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Involuntarymovements 130726040949 Phpapp02
Involuntarymovements 130726040949 Phpapp02
Involuntarymovements 130726040949 Phpapp02
MOVEMENTS
Medical Unit-1
GMC,Surat
But the expression of a well-made man
appears not only in his face,
It is in his limbs and joints also, it is
curiously in the joints of his hips and
wrists.
It is in his walk, the carriage of his neck,
the flex of his waist, and knees...
To see him pass conveys as much as the
best poem,perhaps more.
—Walt Whitman (1819–1892)
Basics
The basal ganglia are group of nuclei located subcortically
which is part of a neuronal network organized in parallel
circuits. The “motor circuit” is most relevant to the
pathophysiology of movement.
Abnormal increment or reduction in the inhibitory output
activity of basal ganglia give rise, respectively, to poverty and
slowness of movement - Hypokinesias (i.e., Parkinson's
disease) or dyskinesias.
It includes the corpus striatum (caudate nucleus and the
lenticular nucleus, which includes the putamen and the globus
pallidus) and other subcortical allied nuclei such as the
subthalamic nucleus (STN), substantia nigra (SN) [consisting of
the pars compacta (SNc) and pars reticulata (SNr)], and, more
recently, the pedunculopontine tegmental nucleus (PPTg).
Currently, the putamen and caudate
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are together referred to as
the striatum.
Coronal view of the brain, showing the main basal ganglia nuclei.
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Obeso J A et al. Physiology 2002;17:51-55
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Outline
1. Overview of approach to the clinical
problem
2. Definition of movement types
3. Elements of history
4. Physical examination
5. Detailed discussion about each movement
type
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Broadly . . .
Hyperkinetic Movements Hypokinetic Movements
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Or More Specifically . . .
Hyperkinetic Movements Hypokinetic Movements
• Tremor (ET-Most common) • Parkinsonism (2nd most
• Chorea/Athetosis
• Dystonia common)
• Ballism • Apraxia
• Myoclonus
• Hesitant gaits
• Tics
• Ataxia • Hypothyroid slowness
• Myokymia • Rigidity
• Myorrhythmia
• Restless Legs
• Hyperkplexia (startle response)
• Akathesia
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Physiological Involuntary movements
Physiological synkinesia
e.g. Bell’s phenomenon, arm swing etc.
Myoclonic jerk
Hypnogogic myoclonus
Benign fasciculation
without other signs of LMN leison
Physiological tremor
low amplitude, high frequency
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Bradykinesia/Akinesia
• Decreased movement
• Common to parkinsonism
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Types of Parkinsonism
• Parkinson’s Disease (most common
encountered by neurologists)
• Drug-induced Parkinsonism (probably the
most common overall)
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Features Suggestive of Atypical PD
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Asynergia/Ataxia
• Asynergia refers to decomposition of
movement due to breakdown of normal
coordinated execution of a voluntary
movement
• One of the cardinal features of cerebellar
disease
• Frequently accompanied by:
– Dysmetria (misjudging of distance)
– Hypometria
– Hypermetria
• The ataxic gait is typified by unsteadiness with
a wide base, body sway and inability to
tandem walk
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Tremor
• Tremor consists of alternating contractions of
agonist and antagonist muscles in an
oscillating, rhythmic manner.
• Distinction between rest-pill rolling, postural,
action or with intention or task specific
• Froment maneuver : Voluntary head
movement enhances the parkinsonian rest
tremer.
• Holmes’ or Rubral tremor : due to leison of
red nucleus, rest + postural + kinetic tremer
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Essential Tremor
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Treatment
•First line- beta-blockers (propranolol 30-240 mg/d), or
anticonvulsants primadone (start with 12.5mg mg/d,increase
upto 125-250mg tid). Second line-benzodiazepines and
methazolamide (Carbonic Anhydrase Inhibitor). Finally if all
else fails- Botox or Surgical therapies such as electronic
stimulation of the VIM nucleus of the thalamus or ablation of
this structure (thalamotomy), can be effective treatments for
those refractory to drug therapy.
Can be confused with:
•Physiologic tremor -physical exertion, hyperthyroidism, acute
hypoglycemia, and other physical and metabolic stressors.
•Induced tremor -Stimulant drugs (including caffeine and
amphetamines), antidepressants, and agonist drugs (used to
treat asthma).
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•Chorea, athetosis, ballism &
dystonia :
Non-rhythmic involuntary
movements may be
combinations of fragments of
purposeful movements &
abnormal postures.
•All due to imbalance of activity
in the complex basal ganglia
circuits.
• Sometimes also known as
“ extrapyramidal disorders”
•Primarily conditions related to
excessive dopaminergic activity
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in the basal ganglia.
Ballismus, Chorea, Athotosis and
Dystonia
These should NOT be thought of as separate entities
amenable to specific definition but rather as a SPECTRUM
of movements that blend into one-another
WHY?
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Because……..
They often co-exist
Even neurologists may often not be able to agree as to
how a particular movement should be classified!
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The spectrum
Myoclonus Ballismus Tic Chorea Athetosis Dystonia
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Ballism
More dramatic ballistic movements of of the arms & legs on one side
of the body (unilateral) and therefore use term “ HEMIBALLISMUS”.
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Stereotypy
• Purposeless voluntary movements carried
out in a uniform fashion at the expense of
other activity
• Refers to coordinated movements that
repeat continually and identically
• Resemble motor tics but there is no driving
urge
• Often repeat themselves in a uniform,
repetitive fashion for long periods of time
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Tics
• An abrupt, brief recurrent jerky non-
rhythmic stereotyped seemingly
purposeless movement (motor tic) or
sound (vocal tic) that is temporarily
suppressible by will power
• Vary in severity over time
• Usually preceded by uncomfortable feeling or
sensory urge that is relieved by carrying out
the movement
• “unvoluntary”
• May be simple or complex
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Tourette’s Syndrome (tics)
•Neuropsychiatric condition characterized by the childhood onset of multiple motor
and vocal tics-Repetitive semi-purposeful movements as blinking, winking, grinning
or screwing up of the eyes. Coprolalia (the inadvertent utterance of obscenities),
echolalia (involuntary repetition of other's phrases), palilalia (involuntary repetition
of one's own utterances) and echopraxia (involuntary mimicking of the action of
others). Worsen under stress!
•Although the pathogenic basis is not understood, Tics are believed to result from
dysfunction in the thalamus, basal ganglia, and frontal cortex of the brain, involving
abnormal activity of the brain chemical, or neurotransmitter, dopamine therefore Tics
can be treated with dopamine receptor blocking or dopamine depleting drugs such as
neuroleptics.
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Tourette’s Syndrome Criteria
• Both multiple motor tics and one or more phonic tics must be
present at some time during the illness, although not
necessarily concurrently
• Tics must occur many times a day, nearly every day, or
intermittently throughout a period of more than one year
• Anatomical location, number, frequency, type, complexity, or
severity of tics must change over time
• Onset of tics before the age of 21 years (the DSM-IV criteria
require onset of tics before age 18)
• Involuntary movements and noises must not be explained by
another medical condition
• Motor tics, phonic tics, or both must be witnessed by a reliable
examiner at some point during the illness or be recorded by
videotape or cinematography
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Causes of Tics
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Chorea(latin choreus, dance)
• Describes a class of involuntary, irregular,
purposeless, nonrhymic, abrupt, rapid, unsustained
movements that seem to flow from one body part to
another
• Characteristically unpredictable in timing, direction
and distribution
• Can be partially suppressed into semi-purposeful
voluntary movements
• e.g. an involuntary movement of arm towards face
may be adapted to look-like an attempt to look at
watch
• Prototypical example is Huntington’s disease
• Treated with dopamine blocking agents,
carbamazepine, valproic acid. Usually self limiting.
• Sex hormons for chorea gravidarum
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Athetosis
Athetosis is a slow continuous stream of slow, sinuous, writhing movements,
typically of the hands and feet.
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Classification of Dystonia
• Primary Vs Secondary
• Focal – Most common
– When a single body part is affected
• Segmental
– Involvement of 2 or more contiguous regions of the
body are affected
• Multifocal
– Involves 2 or more regions, not conforming to
segmental or generalized dystonia
• Generalized
– Movements of one or both of the legs, trunk and
some other part of the body
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Common types of Dystonia
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Examples of focal dystonias
Blepharospasm means the involuntary contraction of the eyelids,
leading to uncontrollable blinking and closure of the eyelids. Affects
women> men
Writer’s cramp:
- Dystonic posturing of arm when hand used to perform
specific tasks e.g. writing, playing piano
Blepherospams+ Oromandibular
dystonia = Meige
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Dystonia)
Dystonia Treatment:
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Akathitic Movements
• “Unable to sit still”
• Feeling of inner, general restlessness that
is reduced or relieved by moving about
• Complex and usually stereotyped
movements
• Can be both generalized and focal
• Can usually be briefly suppressed
• Most common cause is iatrogenic
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Restless Leg syndrome – Ekbom’s
syndrome
The four core symptoms required for diagnosis are as
follows: an urge to move the legs, usually caused or
accompanied by an unpleasant sensation in the legs;
symptoms begin or worsen with rest; partial or complete
relief by movement; worsening during the evening or night.
Primary RLS is usually genetic.
Secondary RLS may be associated with pregnancy or a range
of underlying disorders, including anemia, ferritin deficiency,
renal failure, and peripheral neuropathy.
Most RLS sufferers have mild symptoms that do not require
specific treatment. If symptoms remain intrusive, low doses
of dopamine agonists, e.g., pramipexole (0.25–0.5 mg) and
ropinirole
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1–2 hours before bedtime
Hemifacial Spasm
• Unilateral facial muscle contractions
• Continual, rapid, brief, repetitive spasms
• Can evolve into sustained tonic spasms
• Can often be brought out when patient
voluntarily and forcefully contracts the
facial muscles
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Myoclonus
• Sudden, shock-like involuntary movements
caused by muscular contractions or
inhibitions
• Can occur at rest or during activity (action
myoclonus)
• Can be both arrhythmic or rhythmic
• Numerous classification schemes
• Differs from tics by interfering with
normal movement & not suppressible.
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Myoclonus
May be caused by active muscle contraction
- positive myoclonus
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Myokymia
• Fine quivering or rippling of muscles
• Most commonly occurs in facial muscles
• Distinguished from benign fasciculations on
EMG which shows rhythmic discharges in
doublets and triplets
• Causes: Pontine lesions (MS), Pontine
gliomas
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Myorhythmia
• Term used to describe a slow frequency
(<3Hz), prolonged, rhythmic or repetitive
movement which does not have the sharp
movement of a myoclonic jerk
• Typically described in context of Whipple’s
disease
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Paroxysmal Dyskinesias
• Refer to dyskinetic movements that appear suddenly and
then disappear after a variable period of time
1. Paroxysmal kinesigenic dyskinesia
- Triggered by sudden movement and lasts seconds to
minutes
- Can be hereditary or symptomatic
- Can be dystonic, ballistic or choreic
2. Paroxysmal nonkinesigenic dyskinesia
- Often familial, triggered by stress, fatigue, caffeine or
alcohol
- Lasts minutes to hours
3. Episodic ataxias
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Tardive dyskinesia
Involuntary movements as facial grimacing , chewing movements,
tongue movements (oro-bucco-mandibular dyskinesia).
Appear after weeks, generally years of exposure to dopamine
receptor blocking drugs. Older or classical ‘typical’ antipsychotics e.g.
chlorpromazine, haloperidol
With typical antipsychotics prevalence around 20%
Thought to be due to receptors super sensitivity to these drugs .
Changes in synapse number and dendrite arborisation also occur.
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DEEP BRAIN STIMULATION
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Let’s Talk About History
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Elements of the history
• Time course/functional disability/effect upon
quality of life
• Past medical history, including infections and
toxin exposures
• Drug history (current, previous, recreational)
• Alcohol responsiveness
• Family history
• Neuropsychiatric features
• Autonomic symptoms
• Sleep problems
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Other elements of history
• Do specific actions provoke the movement?
• Do the movements occur in relation to other
actions?
• Do the movements occur during sleep?
• Are there any associated sensory symptoms?
• Can the movements be suppressed?
• Are there aggravating or alleviating factors?
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Points to remember on exam
• Observe casually during history:
– Any involuntary movements and their distribution
– Utterances and vocalizations
– Blink frequency
– Excessive sighing
• Cognitive assessment
• Cardiovascular – orthostatics, limb colour
• Gait, axial tone
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Points to remember on exam..con’d
• Eye movements (range, speed)
• Limb examination (writing, hand posture)
• Tremors/postures
• Tone
• Power and co-ordination
• Fine finger and rapid alternating
movements
• Reflexes/plantars
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Observation
• Rhythmic vs. arrhythmic
• Sustained vs. nonsustained
• Paroxysmal vs. Nonparoxysmal
• Slow vs. fast
• Amplitude
• Supressibility
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Rhythmic vs Arrhythmic
Rhythmic Arrhythmic
• Tremor • Akathitic movements
• Dystonic tremor • Athetosis
• Dystonic myorhythmia • Ballism
• Myoclonus (segmental) • Chorea
• Myoclonus (oscillatory) • Dystonia
• Moving toes/fingers • Hemifacial spasm
• Periodic movements of • Hyperekplexia
sleep • Arrhythmic myoclonus
• Tardive dyskinesia • Tics
(stereotypy)
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Paroxysmal vs. Nonparoxysmal
Paroxysmal Continous
• Tics • Abdominal
• PKD dyskinesias
• Athetosis
• PNKD
• Tremors
• Sterotypies
• Dystonic
• Akathic postures
movements • Myoclonus,
• Moving toes rhythmic
• Myorhythmia • Tardive sterotypy
• Myokymia
• Tic status
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Hyperkinesias that persist during sleep
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