . So we talk about our extrapyramidal

17:31

symptoms , right ? So all the things we've been talking

17:33

about , the resting tremors that we tend to see from

17:36

drug-induced Parkinsonism tend to be a

17:38

little more symmetric bilaterally

17:40

and they tend to occur obviously

17:42

from antipsychotics most often

17:44

, whether those are typical or atypical

17:46

antipsychotics . Now , something you may

17:48

not think about is metoclopramide . So

17:51

we have a lot of folks in nursing homes who are

17:53

on Reglan or metoclopramide for

17:55

gut issues , and so it

17:57

is important to remember this is very often

18:00

a cause of drug-induced Parkinsonism

18:02

. A test that can sometimes be useful

18:04

if the clinical exam isn't pointing in a specific

18:06

direction is a DAT scan . This can

18:09

help differentiate a tremor due to basal

18:11

ganglia dysfunction versus

18:13

drug-induced , although certain medications

18:15

can confound it , particularly

18:17

some antidepressants . So you do have to look at the med

18:19

list and come off of those medications for

18:21

a couple weeks if possible . So

18:23

we talked about hypokinetic , let's talk about hyperkinetic

18:26

. So this comes in many different flavors

18:29

, so let's go through them . First up , we have tremors

18:31

. These are regular oscillations around

18:34

a joint , typically , so they tend to be rhythmic

18:36

, and there are many different tremors

18:38

. We're going to focus on some of the ones that are likely

18:40

to be a little more high value . Essential

18:42

tremor is probably one of our more common tremors . It

18:44

tends to involve the upper extremities . You

18:47

can see this is worse with action

18:49

and posture , but it can also

18:51

affect the head and voice . You

18:53

can sometimes get what's called a yes-yes or

18:55

no-no tremor , when the head is shaking

18:58

either like they're saying yes or no , and

19:00

so this can be very annoying to some people also

19:02

. So there are various medications that we will

19:04

try Propranolol , primidone those

19:07

are kind of our classic agents . To pyramate

19:09

is also tried . Second line agents

19:11

include things like clonazepam

19:13

, gabapentin , other beta blockers

19:16

. You can sometimes try botulinum injections

19:18

for neck tremors or voice

19:20

predominant tremors , although you do want those done by

19:23

someone who really knows what they're doing , as there's a lot

19:25

of important structures there . Deep brain stimulation

19:27

can also be an option for these patients

19:29

as well . The target is different

19:31

than in Parkinson's disease . Here it is the

19:33

ventral intermediate nucleus of the thalamus

19:36

. Non-pharmacologically speaking , there are various

19:38

weighted tools or different modifications

19:41

that can be done to silverware or various

19:43

other things that people will often have difficulty with

19:45

. Trying to bring a full

19:47

glass to someone's mouth or

19:50

a spoonful of soup can be very

19:52

challenging for some of these people , so there are different

19:54

options that an occupational therapist can

19:56

provide to these people . Another common

19:58

tremor is the enhanced physiologic tremor

20:00

. Now everyone has a little bit of a shake

20:02

to their hands if they're stressed or tired , and

20:05

this tends to be worse with stress

20:07

or anxiety or other kinds

20:09

of medications Caffeine . So the treatment

20:11

in these people is usually finding if there is any

20:13

recent triggers or life changes

20:16

that may be contributing to worsening of this tremor

20:18

. So mostly you're counseling about sleep , stress

20:20

and caffeine intake . Another very common

20:22

category are drug-induced tremors . This

20:25

can be from a variety of different classes beta

20:27

agonists , so folks using their albuterol

20:29

inhalers , people on lithium , valproic

20:32

acid , amiodarone , glucocorticoids

20:34

, different

20:39

antidepressants like amitriptyline or fluoxetine . So there are many medications that

20:41

can cause a drug-induced tremor . So if someone has a new tremor you want to find

20:43

out have they been on any new medications

20:46

that correspond temporally with

20:48

the onset of that tremor ? There are other

20:50

tremors , such as rural or cerebellar , but

20:52

those tend to be less common so we won't spend too much

20:54

time on them today . Moving on to

20:57

dystonia so dystonia is

20:59

a slow , usually sustained

21:01

, twisting or posturing movement . A

21:03

classic example would be something like torticollis

21:06

or rye neck that is involving the cervical

21:08

musculature . So dystonia

21:10

comes in a few different categories

21:12

as well . So there's focal , segmental

21:15

and generalized . So let's flip

21:17

that around and start with generalized . These

21:19

tend to be usually younger onset , often

21:22

secondary to inherited metabolic

21:24

or vascular causes . A classic

21:26

example would be someone with a DYT1

21:29

mutation . You can also sometimes

21:31

get what's called a levodopa responsive

21:33

dystonia . So very often you will trial

21:35

younger patients who are having generalized dystonia

21:38

on levodopa to see if they have any benefit

21:40

from it . We're going to skip over segmental

21:42

dystonias a little bit , as they're somewhere in the middle , and

21:45

we're going to talk about focal dystonias , as we tend

21:47

to see these a little bit more often in adults . So

21:49

again , the classic is cervical dystonia

21:51

or torticollis , and with

21:54

cervical dystonia you can sometimes get

21:56

a dystonic tremor . A dystonic

21:58

tremor is somewhat unique as it tends

22:00

to be worse when they look one direction and

22:02

then you can kind of , as they move through the range of

22:04

motion , find a null point . That is

22:06

a point where the tremor tends to

22:08

go away , and so this is very

22:11

suggestive that there is an imbalance in

22:13

the muscle activity in the sternocleidomastoids

22:15

or one of the other cervical muscles that might

22:17

be pulling one way or the other . So

22:19

if you can find that null point , that is also

22:21

very suggestive . A lot of these

22:23

people will also have a sensory trick , such as like

22:25

touching their chin or resting their ear

22:28

on their hand or something like that . That

22:30

may also help briefly

22:32

attenuate that feeling

22:34

that their neck is pulling in a direction . Another

22:36

common dystonia is writer's cramp , so

22:39

that flexion in the hand , that

22:41

cramping sensation after someone's been doing

22:43

a sustained activity for a period of time . And

22:46

dystonia can be very task-specific

22:48

and they are called task-specific dystonias

22:50

. Musicians' dystonia are

22:52

very common . So like , say , a trumpet player , they

22:54

might get their lips involved in embouchure

22:57

dystonia . Or a pianist

22:59

or a violin player . They may also

23:02

get very specific dystonias when they

23:04

are practicing this highly trained movement

23:06

. You can also see dystonia affecting

23:09

the muscles of the face , such as the eyelids or

23:11

even the vocal cords . For many of these focal

23:13

dystonias , the main treatment if you're not

23:15

able to change the movement or otherwise

23:17

train yourself into a different way of

23:19

doing the task is botulinum

23:21

toxin . So these injections

23:24

can be very effective in the appropriately

23:26

selected people and if done by someone

23:28

who knows what muscles specifically

23:30

they are targeting . Other medications

23:32

that you might try include various anticholinergic

23:34

agents benzodiazepines , baclofen

23:37

or sometimes even levodopa . Now

23:39

let's talk about choreoform disorders . So

23:42

a lot of people talk about chorea athetosis

23:44

. A lot of times they get smooshed together and we

23:46

call itoathetosis , as you get these

23:48

flowing types of movements that are

23:50

sometimes described as quote dance line

23:52

and there can be a lot of different causes

23:55

of these types of movements . The classic , prototypical

23:57

one is Huntington's disease . Huntington's

24:00

is associated with a CAG trinucleotide

24:02

repeat and there is a lot

24:04

of genetic counseling that goes into deciding

24:07

whether or not you should be testing for someone who is

24:09

pre-symptomatic . We're not going to go into that

24:11

really right now , but if there is a family

24:13

history , especially if it is happening at

24:15

younger generations , we get that anticipation

24:17

generation to generation . That is

24:20

a very useful tip , even if that

24:22

initial genetic testing comes back negative . But

24:24

it looks a lot like Huntington's . There are Huntington's-like

24:26

syndromes , so sometimes different kinds

24:28

of genetic panels may be appropriate . As

24:31

mentioned , there are many other causes of chorea

24:33

. Certain medications may trigger it different

24:35

endocrine abnormalities , certain metabolic

24:38

abnormalities such as severe hyperglycemia

24:40

that can sometimes trigger some movement abnormalities

24:42

. The classic example for infection

24:45

is streptococcal infection , so

24:48

Sydenham's chorea with rheumatic fever . You can also see it with different autoimmune

24:51

conditions such as lupus , and it is also

24:53

described in pregnancy . Next up is

24:55

myoclonus . These are brief , shock-like

24:58

, jerky types of movements . They do

25:00

not tend to be rhythmic like some of our other movements

25:02

and I would say in the hospital

25:04

these are one of the ones that we are seeing most often

25:06

. A lot of times when I'm consulted for a quote

25:09

tremor it is very often myoclonus

25:11

and or asterixis or negative

25:13

myoclonus . So there can

25:15

be physiologic myoclonus . So

25:17

, for instance , if you've ever fallen asleep in a chair

25:19

which I'm sure , as a medical student , none of us have ever

25:22

done and you jerk yourself awake

25:24

right before you fall out of your seat , that

25:32

is a hypnic jerk . Hiccups are another good example of myoclonic types of

25:34

movement . Very often we will see myoclonus and asterixis with toxic metabolic

25:37

encephalopathy . If you've ever read a neurology consult , I'm sure

25:39

you're familiar with the phrasing . So things to look

25:41

out for uremia , hypercarbia

25:43

, hyperammonemia all these things

25:45

could potentially cause these kinds of movements . Additionally

25:48

, certain kinds of medications are more common offenders

25:50

gabapentin , pregabalin

25:53

, opioids . All these kinds of things

25:55

can also , especially if someone is also having

25:57

a concurrent acute kidney injury , lead

25:59

to myoclonus and or asterixis

26:01

. You can also see epileptic myoclonus

26:04

, whether this is from a genetic generalized

26:06

epilepsy syndrome or from someone who has

26:08

had a severe brain injury , typically

26:10

an anoxic brain injury . So

26:13

very often we will see these kinds of movements and the

26:15

question is are these seizures , are these subcortical

26:17

, with cortical commonly understood to be epileptic

26:20

and subcortical not having associated

26:22

EEG abnormalities with the movements

26:24

themselves . Generally , when we have myoclonus

26:26

in the post-cardiac arrest period with a

26:28

suspected anoxic brain injury , it

26:30

is a poor prognostic indicator

26:33

. However , there are some exceptions

26:35

. One of these is Lance Adams syndrome

26:37

, and these are where people will wake up

26:39

and show general improvement in their cognitive function

26:41

. However , they will still have these myoclonic jerks

26:44

and you tend to treat them similarly to how you might

26:46

a generalized epilepsy with valproic

26:48

acid or clonazepam or other similar medications

26:50

like levotiracetam , topiramate or zanisamide

26:53

. Some sleep-related movement disorders to be aware of

26:55

A very common one is restless leg

26:57

syndrome . The first thing we usually want to do is

26:59

check to see if they have an unidentified iron

27:02

deficiency , as that can sometimes be causative

27:04

. You also want to see if they have any metabolic

27:06

abnormalities that may be contributing , so things

27:08

like uremia in patients with kidney

27:10

disease , if they have untreated sleep apnea , if

27:12

they're on certain medications such as SSRIs

27:15

, antipsychotics or different stimulants , and

27:17

it can also occur during pregnancy . Non-pharmacologic

27:20

treatment usually focuses on sleep hygiene

27:22

. There are different devices that you can wear on

27:24

your legs that kind of vibrate and can be distracting

27:26

, and different exercise protocols can

27:28

also be helpful . Pharmacologically

27:31

, we are usually starting with our gabapentinoids

27:33

. So gabapentin , pregabalin these

27:35

are generally preferred as first line over

27:38

our dopamine agonists like ropinerol , as

27:40

dopamine agonists may lead to augmentation , that

27:42

is to say symptoms occurring earlier in

27:44

the day . Right , we tend to think of it as happening during

27:46

sleep and at night , but we

27:49

might see it happening earlier and earlier

27:51

, intruding into the daytime . So that can obviously

27:53

be quite a problem . Another

27:55

movement disorder in sleep periodic limb movements

27:57

of sleep . This can cause sleep

28:00

fragmentation . The people usually have very frequent

28:02

movements of their legs during sleep . Very

28:04

often this is more disturbing to the person's

28:06

bed partner than it is to the person themselves

28:08

. So usually you're only treating when someone's

28:10

sleep quality is impaired . Lastly , rem

28:13

sleep behavior disorder . So this

28:15

is always tied in with these alpha-synuclein

28:18

diseases , that is to say Parkinson's , dementia

28:20

with Lewy bodies and even multiple system

28:22

atrophy . So this is characterized

28:24

clinically by this dream reenactment

28:26

. Right , people aren't paralyzed properly during

28:29

sleep , so they act out their dreams and

28:31

very often you'll hear a story of maybe the

28:33

bedsheets are all thrown about , the person's

28:35

fallen out of bed , maybe they've even struck their

28:37

spouse in their sleep . So all these kinds of

28:40

things can happen and this kind

28:42

of sleep disorder , this REM sleep behavior disorder

28:44

may precede the

28:46

diagnosis of something like Parkinson's

28:48

or DLB by years . So

28:50

it is always something to be cognizant of if

28:53

you make this diagnosis . Treatment-wise

28:55

, we usually start with something like melatonin . If

28:57

that's not effective , clonazepam will often be tried

29:00

. A few medication side effects

29:02

that can show up in the movement world . So

29:04

an acute dystonic reaction or acute dystonia

29:07

often involving the neck or

29:09

the face or eyes , and this is often as

29:11

an acute reaction to receiving an antipsychotic

29:13

, whether that is for something like nausea or

29:16

for actual behavioral issues . Typically

29:18

the treatment is with diphenhydramine . As

29:21

opposed to acute dystonia , we have tardive

29:23

dyskinesia , so tardive remember

29:25

Latin for tardy , late . So

29:28

this is a late complication of chronic

29:30

antipsychotic use typically . So

29:32

we see repetitive stereotype movements , usually

29:34

involving the face . You can also see

29:36

sometimes some restless movements , some choreoform

29:39

type movements , but you're really looking for a lot of

29:41

abnormal movements around kind of the mouth

29:43

, buccal muscles , lingual muscles , and

29:45

there is a treatment for this now valbenazine

29:48

. It's a monoamine depleter and this can be tried for the

29:50

tardive dyskinesia movements . Finally

29:52

, neuroleptic malignant syndrome , or

29:54

NMS . So as the name implies , this is typically

29:57

a side effect to a neuroleptic medication

29:59

and antipsychotic and it's characterized

30:02

by kind of rapid progressive rigidity , dystonic

30:04

movements , hyperthermia , rhabdomyolysis

30:07

and altered mental status . Typically

30:09

, your CK levels are going to be sky

30:11

high and you need to be treating supportively

30:13

for a lot of this . Medications that you may try

30:16

for treatment with NMS specifically include

30:18

bromocryptine and dantrolene . That

30:20

wraps up our episode on movement disorders for

30:22

our board review series . I hope this

30:24

was useful . I know it's a bit of a whirlwind . There's so

30:26

many things to cover but if you enjoyed this podcast

30:29

, please leave a five-star review on Apple , itunes

30:31

, spotify , wherever you're getting your podcasts these days

30:33

, and please do leave a comment on there as well

30:35

. It's always so good to hear from folks who are listening

30:37

and enjoying the show . Don't forget to share with a friend

30:39

and make sure to subscribe for future episodes . You

30:48

can find me on X , formerly Twitter at DrKentris D-R-K-E-N-T-R-I-S , and you can find the show at

30:50

neuro underscore podcast on X as well . Also , check out our website

30:52

online at theneurotransmitterscom

30:54

. As always , it has been a pleasure and

30:56

I hope to see you again next time .