April 8, 2024 • 30 mins
Welcome back to our IM Board Prep series, that is designed to help internal medicine residents ace the neurology section of their board exams!
This episode is on movement disorders, categorized into hyperkinetic and hypokinetic. In hypokinetic, we cover Parkinsonism vs Parkinson’s disease vs “Parkinson’s plus” syndromes. In hyperkinetic, we focus on tremors, dystonia, choreiform disorders, myoclonus, sleep-related disorders, tardive dyskinesia and neuroleptic malignant syndrome.
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Episode Transcript
Available transcripts are automatically generated. Complete accuracy is not guaranteed.
Dr. Michael Kentris (00:00):
Hello and welcome back to the
Neurotransmitters, your sourcefor everything regarding
clinical neurology.
Today we are continuing ourNeurology IM Board Reviews
series, part 6 today.
And today we are hittingmovement disorders.
As always, I want to remindeveryone that this is not an
exhaustive review on all ofthese entities.
This is an overview intended tohelp people preparing for their
(00:22):
internal medicine, in-serviceexams or board reviews.
With that being said, one of themain ways to differentiate
different kinds of movementdisorders is whether you have
too much or too little movement,that is to say, hyper versus
hypokinetic.
So let's start with some of ourhypokinetics, so people who
have some reduced movements.
(00:43):
The classic example of this isParkinsonism, and you'll notice
I say ism at the end therebecause there are things that
can have Parkinsonian featuresthat are not Parkinson's disease
.
So Parkinsonism we generallydefine as kind of slowing of
movements, rigidity, restingtremors, postural instability,
and since this is Parkinsonism,let's start off with Parkinson's
(01:05):
disease.
So the main acronym that peopleusually learn is TRAP T for
tremor at rest.
Now, this is a resting tremor.
Obviously, this tends to be alow amplitude, high frequency
tremor that we typically seewhen people are sitting arms
relaxed at their side, but youcan also see it when people
outstretch their arms in apostural kind of position and
(01:28):
this tremor may re-emerge aftera few moments.
The R is for rigidity, so a lotof people will have heard the
term lead pipe rigidity.
Now, I don't know about you,but I don't recall the last time
I tried to passively bend alead pipe with my own two hands.
I tried to passively bend alead pipe with my own two hands.
But, that being said, the ideais that rigidity is not velocity
(01:49):
dependent.
It doesn't matter how fast orhow slow you try and bend that
lead pipe.
You're going to get the sameamount of resistance.
And this is in contrast tospasticity, or what you'll
sometimes see in older books asclasp knife spasticity.
So if you've ever tried to opena pocket knife, many of them
(02:09):
will have a little resistance tothem and then they suddenly pop
open the rest of the way andstay open.
Similarly, spasticity is avelocity dependent resistance to
passive movement.
So the faster you move a limb,the more resistance you tend to
get.
Next is for akinesia, orbradykinesia.
This is a reduction not just inthe speed of a movement but
also in the amplitude of amovement.
Some tests you might do duringyour exam.
(02:30):
To evaluate this would befinger tapping having people
open or close their hands,tapping their toe and you do
want to try and do this to arhythm, so either tapping your
foot or clapping your hands, orgiving them some kind of
metronome to which you want themto adhere so you can tell if
they're slowing their movementsa little more subtly.
And lastly, we have P forpostural instability, and this
(02:51):
can manifest in severaldifferent ways.
One of the classic ones is gaitfreezing.
People will be walking and allof a sudden, their feet feel
like they're stuck to the ground.
They just can't lift them off,and this can result in falls.
Obviously, some situations thatmay trigger this are when
someone is passing through adoorway or when they're walking
across a floor and it goes fromcarpet to a tile or vice versa.
You will also see what's calledon-block turning, so they will
(03:13):
take many steps and they don'treally turn their shoulders in
advance of the rest of theirbody.
You may also see a stoopedposture.
Sometimes this is calledcamptochormia, and this is a
very stooped.
Sometimes this is calledcamptochormia, and this is a
very stooped.
Forward-flexed posture of thetrunk, and again, that acronym
is TRAP, tremor at rest rigidity, akinesia, slash bradykinesia
and postural instability.
(03:34):
Now there are other symptoms, ofcourse.
Some of those ones may includea masked face, so reduced facial
expression.
Hypophonia, so their voice willbecome very quiet.
Micrographia, so their voicewill become very quiet.
Micrographia, so their writingmay become very small.
Now with Parkinson's disease, alot of people end up fixated on
the motor symptoms becausethat's what's easy to see.
But there are many non-motorsymptoms as well, things that
(03:56):
can come along with Parkinson'sdisease as it progresses.
You can develop dementia.
There can be associateddepression or anxiety,
pseudobulbar affect or emotionalincontinence.
You can get sleep disorders, acommon one being REM sleep
behavior disorder, where peoplekind of act out their dreams.
We'll talk more about thatlater.
Autonomic dysfunction also verycommon.
Constipation, gut motilityissues, sialorrhea so drooling
(04:20):
can become an issue hyposmia,loss of smell, musculoskeletal
issues like I mentioned, thecamptochormia.
Sometimes that can lead to backpain, poor posture.
You can also develop dystonias,especially when we start
getting on higher doses oftreatments for Parkinson's
disease.
You can also get pain fromrigidity, visceral pain, again
related to that, constipation.
So many things that you need tobe aware of with these patients,
(04:42):
especially as the diseaseprogresses.
In particular I want toemphasize two big risks that you
want to assess for in thesepatients.
So one is depression screeningso making sure you're using a
validated tool to screen fordepression in these patients is
appropriate.
And the second are falls.
We want to make sure that we'reassessing gait on a regular
basis in these patients.
Now, one of the main tests thatmaybe not everyone does is
(05:04):
something called a pull test.
So you are going to standbehind the patient.
I recommend that you have yourback to a wall and you're going
to tell the patient what you'redoing.
You're going to place bothhands on their shoulders and
you're going to give them abrisk pull, and if they take
more than just a few stepsbackwards, that's considered a
positive pull test and thatsuggests that they are at
(05:25):
increased risk of falls.
Now I want to emphasize havingyour own back to the wall,
especially if you are a smallerperson and your patient is on
the larger side, becausesometimes with Parkinson's, the
patient may fall right over likea tree going down, and so if
they are larger than you, youwill be going down with them.
So having the back to your wallmeans that you just get a
little bump against your back,as opposed to both of you
(05:46):
hitting the ground.
Now there are obviously othercomponents to the gait
evaluation, but this is one thatnot everyone is probably
thinking about if you are mostlyworking in the internal
medicine or primary care clinics.
So let's talk a little bit abouttreatment, particularly
pharmacologic treatment forParkinson's disease.
So most of our treatmentsrevolve around dopamine therapy,
(06:07):
right?
The idea is that we have lossof dopamine producing neurons
and we need to replace thatdopamine.
So the main medication iscarbidopa, levodopa, or brand
name, is Sinemet.
So there are differentformulations of carbidopa,
levodopa there's immediaterelease, extended release,
continued release, enteral gel.
So our main one that we'regoing to be using most of the
(06:30):
time is this immediate releaseformulation and that's usually
going to be dosed at least threetimes daily and it is in a
combination tablet, right?
Carbidopa helps preventperipheral metabolism of the
levodopa.
We found that levodopa by itselftends to cause lots of emesis,
(06:53):
orthostatic hypotension, hencethe name Sinemet, sin without
emet, emesis vomiting so withoutvomiting is kind of the idea
there, one of those clever brandnames that you come across from
time to time.
This is my own personalexperience and not an official
recommendation.
I usually start with the 25-100combination tablets.
I'll usually start those threetimes daily and I tend to
increase in frequency more sothan in the single dose.
Or, as one of my instructorstold me years ago, you are doing
(07:15):
fence posts, not telephoneposts, in terms of your dosing.
So you want lots of small,frequent doses rather than a few
big high doses.
Now, some of these extendedreleases out there can help
alleviate the need for frequentmedication dosing, as sometimes
the regimens can become quitecumbersome in terms of how often
someone's supposed to be takingtheir medication.
(07:35):
One of the theories that it isimportant to keep in mind is the
narrowing therapeutic windowover time with Parkinson's
disease.
So when we say the therapeuticwindow is narrowing as the
disease advances, what we meanis that normally you take a dose
of carbidopa, levodopa, youhave a period of effect right
the on time, and this is wheremaybe rigidity is reduced or
(07:57):
tremors are reduced, what haveyou and Then it wears off, the
symptoms come back.
So as we go on, we start seeingmore off time and we also start
seeing periods of too muchdopamine activity or peak dose
dyskinesias or other peak dosetype side effects, and we'll
talk about some of themanagement strategies for that.
So that is why people tend tolean towards these frequent
(08:19):
small doses rather than fewerhigh doses, because that window
over time starts to narrow andso you end up overshooting and
undershooting more often withless frequent dosing.
At least, that is one of thetheories of treatment.
Other medications that we'llsometimes use include dopamine
agonists, so Pramopexol,rapinrol.
There is often a debate goingback and forth about which
(08:41):
should you start first carbidopa, levodopa or dopamine agonists.
Some of the thought was that ifyou start carbidopa levodopa
too early, you are more likelyto lead to dyskinesias earlier.
At the time that I'm recordingthis, in April 2024, the trend
is more towards startingcarbidopa levodopa over dopamine
agonists, partially related tosome of the side effects, and
that there isn't really thatmuch difference in dyskinesia
(09:03):
development.
So something to keep in mindwith all of our dopaminergic
therapies is that as youincrease dopamine, the risk for
certain kinds of side effectsdoes increase, in particular
impulsivity, behavior changes,hallucinations, things of that
nature.
With carbidopa levodopa we alsosee some risk for peripheral
edema and orthostatichypotension creep in as the dose
(09:24):
goes up.
Also, another class ofmedications that are sometimes
used are thecatechol-O-methyltransferase
inhibitors, or COMT, and theseare Entocopone and Tolcopone
Entocopone the brand name isCompton, right?
Another one of those cleverbrand names there, and this
helps to prolong the levodopaeffect, right?
The idea is that it's kind ofblocking that peripheral
(09:45):
metabolism again.
Another class are the monoamineoxidase type B inhibitors, or
MAOI-Bs, and selagiline andresagiline are the ones here.
Now I mentioned peak dosedyskinesias, that is to say,
dyskinesias are excessivemovements that tend to occur as
the dopamine overshoots thetherapeutic window, and one
(10:06):
medication that is used arethese glutamate NMDA antagonists
or amantadine.
So amantadine can sometimeshelp with these side effects as
well.
As we mentioned earlier,hallucinations, particularly
visual in nature, can developwith Parkinson's disease.
Usually later, if it's early,we start thinking about one of
our Parkinson's plusquote-unquote syndromes and
we'll talk about those later.
(10:26):
So as far as treatment of thesehallucinations, quetiapine is
very commonly used.
We usually are talking aboutlow doses, right?
If we're using dopamineblocking therapy in someone whom
we're also giving dopaminergictherapies.
So we don't want to really beself-defeating and giving
medications that counteract whatwe're trying to do in the first
place.
So that is why we tend to uselower doses of quetiapine.
(10:50):
You'll also see a lot ofmovement disorder specialists
using clozapine, which is alsovery well tolerated.
As far as the Parkinson's sideof things, but you do have to be
very careful in terms of theother side effects with
clozapine, particularly ourwhite blood cell count.
There is a relatively newmedication on the market for
Parkinson's psychosisspecifically, and that is
(11:10):
pimivanserin.
Now, this is a differentmechanism.
This is a selective serotonin5-HT2B receptor inverse agonist.
That is a lot to remember, butthis medication is not supposed
to have the same dopamineblockade issues that other
things for Parkinson's relatedhallucinations or psychosis may
have.
So it's definitely something tokeep in mind, although cost may
(11:32):
be a limiting issue in somesituations.
As we mentioned earlier, youcan get some autonomic issues.
In particular, orthostatichypotension another fall
contributor can be very commonin Parkinson's disease.
So one of the medications thatwe'll sometimes use is a
norepinephrine precursor andit's droxidopa, and so that can
sometimes be used in people whoare having issues with blood
(11:52):
pressure management, as well asmore traditional blood pressure
elevating medications.
It's also important to rememberthe non-pharmacologic options as
far as compression stockings, ahigh salt-containing diet and
things of that nature, and Ialso want to mention deep brain
stimulation.
So this is primarily forpatients who are having severe
motor fluctuations or they'rehaving refractory tremor.
(12:12):
You also tend to avoid DBS inpatients who are already
starting to develop some signsof dementia, as they don't tend
to have as good of outcomes asthose who don't when they're
undergoing this kind of surgery.
The targets for DBS there arevarious ones, but the classic
ones are the subthalamic nucleusand the globus pallidus interna
.
Now we did spend a fair amountof time just talking about the
(12:33):
pharmacologic management ofParkinson's disease, but just as
important, you need to makesure that these patients are
undergoing exercise regularly.
There are certain kinds ofphysical therapy protocols the
big and loud protocol, where wefocus a lot on the hypophonia,
on exaggerated movements, thekinds of activities that people
with Parkinson's tend to havemore difficulty with.
(12:53):
So it is important to take amultidisciplinary approach to
these patients and focus on thebasic things as well.
And again, don't forget aboutthose non-motor symptoms of
constipation, sialorrhea orhypophonia, as these things can
be quite disturbing to people'squality of life.
Let's change gears slightly andtalk about the Parkinson's Plus
syndromes.
These are often calledParkinson's Plus as they have
(13:15):
Parkinsonism plus an additionalfinding on exam or by history
that makes you think this isjust a little different than it
should be.
Now there are a lot ofdifferent things that can go
into the diagnosis of thesedifferent entities.
But again, right, this is amore superficial look and we're
going to focus on just a couplesalient points on each one of
these that will hopefullyidentify them in the question
(13:37):
stem or maybe even in real lifefor you.
So first of all, we haveprogressive supernuclear palsy,
or PSP.
Now the two things that you'llsee sometimes in a question stem
that should make you think PSPif someone has had a relatively
short duration of Parkinsonismand they are falling a lot, so
early falls is a big one.
Now, another thing they mayalso mention is that there is
(13:59):
impairment of vertical gaze.
Right, they supernuclear, can'tmove their eyes that well, so
early falls, vertical eyemovements, think PSP.
Next up, corticobasaldegeneration or corticobasal
syndrome.
So this tends to be moreunilateral and you tend to see
on imaging you might haveatrophy of one hemisphere or one
(14:19):
basal ganglia and sometimesyou'll see that they have the
alien hand syndrome.
So one hand is kind of doingits own thing and it feels like
the patient can't control thathand.
So it tends to be veryunilateral as compared to a lot
of these other syndromes,although in the early stages of
Parkinson's you may have a moreunilateral resting tremor, but
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this tends to be much moremarked.
You may also see something likeassociated neglect or sensory
disturbances on the side that isaffected as well in
corticobasal degeneration,multiple system atrophy or MSA.
This is characterizedclassically by a very prominent
orthostatic hypotension.
Lots of dysautonomia is theclassic form.
So MSA does come in threedifferent subtypes MSA-A for
(15:03):
autonomic, msa-c for cerebellarand MSA-P for Parkinsonian.
So the autonomic type obviouslyhas more autonomics involved.
They all do to an extent.
The MSA-C might have some moreataxia and the MSA-P has a lot
of Parkinsonism.
But in a question vignette Iwould expect to hear a lot about
(15:23):
very early orthostatichypotension, erectile
dysfunction, urinary retention,a lot of these autonomic issues.
Lastly, we have dementia withLewy bodies or DLB.
Now we did mention DLB a bit inour dementia podcast earlier in
this series, so do look back atthat if you want more about
Lewy body.
But the main things that youwant to think about with Lewy
(15:45):
body versus Parkinson's diseaseis that the cognitive changes
and the visual hallucinationswill happen earlier with DLB as
opposed to Parkinson's disease.
So typically the rule of thumbis if you have cognitive changes
before motor think Lewy body,if you have motor before
cognitive think Parkinson'sdisease.
This is, of course, anoversimplification and it is a
(16:06):
spectrum, but on the test that'sthe best way to think about it.
So hopefully by now you'regetting the picture that just
because someone may haveParkinsonism does not mean they
have Parkinson's disease.
So we've mentioned severaldifferent disorders that can
have Parkinsonian features tothem, but there are others, so a
couple again that we mentionedin our dementia podcast normal
pressure hydrocephalus maydevelop some Parkinsonian
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features and vascular dementiamay also develop some
Parkinsonian features.
So, again, always important toget the MRIs and make sure that
you don't see signs that aresuggestive of NPH or old strokes
that might point you in adifferent direction.
You can still try dopaminetherapy in these patients who
may have Parkinsonism, asopposed to traditional
Parkinson's disease, and you mayget some effective results in
(16:50):
some people.
However, you should not expectall people to respond as if they
had Parkinson's disease.
A last category of ParkinsonismI want to mention is
drug-induced Parkinsonism.
So we talk about ourextrapyramidal symptoms, right?
So all the things we've beentalking about, the resting
tremors that we tend to see fromdrug-induced Parkinsonism tend
to be a little more symmetricbilaterally and they tend to
(17:11):
occur obviously fromantipsychotics most often,
whether those are typical oratypical antipsychotics.
Now, something you may notthink about is metoclopramide.
So we have a lot of folks innursing homes who are on Reglan
or metoclopramide for gut issues, and so it is important to
remember this is very often acause of drug-induced
Parkinsonism.
(17:32):
A test that can sometimes beuseful if the clinical exam
isn't pointing in a specificdirection is a DAT scan.
This can help differentiate atremor due to basal ganglia
dysfunction versus drug-induced,although certain medications
can confound it, particularlysome antidepressants.
So you do have to look at themed list and come off of those
medications for a couple weeksif possible.
(17:53):
So we talked about hypokinetic,let's talk about hyperkinetic.
So this comes in many differentflavors, so let's go through
them.
First up, we have tremors.
These are regular oscillationsaround a joint, typically, so
they tend to be rhythmic, andthere are many different tremors
.
We're going to focus on some ofthe ones that are likely to be
a little more high value.
Essential tremor is probablyone of our more common tremors.
(18:15):
It tends to involve the upperextremities.
You can see this is worse withaction and posture, but it can
also affect the head and voice.
You can sometimes get what'scalled a yes-yes or no-no tremor
, when the head is shakingeither like they're saying yes
or no, and so this can be veryannoying to some people also.
So there are variousmedications that we will try
(18:35):
Propranolol, primidone those arekind of our classic agents.
To pyramate is also tried.
Second line agents includethings like clonazepam,
gabapentin, other beta blockers.
You can sometimes try botulinuminjections for neck tremors or
voice predominant tremors,although you do want those done
by someone who really knows whatthey're doing, as there's a lot
of important structures there.
(18:56):
Deep brain stimulation can alsobe an option for these patients
as well.
The target is different than inParkinson's disease.
Here it is the ventralintermediate nucleus of the
thalamus.
Non-pharmacologically speaking,there are various weighted
tools or different modificationsthat can be done to silverware
or various other things thatpeople will often have
difficulty with.
Trying to bring a full glass tosomeone's mouth or a spoonful
(19:21):
of soup can be very challengingfor some of these people, so
there are different options thatan occupational therapist can
provide to these people.
Another common tremor is theenhanced physiologic tremor.
Now everyone has a little bitof a shake to their hands if
they're stressed or tired, andthis tends to be worse with
stress or anxiety or other kindsof medications Caffeine.
(19:41):
So the treatment in thesepeople is usually finding if
there is any recent triggers orlife changes that may be
contributing to worsening ofthis tremor.
So mostly you're counselingabout sleep, stress and caffeine
intake.
Another very common categoryare drug-induced tremors.
This can be from a variety ofdifferent classes beta agonists,
so folks using their albuterolinhalers, people on lithium,
(20:01):
valproic acid, amiodarone,glucocorticoids, different
antidepressants likeamitriptyline or fluoxetine.
So there are many medicationsthat can cause a drug-induced
tremor.
So if someone has a new tremoryou want to find out have they
been on any new medications thatcorrespond temporally with the
onset of that tremor?
There are other tremors, suchas rural or cerebellar, but
(20:23):
those tend to be less common sowe won't spend too much time on
them today.
Moving on to dystonia sodystonia is a slow, usually
sustained, twisting or posturingmovement.
A classic example would besomething like torticollis or
rye neck that is involving thecervical musculature.
So dystonia comes in a fewdifferent categories as well.
(20:43):
So there's focal, segmental andgeneralized.
So let's flip that around andstart with generalized.
These tend to be usuallyyounger onset, often secondary
to inherited metabolic orvascular causes.
A classic example would besomeone with a DYT1 mutation.
You can also sometimes getwhat's called a levodopa
responsive dystonia.
(21:04):
So very often you will trialyounger patients who are having
generalized dystonia on levodopato see if they have any benefit
from it.
We're going to skip oversegmental dystonias a little bit
, as they're somewhere in themiddle, and we're going to talk
about focal dystonias, as wetend to see these a little bit
more often in adults.
So again, the classic iscervical dystonia or torticollis
, and with cervical dystonia youcan sometimes get a dystonic
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tremor.
A dystonic tremor is somewhatunique as it tends to be worse
when they look one direction andthen you can kind of, as they
move through the range of motion, find a null point.
That is a point where thetremor tends to go away, and so
this is very suggestive thatthere is an imbalance in the
muscle activity in thesternocleidomastoids or one of
the other cervical muscles thatmight be pulling one way or the
(21:49):
other.
So if you can find that nullpoint, that is also very
suggestive.
A lot of these people will alsohave a sensory trick, such as
like touching their chin orresting their ear on their hand
or something like that.
That may also help brieflyattenuate that feeling that
their neck is pulling in adirection.
Another common dystonia iswriter's cramp, so that flexion
(22:10):
in the hand, that crampingsensation after someone's been
doing a sustained activity for aperiod of time.
And dystonia can be verytask-specific and they are
called task-specific dystonias.
Musicians' dystonia are verycommon.
So like, say, a trumpet player,they might get their lips
involved in embouchure dystonia.
Or a pianist or a violin player.
(22:31):
They may also get very specificdystonias when they are
practicing this highly trainedmovement.
You can also see dystoniaaffecting the muscles of the
face, such as the eyelids oreven the vocal cords.
For many of these focaldystonias, the main treatment if
you're not able to change themovement or otherwise train
yourself into a different way ofdoing the task is botulinum
(22:52):
toxin.
So these injections can be veryeffective in the appropriately
selected people and if done bysomeone who knows what muscles
specifically they are targeting.
Other medications that youmight try include various
anticholinergic agentsbenzodiazepines, baclofen or
sometimes even levodopa.
Now let's talk about choreoformdisorders.
So a lot of people talk aboutchorea athetosis.
(23:14):
A lot of times they getsmooshed together and we call
itoathetosis, as you get theseflowing types of movements that
are sometimes described as quotedance line and there can be a
lot of different causes of thesetypes of movements.
The classic, prototypical oneis Huntington's disease.
Huntington's is associated witha CAG trinucleotide repeat and
there is a lot of geneticcounseling that goes into
(23:37):
deciding whether or not youshould be testing for someone
who is pre-symptomatic.
We're not going to go into thatreally right now, but if there
is a family history, especiallyif it is happening at younger
generations, we get thatanticipation generation to
generation.
That is a very useful tip, evenif that initial genetic testing
comes back negative.
But it looks a lot likeHuntington's.
There are Huntington's-likesyndromes, so sometimes
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different kinds of geneticpanels may be appropriate.
As mentioned, there are manyother causes of chorea.
Certain medications may triggerit different endocrine
abnormalities, certain metabolicabnormalities such as severe
hyperglycemia that can sometimestrigger some movement
abnormalities.
The classic example forinfection is streptococcal
infection, so Sydenham's choreawith rheumatic fever.
(24:19):
You can also see it withdifferent autoimmune conditions
such as lupus, and it is alsodescribed in pregnancy.
Next up is myoclonus.
These are brief, shock-like,jerky types of movements.
They do not tend to be rhythmiclike some of our other
movements and I would say in thehospital these are one of the
ones that we are seeing mostoften.
A lot of times when I'mconsulted for a quote tremor it
(24:40):
is very often myoclonus and orasterixis or negative myoclonus.
So there can be physiologicmyoclonus.
So, for instance, if you'veever fallen asleep in a chair
which I'm sure, as a medicalstudent, none of us have ever
done and you jerk yourself awakeright before you fall out of
your seat, that is a hypnic jerk.
(25:03):
Hiccups are another goodexample of myoclonic types of
movement.
Very often we will see myoclonusand asterixis with toxic
metabolic encephalopathy.
If you've ever read a neurologyconsult, I'm sure you're
familiar with the phrasing.
So things to look out foruremia, hypercarbia,
hyperammonemia all these thingscould potentially cause these
kinds of movements.
Additionally, certain kinds ofmedications are more common
offenders gabapentin, pregabalin, opioids.
(25:24):
All these kinds of things canalso, especially if someone is
also having a concurrent acutekidney injury, lead to myoclonus
and or asterixis.
You can also see epilepticmyoclonus, whether this is from
a genetic generalized epilepsysyndrome or from someone who has
had a severe brain injury,typically an anoxic brain injury
.
So very often we will see thesekinds of movements and the
(25:45):
question is are these seizures,are these subcortical, with
cortical commonly understood tobe epileptic and subcortical not
having associated EEGabnormalities with the movements
themselves.
Generally, when we havemyoclonus in the post-cardiac
arrest period with a suspectedanoxic brain injury, it is a
poor prognostic indicator.
However, there are someexceptions.
One of these is Lance Adamssyndrome, and these are where
(26:08):
people will wake up and showgeneral improvement in their
cognitive function.
However, they will still havethese myoclonic jerks and you
tend to treat them similarly tohow you might a generalized
epilepsy with valproic acid orclonazepam or other similar
medications like levotiracetam,topiramate or zanisamide.
Some sleep-related movementdisorders to be aware of A very
common one is restless legsyndrome.
The first thing we usually wantto do is check to see if they
(26:30):
have an unidentified irondeficiency, as that can
sometimes be causative.
You also want to see if theyhave any metabolic abnormalities
that may be contributing, sothings like uremia in patients
with kidney disease, if theyhave untreated sleep apnea, if
they're on certain medicationssuch as SSRIs, antipsychotics or
different stimulants, and itcan also occur during pregnancy.
Non-pharmacologic treatmentusually focuses on sleep hygiene
(26:52):
.
There are different devicesthat you can wear on your legs
that kind of vibrate and can bedistracting, and different
exercise protocols can also behelpful.
Pharmacologically, we areusually starting with our
gabapentinoids.
So gabapentin, pregabalin theseare generally preferred as
first line over our dopamineagonists like ropinerol, as
dopamine agonists may lead toaugmentation, that is to say
(27:13):
symptoms occurring earlier inthe day.
Right, we tend to think of itas happening during sleep and at
night, but we might see ithappening earlier and earlier,
intruding into the daytime.
So that can obviously be quitea problem.
Another movement disorder insleep periodic limb movements of
sleep.
This can cause sleepfragmentation.
The people usually have veryfrequent movements of their legs
(27:33):
during sleep.
Very often this is moredisturbing to the person's bed
partner than it is to the personthemselves.
So usually you're only treatingwhen someone's sleep quality is
impaired.
Lastly, rem sleep behaviordisorder.
So this is always tied in withthese alpha-synuclein diseases,
that is to say Parkinson's,dementia with Lewy bodies and
even multiple system atrophy.
So this is characterizedclinically by this dream
(27:56):
reenactment.
Right, people aren't paralyzedproperly during sleep, so they
act out their dreams and veryoften you'll hear a story of
maybe the bedsheets are allthrown about, the person's
fallen out of bed, maybe they'veeven struck their spouse in
their sleep.
So all these kinds of thingscan happen and this kind of
sleep disorder, this REM sleepbehavior disorder may precede
(28:16):
the diagnosis of something likeParkinson's or DLB by years.
So it is always something to becognizant of if you make this
diagnosis.
Treatment-wise, we usuallystart with something like
melatonin.
If that's not effective,clonazepam will often be tried.
A few medication side effectsthat can show up in the movement
world.
So an acute dystonic reactionor acute dystonia often
(28:38):
involving the neck or the faceor eyes, and this is often as an
acute reaction to receiving anantipsychotic, whether that is
for something like nausea or foractual behavioral issues.
Typically the treatment is withdiphenhydramine.
As opposed to acute dystonia,we have tardive dyskinesia, so
tardive remember Latin for tardy, late.
So this is a late complicationof chronic antipsychotic use
(29:01):
typically.
So we see repetitive stereotypemovements, usually involving
the face.
You can also see sometimes somerestless movements, some
choreoform type movements, butyou're really looking for a lot
of abnormal movements aroundkind of the mouth, buccal
muscles, lingual muscles, andthere is a treatment for this
now valbenazine.
It's a monoamine depleter andthis can be tried for the
tardive dyskinesia movements.
(29:22):
Finally, neuroleptic malignantsyndrome, or NMS.
So as the name implies, this istypically a side effect to a
neuroleptic medication andantipsychotic and it's
characterized by kind of rapidprogressive rigidity, dystonic
movements, hyperthermia,rhabdomyolysis and altered
mental status.
Typically, your CK levels aregoing to be sky high and you
(29:42):
need to be treating supportivelyfor a lot of this.
Medications that you may tryfor treatment with NMS
specifically includebromocryptine and dantrolene.
That wraps up our episode onmovement disorders for our board
review series.
I hope this was useful.
I know it's a bit of awhirlwind.
There's so many things to coverbut if you enjoyed this podcast
, please leave a five-starreview on Apple, itunes, spotify
, wherever you're getting yourpodcasts these days, and please
(30:04):
do leave a comment on there aswell.
It's always so good to hearfrom folks who are listening and
enjoying the show.
Don't forget to share with afriend and make sure to
subscribe for future episodes.
You can find me on X, formerlyTwitter at DrKentris
D-R-K-E-N-T-R-I-S, and you canfind the show at neuro
underscore podcast on X as well.
Also, check out our websiteonline at
theneurotransmitterscom.
(30:24):
As always, it has been apleasure and I hope to see you
again next time.
Hello and welcome back to the
Neurotransmitters, your sourcefor everything regarding
clinical neurology.
Today we are continuing ourNeurology IM Board Reviews
series, part 6 today.
And today we are hittingmovement disorders.
As always, I want to remindeveryone that this is not an
exhaustive review on all ofthese entities.
This is an overview intended tohelp people preparing for their
(00:22):
internal medicine, in-serviceexams or board reviews.
With that being said, one of themain ways to differentiate
different kinds of movementdisorders is whether you have
too much or too little movement,that is to say, hyper versus
hypokinetic.
So let's start with some of ourhypokinetics, so people who
have some reduced movements.
(00:43):
The classic example of this isParkinsonism, and you'll notice
I say ism at the end therebecause there are things that
can have Parkinsonian featuresthat are not Parkinson's disease
.
So Parkinsonism we generallydefine as kind of slowing of
movements, rigidity, restingtremors, postural instability,
and since this is Parkinsonism,let's start off with Parkinson's
(01:05):
disease.
So the main acronym that peopleusually learn is TRAP T for
tremor at rest.
Now, this is a resting tremor.
Obviously, this tends to be alow amplitude, high frequency
tremor that we typically seewhen people are sitting arms
relaxed at their side, but youcan also see it when people
outstretch their arms in apostural kind of position and
(01:28):
this tremor may re-emerge aftera few moments.
The R is for rigidity, so a lotof people will have heard the
term lead pipe rigidity.
Now, I don't know about you,but I don't recall the last time
I tried to passively bend alead pipe with my own two hands.
I tried to passively bend alead pipe with my own two hands.
But, that being said, the ideais that rigidity is not velocity
(01:49):
dependent.
It doesn't matter how fast orhow slow you try and bend that
lead pipe.
You're going to get the sameamount of resistance.
And this is in contrast tospasticity, or what you'll
sometimes see in older books asclasp knife spasticity.
So if you've ever tried to opena pocket knife, many of them
(02:09):
will have a little resistance tothem and then they suddenly pop
open the rest of the way andstay open.
Similarly, spasticity is avelocity dependent resistance to
passive movement.
So the faster you move a limb,the more resistance you tend to
get.
Next is for akinesia, orbradykinesia.
This is a reduction not just inthe speed of a movement but
also in the amplitude of amovement.
Some tests you might do duringyour exam.
(02:30):
To evaluate this would befinger tapping having people
open or close their hands,tapping their toe and you do
want to try and do this to arhythm, so either tapping your
foot or clapping your hands, orgiving them some kind of
metronome to which you want themto adhere so you can tell if
they're slowing their movementsa little more subtly.
And lastly, we have P forpostural instability, and this
(02:51):
can manifest in severaldifferent ways.
One of the classic ones is gaitfreezing.
People will be walking and allof a sudden, their feet feel
like they're stuck to the ground.
They just can't lift them off,and this can result in falls.
Obviously, some situations thatmay trigger this are when
someone is passing through adoorway or when they're walking
across a floor and it goes fromcarpet to a tile or vice versa.
You will also see what's calledon-block turning, so they will
(03:13):
take many steps and they don'treally turn their shoulders in
advance of the rest of theirbody.
You may also see a stoopedposture.
Sometimes this is calledcamptochormia, and this is a
very stooped.
Sometimes this is calledcamptochormia, and this is a
very stooped.
Forward-flexed posture of thetrunk, and again, that acronym
is TRAP, tremor at rest rigidity, akinesia, slash bradykinesia
and postural instability.
(03:34):
Now there are other symptoms, ofcourse.
Some of those ones may includea masked face, so reduced facial
expression.
Hypophonia, so their voice willbecome very quiet.
Micrographia, so their voicewill become very quiet.
Micrographia, so their writingmay become very small.
Now with Parkinson's disease, alot of people end up fixated on
the motor symptoms becausethat's what's easy to see.
But there are many non-motorsymptoms as well, things that
(03:56):
can come along with Parkinson'sdisease as it progresses.
You can develop dementia.
There can be associateddepression or anxiety,
pseudobulbar affect or emotionalincontinence.
You can get sleep disorders, acommon one being REM sleep
behavior disorder, where peoplekind of act out their dreams.
We'll talk more about thatlater.
Autonomic dysfunction also verycommon.
Constipation, gut motilityissues, sialorrhea so drooling
(04:20):
can become an issue hyposmia,loss of smell, musculoskeletal
issues like I mentioned, thecamptochormia.
Sometimes that can lead to backpain, poor posture.
You can also develop dystonias,especially when we start
getting on higher doses oftreatments for Parkinson's
disease.
You can also get pain fromrigidity, visceral pain, again
related to that, constipation.
So many things that you need tobe aware of with these patients,
(04:42):
especially as the diseaseprogresses.
In particular I want toemphasize two big risks that you
want to assess for in thesepatients.
So one is depression screeningso making sure you're using a
validated tool to screen fordepression in these patients is
appropriate.
And the second are falls.
We want to make sure that we'reassessing gait on a regular
basis in these patients.
Now, one of the main tests thatmaybe not everyone does is
(05:04):
something called a pull test.
So you are going to standbehind the patient.
I recommend that you have yourback to a wall and you're going
to tell the patient what you'redoing.
You're going to place bothhands on their shoulders and
you're going to give them abrisk pull, and if they take
more than just a few stepsbackwards, that's considered a
positive pull test and thatsuggests that they are at
(05:25):
increased risk of falls.
Now I want to emphasize havingyour own back to the wall,
especially if you are a smallerperson and your patient is on
the larger side, becausesometimes with Parkinson's, the
patient may fall right over likea tree going down, and so if
they are larger than you, youwill be going down with them.
So having the back to your wallmeans that you just get a
little bump against your back,as opposed to both of you
(05:46):
hitting the ground.
Now there are obviously othercomponents to the gait
evaluation, but this is one thatnot everyone is probably
thinking about if you are mostlyworking in the internal
medicine or primary care clinics.
So let's talk a little bit abouttreatment, particularly
pharmacologic treatment forParkinson's disease.
So most of our treatmentsrevolve around dopamine therapy,
(06:07):
right?
The idea is that we have lossof dopamine producing neurons
and we need to replace thatdopamine.
So the main medication iscarbidopa, levodopa, or brand
name, is Sinemet.
So there are differentformulations of carbidopa,
levodopa there's immediaterelease, extended release,
continued release, enteral gel.
So our main one that we'regoing to be using most of the
(06:30):
time is this immediate releaseformulation and that's usually
going to be dosed at least threetimes daily and it is in a
combination tablet, right?
Carbidopa helps preventperipheral metabolism of the
levodopa.
We found that levodopa by itselftends to cause lots of emesis,
(06:53):
orthostatic hypotension, hencethe name Sinemet, sin without
emet, emesis vomiting so withoutvomiting is kind of the idea
there, one of those clever brandnames that you come across from
time to time.
This is my own personalexperience and not an official
recommendation.
I usually start with the 25-100combination tablets.
I'll usually start those threetimes daily and I tend to
increase in frequency more sothan in the single dose.
Or, as one of my instructorstold me years ago, you are doing
(07:15):
fence posts, not telephoneposts, in terms of your dosing.
So you want lots of small,frequent doses rather than a few
big high doses.
Now, some of these extendedreleases out there can help
alleviate the need for frequentmedication dosing, as sometimes
the regimens can become quitecumbersome in terms of how often
someone's supposed to be takingtheir medication.
(07:35):
One of the theories that it isimportant to keep in mind is the
narrowing therapeutic windowover time with Parkinson's
disease.
So when we say the therapeuticwindow is narrowing as the
disease advances, what we meanis that normally you take a dose
of carbidopa, levodopa, youhave a period of effect right
the on time, and this is wheremaybe rigidity is reduced or
(07:57):
tremors are reduced, what haveyou and Then it wears off, the
symptoms come back.
So as we go on, we start seeingmore off time and we also start
seeing periods of too muchdopamine activity or peak dose
dyskinesias or other peak dosetype side effects, and we'll
talk about some of themanagement strategies for that.
So that is why people tend tolean towards these frequent
(08:19):
small doses rather than fewerhigh doses, because that window
over time starts to narrow andso you end up overshooting and
undershooting more often withless frequent dosing.
At least, that is one of thetheories of treatment.
Other medications that we'llsometimes use include dopamine
agonists, so Pramopexol,rapinrol.
There is often a debate goingback and forth about which
(08:41):
should you start first carbidopa, levodopa or dopamine agonists.
Some of the thought was that ifyou start carbidopa levodopa
too early, you are more likelyto lead to dyskinesias earlier.
At the time that I'm recordingthis, in April 2024, the trend
is more towards startingcarbidopa levodopa over dopamine
agonists, partially related tosome of the side effects, and
that there isn't really thatmuch difference in dyskinesia
(09:03):
development.
So something to keep in mindwith all of our dopaminergic
therapies is that as youincrease dopamine, the risk for
certain kinds of side effectsdoes increase, in particular
impulsivity, behavior changes,hallucinations, things of that
nature.
With carbidopa levodopa we alsosee some risk for peripheral
edema and orthostatichypotension creep in as the dose
(09:24):
goes up.
Also, another class ofmedications that are sometimes
used are thecatechol-O-methyltransferase
inhibitors, or COMT, and theseare Entocopone and Tolcopone
Entocopone the brand name isCompton, right?
Another one of those cleverbrand names there, and this
helps to prolong the levodopaeffect, right?
The idea is that it's kind ofblocking that peripheral
(09:45):
metabolism again.
Another class are the monoamineoxidase type B inhibitors, or
MAOI-Bs, and selagiline andresagiline are the ones here.
Now I mentioned peak dosedyskinesias, that is to say,
dyskinesias are excessivemovements that tend to occur as
the dopamine overshoots thetherapeutic window, and one
(10:06):
medication that is used arethese glutamate NMDA antagonists
or amantadine.
So amantadine can sometimeshelp with these side effects as
well.
As we mentioned earlier,hallucinations, particularly
visual in nature, can developwith Parkinson's disease.
Usually later, if it's early,we start thinking about one of
our Parkinson's plusquote-unquote syndromes and
we'll talk about those later.
(10:26):
So as far as treatment of thesehallucinations, quetiapine is
very commonly used.
We usually are talking aboutlow doses, right?
If we're using dopamineblocking therapy in someone whom
we're also giving dopaminergictherapies.
So we don't want to really beself-defeating and giving
medications that counteract whatwe're trying to do in the first
place.
So that is why we tend to uselower doses of quetiapine.
(10:50):
You'll also see a lot ofmovement disorder specialists
using clozapine, which is alsovery well tolerated.
As far as the Parkinson's sideof things, but you do have to be
very careful in terms of theother side effects with
clozapine, particularly ourwhite blood cell count.
There is a relatively newmedication on the market for
Parkinson's psychosisspecifically, and that is
(11:10):
pimivanserin.
Now, this is a differentmechanism.
This is a selective serotonin5-HT2B receptor inverse agonist.
That is a lot to remember, butthis medication is not supposed
to have the same dopamineblockade issues that other
things for Parkinson's relatedhallucinations or psychosis may
have.
So it's definitely something tokeep in mind, although cost may
(11:32):
be a limiting issue in somesituations.
As we mentioned earlier, youcan get some autonomic issues.
In particular, orthostatichypotension another fall
contributor can be very commonin Parkinson's disease.
So one of the medications thatwe'll sometimes use is a
norepinephrine precursor andit's droxidopa, and so that can
sometimes be used in people whoare having issues with blood
(11:52):
pressure management, as well asmore traditional blood pressure
elevating medications.
It's also important to rememberthe non-pharmacologic options as
far as compression stockings, ahigh salt-containing diet and
things of that nature, and Ialso want to mention deep brain
stimulation.
So this is primarily forpatients who are having severe
motor fluctuations or they'rehaving refractory tremor.
(12:12):
You also tend to avoid DBS inpatients who are already
starting to develop some signsof dementia, as they don't tend
to have as good of outcomes asthose who don't when they're
undergoing this kind of surgery.
The targets for DBS there arevarious ones, but the classic
ones are the subthalamic nucleusand the globus pallidus interna
.
Now we did spend a fair amountof time just talking about the
(12:33):
pharmacologic management ofParkinson's disease, but just as
important, you need to makesure that these patients are
undergoing exercise regularly.
There are certain kinds ofphysical therapy protocols the
big and loud protocol, where wefocus a lot on the hypophonia,
on exaggerated movements, thekinds of activities that people
with Parkinson's tend to havemore difficulty with.
(12:53):
So it is important to take amultidisciplinary approach to
these patients and focus on thebasic things as well.
And again, don't forget aboutthose non-motor symptoms of
constipation, sialorrhea orhypophonia, as these things can
be quite disturbing to people'squality of life.
Let's change gears slightly andtalk about the Parkinson's Plus
syndromes.
These are often calledParkinson's Plus as they have
(13:15):
Parkinsonism plus an additionalfinding on exam or by history
that makes you think this isjust a little different than it
should be.
Now there are a lot ofdifferent things that can go
into the diagnosis of thesedifferent entities.
But again, right, this is amore superficial look and we're
going to focus on just a couplesalient points on each one of
these that will hopefullyidentify them in the question
(13:37):
stem or maybe even in real lifefor you.
So first of all, we haveprogressive supernuclear palsy,
or PSP.
Now the two things that you'llsee sometimes in a question stem
that should make you think PSPif someone has had a relatively
short duration of Parkinsonismand they are falling a lot, so
early falls is a big one.
Now, another thing they mayalso mention is that there is
(13:59):
impairment of vertical gaze.
Right, they supernuclear, can'tmove their eyes that well, so
early falls, vertical eyemovements, think PSP.
Next up, corticobasaldegeneration or corticobasal
syndrome.
So this tends to be moreunilateral and you tend to see
on imaging you might haveatrophy of one hemisphere or one
(14:19):
basal ganglia and sometimesyou'll see that they have the
alien hand syndrome.
So one hand is kind of doingits own thing and it feels like
the patient can't control thathand.
So it tends to be veryunilateral as compared to a lot
of these other syndromes,although in the early stages of
Parkinson's you may have a moreunilateral resting tremor, but
(14:39):
this tends to be much moremarked.
You may also see something likeassociated neglect or sensory
disturbances on the side that isaffected as well in
corticobasal degeneration,multiple system atrophy or MSA.
This is characterizedclassically by a very prominent
orthostatic hypotension.
Lots of dysautonomia is theclassic form.
So MSA does come in threedifferent subtypes MSA-A for
(15:03):
autonomic, msa-c for cerebellarand MSA-P for Parkinsonian.
So the autonomic type obviouslyhas more autonomics involved.
They all do to an extent.
The MSA-C might have some moreataxia and the MSA-P has a lot
of Parkinsonism.
But in a question vignette Iwould expect to hear a lot about
(15:23):
very early orthostatichypotension, erectile
dysfunction, urinary retention,a lot of these autonomic issues.
Lastly, we have dementia withLewy bodies or DLB.
Now we did mention DLB a bit inour dementia podcast earlier in
this series, so do look back atthat if you want more about
Lewy body.
But the main things that youwant to think about with Lewy
(15:45):
body versus Parkinson's diseaseis that the cognitive changes
and the visual hallucinationswill happen earlier with DLB as
opposed to Parkinson's disease.
So typically the rule of thumbis if you have cognitive changes
before motor think Lewy body,if you have motor before
cognitive think Parkinson'sdisease.
This is, of course, anoversimplification and it is a
(16:06):
spectrum, but on the test that'sthe best way to think about it.
So hopefully by now you'regetting the picture that just
because someone may haveParkinsonism does not mean they
have Parkinson's disease.
So we've mentioned severaldifferent disorders that can
have Parkinsonian features tothem, but there are others, so a
couple again that we mentionedin our dementia podcast normal
pressure hydrocephalus maydevelop some Parkinsonian
(16:27):
features and vascular dementiamay also develop some
Parkinsonian features.
So, again, always important toget the MRIs and make sure that
you don't see signs that aresuggestive of NPH or old strokes
that might point you in adifferent direction.
You can still try dopaminetherapy in these patients who
may have Parkinsonism, asopposed to traditional
Parkinson's disease, and you mayget some effective results in
(16:50):
some people.
However, you should not expectall people to respond as if they
had Parkinson's disease.
A last category of ParkinsonismI want to mention is
drug-induced Parkinsonism.
So we talk about ourextrapyramidal symptoms, right?
So all the things we've beentalking about, the resting
tremors that we tend to see fromdrug-induced Parkinsonism tend
to be a little more symmetricbilaterally and they tend to
(17:11):
occur obviously fromantipsychotics most often,
whether those are typical oratypical antipsychotics.
Now, something you may notthink about is metoclopramide.
So we have a lot of folks innursing homes who are on Reglan
or metoclopramide for gut issues, and so it is important to
remember this is very often acause of drug-induced
Parkinsonism.
(17:32):
A test that can sometimes beuseful if the clinical exam
isn't pointing in a specificdirection is a DAT scan.
This can help differentiate atremor due to basal ganglia
dysfunction versus drug-induced,although certain medications
can confound it, particularlysome antidepressants.
So you do have to look at themed list and come off of those
medications for a couple weeksif possible.
(17:53):
So we talked about hypokinetic,let's talk about hyperkinetic.
So this comes in many differentflavors, so let's go through
them.
First up, we have tremors.
These are regular oscillationsaround a joint, typically, so
they tend to be rhythmic, andthere are many different tremors
.
We're going to focus on some ofthe ones that are likely to be
a little more high value.
Essential tremor is probablyone of our more common tremors.
(18:15):
It tends to involve the upperextremities.
You can see this is worse withaction and posture, but it can
also affect the head and voice.
You can sometimes get what'scalled a yes-yes or no-no tremor
, when the head is shakingeither like they're saying yes
or no, and so this can be veryannoying to some people also.
So there are variousmedications that we will try
(18:35):
Propranolol, primidone those arekind of our classic agents.
To pyramate is also tried.
Second line agents includethings like clonazepam,
gabapentin, other beta blockers.
You can sometimes try botulinuminjections for neck tremors or
voice predominant tremors,although you do want those done
by someone who really knows whatthey're doing, as there's a lot
of important structures there.
(18:56):
Deep brain stimulation can alsobe an option for these patients
as well.
The target is different than inParkinson's disease.
Here it is the ventralintermediate nucleus of the
thalamus.
Non-pharmacologically speaking,there are various weighted
tools or different modificationsthat can be done to silverware
or various other things thatpeople will often have
difficulty with.
Trying to bring a full glass tosomeone's mouth or a spoonful
(19:21):
of soup can be very challengingfor some of these people, so
there are different options thatan occupational therapist can
provide to these people.
Another common tremor is theenhanced physiologic tremor.
Now everyone has a little bitof a shake to their hands if
they're stressed or tired, andthis tends to be worse with
stress or anxiety or other kindsof medications Caffeine.
(19:41):
So the treatment in thesepeople is usually finding if
there is any recent triggers orlife changes that may be
contributing to worsening ofthis tremor.
So mostly you're counselingabout sleep, stress and caffeine
intake.
Another very common categoryare drug-induced tremors.
This can be from a variety ofdifferent classes beta agonists,
so folks using their albuterolinhalers, people on lithium,
(20:01):
valproic acid, amiodarone,glucocorticoids, different
antidepressants likeamitriptyline or fluoxetine.
So there are many medicationsthat can cause a drug-induced
tremor.
So if someone has a new tremoryou want to find out have they
been on any new medications thatcorrespond temporally with the
onset of that tremor?
There are other tremors, suchas rural or cerebellar, but
(20:23):
those tend to be less common sowe won't spend too much time on
them today.
Moving on to dystonia sodystonia is a slow, usually
sustained, twisting or posturingmovement.
A classic example would besomething like torticollis or
rye neck that is involving thecervical musculature.
So dystonia comes in a fewdifferent categories as well.
(20:43):
So there's focal, segmental andgeneralized.
So let's flip that around andstart with generalized.
These tend to be usuallyyounger onset, often secondary
to inherited metabolic orvascular causes.
A classic example would besomeone with a DYT1 mutation.
You can also sometimes getwhat's called a levodopa
responsive dystonia.
(21:04):
So very often you will trialyounger patients who are having
generalized dystonia on levodopato see if they have any benefit
from it.
We're going to skip oversegmental dystonias a little bit
, as they're somewhere in themiddle, and we're going to talk
about focal dystonias, as wetend to see these a little bit
more often in adults.
So again, the classic iscervical dystonia or torticollis
, and with cervical dystonia youcan sometimes get a dystonic
(21:27):
tremor.
A dystonic tremor is somewhatunique as it tends to be worse
when they look one direction andthen you can kind of, as they
move through the range of motion, find a null point.
That is a point where thetremor tends to go away, and so
this is very suggestive thatthere is an imbalance in the
muscle activity in thesternocleidomastoids or one of
the other cervical muscles thatmight be pulling one way or the
(21:49):
other.
So if you can find that nullpoint, that is also very
suggestive.
A lot of these people will alsohave a sensory trick, such as
like touching their chin orresting their ear on their hand
or something like that.
That may also help brieflyattenuate that feeling that
their neck is pulling in adirection.
Another common dystonia iswriter's cramp, so that flexion
(22:10):
in the hand, that crampingsensation after someone's been
doing a sustained activity for aperiod of time.
And dystonia can be verytask-specific and they are
called task-specific dystonias.
Musicians' dystonia are verycommon.
So like, say, a trumpet player,they might get their lips
involved in embouchure dystonia.
Or a pianist or a violin player.
(22:31):
They may also get very specificdystonias when they are
practicing this highly trainedmovement.
You can also see dystoniaaffecting the muscles of the
face, such as the eyelids oreven the vocal cords.
For many of these focaldystonias, the main treatment if
you're not able to change themovement or otherwise train
yourself into a different way ofdoing the task is botulinum
(22:52):
toxin.
So these injections can be veryeffective in the appropriately
selected people and if done bysomeone who knows what muscles
specifically they are targeting.
Other medications that youmight try include various
anticholinergic agentsbenzodiazepines, baclofen or
sometimes even levodopa.
Now let's talk about choreoformdisorders.
So a lot of people talk aboutchorea athetosis.
(23:14):
A lot of times they getsmooshed together and we call
itoathetosis, as you get theseflowing types of movements that
are sometimes described as quotedance line and there can be a
lot of different causes of thesetypes of movements.
The classic, prototypical oneis Huntington's disease.
Huntington's is associated witha CAG trinucleotide repeat and
there is a lot of geneticcounseling that goes into
(23:37):
deciding whether or not youshould be testing for someone
who is pre-symptomatic.
We're not going to go into thatreally right now, but if there
is a family history, especiallyif it is happening at younger
generations, we get thatanticipation generation to
generation.
That is a very useful tip, evenif that initial genetic testing
comes back negative.
But it looks a lot likeHuntington's.
There are Huntington's-likesyndromes, so sometimes
(23:58):
different kinds of geneticpanels may be appropriate.
As mentioned, there are manyother causes of chorea.
Certain medications may triggerit different endocrine
abnormalities, certain metabolicabnormalities such as severe
hyperglycemia that can sometimestrigger some movement
abnormalities.
The classic example forinfection is streptococcal
infection, so Sydenham's choreawith rheumatic fever.
(24:19):
You can also see it withdifferent autoimmune conditions
such as lupus, and it is alsodescribed in pregnancy.
Next up is myoclonus.
These are brief, shock-like,jerky types of movements.
They do not tend to be rhythmiclike some of our other
movements and I would say in thehospital these are one of the
ones that we are seeing mostoften.
A lot of times when I'mconsulted for a quote tremor it
(24:40):
is very often myoclonus and orasterixis or negative myoclonus.
So there can be physiologicmyoclonus.
So, for instance, if you'veever fallen asleep in a chair
which I'm sure, as a medicalstudent, none of us have ever
done and you jerk yourself awakeright before you fall out of
your seat, that is a hypnic jerk.
(25:03):
Hiccups are another goodexample of myoclonic types of
movement.
Very often we will see myoclonusand asterixis with toxic
metabolic encephalopathy.
If you've ever read a neurologyconsult, I'm sure you're
familiar with the phrasing.
So things to look out foruremia, hypercarbia,
hyperammonemia all these thingscould potentially cause these
kinds of movements.
Additionally, certain kinds ofmedications are more common
offenders gabapentin, pregabalin, opioids.
(25:24):
All these kinds of things canalso, especially if someone is
also having a concurrent acutekidney injury, lead to myoclonus
and or asterixis.
You can also see epilepticmyoclonus, whether this is from
a genetic generalized epilepsysyndrome or from someone who has
had a severe brain injury,typically an anoxic brain injury
.
So very often we will see thesekinds of movements and the
(25:45):
question is are these seizures,are these subcortical, with
cortical commonly understood tobe epileptic and subcortical not
having associated EEGabnormalities with the movements
themselves.
Generally, when we havemyoclonus in the post-cardiac
arrest period with a suspectedanoxic brain injury, it is a
poor prognostic indicator.
However, there are someexceptions.
One of these is Lance Adamssyndrome, and these are where
(26:08):
people will wake up and showgeneral improvement in their
cognitive function.
However, they will still havethese myoclonic jerks and you
tend to treat them similarly tohow you might a generalized
epilepsy with valproic acid orclonazepam or other similar
medications like levotiracetam,topiramate or zanisamide.
Some sleep-related movementdisorders to be aware of A very
common one is restless legsyndrome.
The first thing we usually wantto do is check to see if they
(26:30):
have an unidentified irondeficiency, as that can
sometimes be causative.
You also want to see if theyhave any metabolic abnormalities
that may be contributing, sothings like uremia in patients
with kidney disease, if theyhave untreated sleep apnea, if
they're on certain medicationssuch as SSRIs, antipsychotics or
different stimulants, and itcan also occur during pregnancy.
Non-pharmacologic treatmentusually focuses on sleep hygiene
(26:52):
.
There are different devicesthat you can wear on your legs
that kind of vibrate and can bedistracting, and different
exercise protocols can also behelpful.
Pharmacologically, we areusually starting with our
gabapentinoids.
So gabapentin, pregabalin theseare generally preferred as
first line over our dopamineagonists like ropinerol, as
dopamine agonists may lead toaugmentation, that is to say
(27:13):
symptoms occurring earlier inthe day.
Right, we tend to think of itas happening during sleep and at
night, but we might see ithappening earlier and earlier,
intruding into the daytime.
So that can obviously be quitea problem.
Another movement disorder insleep periodic limb movements of
sleep.
This can cause sleepfragmentation.
The people usually have veryfrequent movements of their legs
(27:33):
during sleep.
Very often this is moredisturbing to the person's bed
partner than it is to the personthemselves.
So usually you're only treatingwhen someone's sleep quality is
impaired.
Lastly, rem sleep behaviordisorder.
So this is always tied in withthese alpha-synuclein diseases,
that is to say Parkinson's,dementia with Lewy bodies and
even multiple system atrophy.
So this is characterizedclinically by this dream
(27:56):
reenactment.
Right, people aren't paralyzedproperly during sleep, so they
act out their dreams and veryoften you'll hear a story of
maybe the bedsheets are allthrown about, the person's
fallen out of bed, maybe they'veeven struck their spouse in
their sleep.
So all these kinds of thingscan happen and this kind of
sleep disorder, this REM sleepbehavior disorder may precede
(28:16):
the diagnosis of something likeParkinson's or DLB by years.
So it is always something to becognizant of if you make this
diagnosis.
Treatment-wise, we usuallystart with something like
melatonin.
If that's not effective,clonazepam will often be tried.
A few medication side effectsthat can show up in the movement
world.
So an acute dystonic reactionor acute dystonia often
(28:38):
involving the neck or the faceor eyes, and this is often as an
acute reaction to receiving anantipsychotic, whether that is
for something like nausea or foractual behavioral issues.
Typically the treatment is withdiphenhydramine.
As opposed to acute dystonia,we have tardive dyskinesia, so
tardive remember Latin for tardy, late.
So this is a late complicationof chronic antipsychotic use
(29:01):
typically.
So we see repetitive stereotypemovements, usually involving
the face.
You can also see sometimes somerestless movements, some
choreoform type movements, butyou're really looking for a lot
of abnormal movements aroundkind of the mouth, buccal
muscles, lingual muscles, andthere is a treatment for this
now valbenazine.
It's a monoamine depleter andthis can be tried for the
tardive dyskinesia movements.
(29:22):
Finally, neuroleptic malignantsyndrome, or NMS.
So as the name implies, this istypically a side effect to a
neuroleptic medication andantipsychotic and it's
characterized by kind of rapidprogressive rigidity, dystonic
movements, hyperthermia,rhabdomyolysis and altered
mental status.
Typically, your CK levels aregoing to be sky high and you
(29:42):
need to be treating supportivelyfor a lot of this.
Medications that you may tryfor treatment with NMS
specifically includebromocryptine and dantrolene.
That wraps up our episode onmovement disorders for our board
review series.
I hope this was useful.
I know it's a bit of awhirlwind.
There's so many things to coverbut if you enjoyed this podcast
, please leave a five-starreview on Apple, itunes, spotify
, wherever you're getting yourpodcasts these days, and please
(30:04):
do leave a comment on there aswell.
It's always so good to hearfrom folks who are listening and
enjoying the show.
Don't forget to share with afriend and make sure to
subscribe for future episodes.
You can find me on X, formerlyTwitter at DrKentris
D-R-K-E-N-T-R-I-S, and you canfind the show at neuro
underscore podcast on X as well.
Also, check out our websiteonline at
theneurotransmitterscom.
(30:24):
As always, it has been apleasure and I hope to see you
again next time.
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