Transcript Movement Disorders - MedicalTerminologyLower2014-15

Tic
Myoclonus
Chorea
Akathisia
Stereotypy
Dystonia
Stacy Peterson, D.C., D.A.C.R.B.
Associate Professor
Northwestern Health Sciences University
Tremor
By the end of this lecture series and after associated outside reading, the
student should be able to:


Define and describe the various abnormalities of movement.
Identify different neurologic conditions by the type of
movement abnormality that the disorder is characterized by,
particularly as it relates to the following conditions: multiple
sclerosis (MS), Parkinson’s disease and amyotrophic lateral
sclerosis (ALS)
Fibrillation
Myokymia
Fasciculation
Tremor
Ballismus
Akathisia
Myoclonus
Dystonia
Stereotypy
Asterixis
Athetosis
Tardive dyskinesia
Chorea
Tic

Spontaneous, independent contractions of individual
muscle fibers.

These contractions can not be observed or palpated.

Electromyographic (EMG) evidence can be found in
denervated muscle beginning 10-21 days after the muscle
has been deprived of its nerve supply.

Twitches observed (or palpated) in resting muscle that
result from spontaneous firing of one or more motor units.

Fasciculations do not produce joint movement (a brief
twitch of a muscle that produces joint movement is likely
chorea)

Association with:
* ALS (amyotrophic lateral sclerosis)
A patient with ALS rarely seeks medical attention
because of fasciculations alone.
Question:
What is the M/C chief complaint for a patient who
has ALS?

What is the M/C chief complaint for a patient who has
ALS?
Weakness

Association with:
* Diseases of the LMN
* Intrinsic diseases of the spinal cord
* Normal individuals

Video

Rippling movements observed under the skin, usually in
the face. Video

Association with:
* Neuromyotonia (peripheral nerve excitability
disorder that is autoimmune in nature)
* Multiple sclerosis
* Lesions of the brainstem
Enhanced Physiologic
Toxic
Parkinsonian
Essential
Midbrain
Dystonic


These tremors occur transiently in normal persons who are
under emotional stress, extremely fatigued, hyper- or hypoglycemic, hyperthyroid, cold or taking sympathomimetic
medications.
Fast – 14 cycles/sec

When EPT becomes clinically symptomatic with posture or
movement without provoking factors, it becomes
phenomenologically similar to Essential Tremor (ET) and may
be difficult to separate early in its course.
Press to
return to list
of tremors

These tremors occur in toxic-metabolic derangements,
sometimes in conjunction with encephalopathy.

Disorders such as uremia, thyrotoxicosis and alcohol or drug
withdrawal may be precipitants, as are certain antipsychotic
or neuroleptic drugs.

Lithium and Valproate are frequent causes of this tremor
type.

Video
Press to
return to list
of tremors

This tremor occurs with other manifestations of parkinsonism
and is characterized by tremor when the limb or other body
part is at rest.

The tremor usually diminishes with action.

PD rest tremors typically start unilaterally and distally as the
classic 3-6 Hz “pill-rolling” sinusoidal oscillations and progress
more proximally as they generalize to both sides, though
tremor may occasionally start anywhere (e.g., the lower lip).
Video

Tremor severity does not appear to be correlated with the
loss of dopamine. In fact, in some patients with PD, the
tremor does not improve or even worsens with levodopa.

It is generally believed that rest tremors are modulated
predominantly centrally within the corticobasal ganglia and
the corticocerebellar circuitry.
Press to
return to list
of tremors

Essential tremor (ET) asymmetrically involves the limbs, head
and voice in various combinations.

This tremor is typically absent in the resting position but
develops with sustained postures and action.

The hand tremor (usually most prominent) often becomes
exacerbated with action as the target is approached (terminal
tremor).

An essential voice tremor may be appreciated during normal
conversation or may be noted only when the patient is asked
to hold a musical note (“ahhhh…”).

A family history of this tremor is prominent in approximately
half the cases.

Alcohol consumption tends to
decrease this type of tremor.

Advancing age is the most obvious risk factor.

While traditionally considered a functional monosymptomatic
condition, an accumulation of both epidemiologic &
pathologic studies have argued that ET may be a
neurodegenerative disease with both an ↑ risk of nontremulous co-morbidities & structural pathologic changes in
the cerebellum or olivocerebellothalamic pathway.

Treatment: Primidone (anticonvulsant drug) or MRI-guided
focus ultrasound
Video
Press to
return to list
of tremors

This tremor is typical of that seen in multiple sclerosis,
Wilson’s disease or certain brain injuries.

It is characterized by wide tremor amplitude that is
prominent with sustained postures or actions.
Video

This tremor frequently involves the upper limbs, but may also
involve the trunk, head & lower limbs.

Midbrain tremors are caused by lesions of the
cerebellothalamic & nigrostriatal pathways, thus causing
tremors at rest, with posture & kinetically.
Press to
return to list
of tremors

DT is typically postural or task-related & is characterized by an
irregular or jerky rhythmic low-to-mid-frequency tremor
secondary to co-contraction of agonist & antagonist muscles.

The key identifiable feature of the DT is the Null point, a
position in which the tremor almost fully abates, and/or geste
antagoniste (a tactile or proprioceptive sensory trick that
reduces the abnormal posture) as well as its “jerky” quality.
Video

Treatment: Deep-Brain Stimulation Therapy (for ET, PD
& Dystonia)
Video
Press to
return to list
of tremors

Involuntary, lightening-like jerk of an area of the body.

Movements are * Simple, subtle or overt
Video for myoclonus-dystonia syndrome
Video for palatal myoclonus

Any level of the CNS…cortex, basal ganglia, brain stem, spinal
cord, etc.

Epilepsy, toxic / metabolic encephalopathy (narcotic drugs,
renal/hepatic failure), Alzheimer’s disease, Creutzfeldt-Jacob
disease, Lewy-Body disease

Sudden relaxation of muscles held against gravity

“Negative myoclonus”

Video of a “flapping tremor”

Causes: Toxic / metabolic encephalopathies, structural lesions
of the cerebral hemispheres

A rapid and flowing movement of one or more body parts that
is not stereotyped, & is manifested as a random event.

Looks semi-voluntary, but is not voluntary

Referred to as a “dance” (but not a very good dance) Video

Sydenham’s chorea (chorea caused by rheumatic fever)
Pregnancy (chorea gravidarum)
Lupus (lupus chorea)
Neuroleptic drugs (such as too much dopamine)

Females >>males
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Wild, flailing, sometimes violent movements.
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Subtype of chorea (“big” chorea)
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Fast, usually proximal movements; usually one side –
hemiballism

Video

Cause: stroke in subthalamic nucleus

Continuous, intermittent contraction of many muscles,
making body part turn, eventually in a fixed way

Spasmodic torticollis (focal dystonia)

Blepharospasm - spasmodic winking of the eyelids Video

Meige Syndrome - Forceful dystonic spasms of the face, in
particular when trying to speak or to eat
Henry Meige (1866-1940)

Writer’s cramp
Scott Adams, and his creation Dilbert

Cramping of Parkinson’s disease

Voice dystonias (3 types):
Abductor spasmodic dystonia
Adductor spasmodic dystonia
Mixed
Video

Rx: botulinum toxin; dopamine (this may be a dopamine
responsive dyskinesia)
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Moving dystonia

Slow, writhing dystonic posturing of the distal limbs,
especially fingers, hand & wrist.

Video
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Video
Question:
What do you think? Is this an accurate representation of
dystonia, voice dystonia and athetosis?

Subjective feeling, defined as a sense of inner restlessness, that
may be associated with adventitious movements.

The sensation of inner restlessness tends to elicit movements
that help relieve the inner tension.

Unlike other movement disorders, akathisia can be diagnosed
solely on the basis of the patient’s subjective symptoms in the
absence of any objective signs.

Parkinson’s disease,
neuroleptic drugs,
restless leg syndrome,
OCD;
dopamine responsive dyskinesia

Orobuccolingual movements (typically opening & closing
mouth movements with pursing of the lips are intermixed with
intermittent protrusion of the tongue)
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Anti-psychotic medications, such as those used to treat
Schizophrenia

Individuals with fetal alcohol syndrome, other developmental
disabilities, and other brain disorders are vulnerable to the
development of TDs, even after receiving only 1 dose of the
causative agent

Video

A repetitive or ritualistic movement, posture, or utterance

Stereotypies may be simple movements such as body rocking,
or complex, such as self-caressing, crossing and uncrossing of
legs, and marching in place.
Video

They are associated with mental retardation, autism spectrum
disorders, tardive dyskinesia, schizophrenia

Stereotypic movement disorder (SMD)
Common repetitive movements of SMD include head banging,
arm waving, hand shaking, rocking and rhythmic movements,
self-biting, self-hitting, skin-picking; other stereotypies are
thumb-sucking, nail biting, trichotillomania (hair pulling),
bruxism and abnormal running or skipping.

Simple or complex stereotyped motor behaviors that occur in
response to an irresistible urge.

Partial voluntary control

Simple: eye blinking, shoulder shrugs, grimacing, grunting &
sniffing
Complex: vocalizations, jumping, kicking, squatting or
compulsive touching; coprolalia is rare
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Obsessive-compulsive association is strong

Chronic tic disorder starting in youth that includes vocalizations

Organic disorder & does not simply reflect psychological factors
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Genetic link

Nicotinic agonists appear to relieve dyskinesias in some people
with Tourette’s Syndrome

Video
How well can you recognize the various movement disorders?
Let’s review!

A patient presents with the following tremor:
Video
Which of the following tremor types does this patient
demonstrate?
a.
b.
c.
d.
Dystonic
Holmes
Parkinsonian
Essential
I can see why you chose this answer:

Holmes tremors DO involve the head

The amplitude of Holmes tremors are wide, similar to this
presentation.
However, this is not the best answer.
Try again!
This answer cannot be supported based on the presentation
of the patient.
Try again!
I can see why you chose this answer:

Essential tremors DO involve the head.

Essential tremors are WORSE with sustained postures.

Essential tremors CAN involve phonation.
However, this is not the best answer based on the
presentation. Also, we do not have evidence of a family
history correlation or amelioration with alcohol
consumption.
Try again!
The following points support the answer chosen:

The patient has a moderate-high amplitude neck tremor.

The tremor has a “jerky” quality, predominantly in the “no-no”
direction.

There is a corresponding left laterocollis and retrocollis.
Click here
for Next
Question

A patient presents with the following tremor:
Video
Which of the following tremor types does this patient
demonstrate?
a.
b.
c.
d.
Dystonic
Holmes
Parkinsonian
Essential

Dystonic tremors are “jerky” and do not have the smooth
sinusoidal movement seen here.
Try again!

Rubral tremors are wide amplitude tremors.

If this were a rubral tremor, the tremor would be apparent
with sustained postures AND action.
Try again!

Essential tremors do not abate with movement.
Try again!
The following points support the answer chosen:


This tremor demonstrates latency between the resting tremor
(right hand) and (6 seconds later) the appearance of a postural
tremor.
The tremor is sinusoidal (pronation/supination) and low
frequency.
Click here
for Next
Question

Review the following video:
Video
Which of the following terms best describes these
movements?
a.
b.
c.
d.
Fibrillation
Fasciculation
Myokymia
Myoneuria

Fibrillations are only seen via electromyography.
Try again!

Fasciculations have a twitching versus rippling characteristic.

Fasciculations do not produce joint movement, as is seen with
the individual’s great toe.
Try again!

This is a nonsense word.
Try again!
The following point supports the answer chosen:

The most common location for myokymia is in the face (last
portion of the video). Facial myokymia is a fine rippling of
muscles on one side of the face and may reflect an underlying
tumor in the brainstem (typically a brainstem glioma), loss of
myelin in the brainstem (associated with multiple sclerosis) or
the recovery stage of Guillain–Barré syndrome.
Click here
for Next
Question

Which of the following is the most likely cause of ballismus?
a.
b.
c.
d.
Stroke in the subthalamic nucleus
Systemic lupus erythematosus
Cerebellar cortex structural lesion
Metabolic encephalopathy

SLE is known to cause chorea (lupus chorea).
Try again!

Cerebral infarcts can cause ballismus, affecting the
contralateral basal ganglia or subthalamic nucleus.
Try again!

Metabolic encephalopathies are known to cause numerous
movement disorders, most notably myoclonus and asterixis.
Try again!
The following point supports the answer chosen:



Ballismus is m/c observed in the setting of structural lesions of the
contralateral basal ganglia & subthalamic nucleus. Cerebral infarct is
the m/c cause, but tumors of the same region have also resulted in
ballismus.
Systemic derangements including fever & hyperglycemia have been
reported to cause or exacerbate hemiballismus in patients with
underlying lesions or a history of less severe movement disorders.
Medications including levodopa, phenytoin & oral contraceptives have
also been implicated.
Click here
for Next
Question

Which of the following terms describes a non-voluntary,
violent flinging movement of the arm?
a.
b.
c.
d.
Chorea
Ballismus
Athetosis
Epilepsy

See table below for comparisons:
Chorea
Ballismus
Athetosis
Rapid
Rapid
Slow
Involuntary
Involuntary
Involuntary
Non-stereotypical
Non-stereotypical
Non-stereotypical
Semi-purposeful or
non-purposeful
Non-purposeful
Non-purposeful
Dance-like
Violent flinging
movement
Writhing
> Distally
> Proximally
Propensity for
affecting upper
limbs

Epilepsy is a condition that can be a cause of movement
disorders (typically myoclonus), but is not a descriptor of the
abnormal movement .
Try again!

See table below for comparisons:
Chorea
Ballismus
Athetosis
Rapid
Rapid
Slow
Involuntary
Involuntary
Involuntary
Non-stereotypical
Non-stereotypical
Non-stereotypical
Semi-purposeful or
non-purposeful
Non-purposeful
Non-purposeful
Dance-like
Violent flinging
movement
Writhing
> Distally
> Proximally
Propensity for
affecting upper
limbs
Click here
for the last
Question!

Pacing back and forth in a room is an example of _________.
a.
b.
c.
d.
Akathisia
Athetosis
Tourette’s
Schizophrenia

Athetosis is a writhing (snake-like) type of movement.
Try again!
This is a “catnip” snake.

Tourette’s is a complex disorder involving many tics or
stereotypies…not just one. Also, it involves learning & mood
disorders as well as many other components.
Try again!

Schizophrenia can cause various movement disorders, the
most common being tardive dyskinesias.
Try again!

Examples of akathisia include pacing back and forth,
repetitively crossing legs, and foot tapping or leg shaking.
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Venn Diagram (Slide 1): modified from http://emedicine.medscape.com/article/1151826-overview#showall
Fibrillation pic: http://www.dartmouth.edu/~dons/electrodiagnosis/EDXfigures/Fig_1.htm
Muhammad Ali & Michael J Fox pic: http://static.ddmcdn.com/gif/parkinsons-disease-4.jpg
Asterixis flapping tremor pic: http://1.bp.blogspot.com/-7vYIsM8GP38/T0tINewxqYI/AAAAAAAAAEY/40tZa_duKVw/s1600/med9780199204854-graphic0152204001.jpeg
Chorea dance pic: http://1.bp.blogspot.com/e9VRgsp1Vf8/UWwAMQ1jznI/AAAAAAAABNA/zuPW42w7mug/s1600/vitus.jpg
Miley Cyrus mimicking TDs:
http://rack.3.mshcdn.com/media/ZgkyMDEzLzEwLzAyLzMzL01pbGV5Q3lydXNULmI0YTdkLmpwZwpwCXRodW1iCTEyMDB4OTYwMD4/c
bea3bcf/06e/Miley-Cyrus-Tongue.jpg
Antipsychotic meds effect on schizophrenia (TDs): http://writhesafely.wordpress.com/2007/01/15/you-should-have-seen-her-beforethe-abilify/
Pic of leg/foot (akathisia): http://thoughtbroadcast.com/tag/akathisia/
Stroke in subthalamic nucleus MRI pic: http://test.classconnection.s3.amazonaws.com/726/flashcards/196726/jpg/hemivallismus.jpg
Spasmodic torticollis pic: http://www.torticollis.org/spasmodic-torticollis-symptoms.html
Writer’s cramp 2 pics: http://ocdinformation.wordpress.com/2010/09/30/
Meige pic: http://www.infodystonia.com/tagged/meige
Diagram Tourette’s Syndrome: http://pertobello.com/2012/03/20/how-to-ask-for-money-without-sounding-like-jimmy-whales/
Rubral tremor PET pic: http://jnnp.bmj.com/content/72/suppl_1/i3/F6.large.jpg&
Nonsense pic: http://serendip.brynmawr.edu/exchange/node/6777
Smiley face in flower pic: http://www.google.com/imgres?imgurl=&imgrefurl=http%3A%2F%2Fwww.smiley-faces.org%2Fsmiley-face
wallpaper.php&h=0&w=0&sz=1&tbnid=LS0KRbYm4VxxEM&tbnh=194&tbnw=259&zoom=1&docid=wn6EjDqZsJ3N9M&ei=h8MKU_upH8
n8rAGL2IDICQ&ved=0CAsQsCUoAw
Epilepsy pic: http://www.end-epilepsy.org/api/Index.cfm/cms.page/i/2669/The-Epilepsies/
Cat & snake pic: http://pattihaskins.wordpress.com/2012/06/14/catnip-snake-love-2/
The End pic: Microsoft Word 2007 Clip Art
Question mark pics: Microsoft Word 2007 Clip Art
All sounds were obtained from www.soundjay.com, except for the clapping hands, which was obtained from Microsoft Office
PowerPoint 2007