Involuntary movements

INVOLUNTARY
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)

 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 are together referred to as
the striatum.
Basics
2www.medicalgeek.com - vitrag24

Coronal view of the brain, showing the main basal ganglia nuclei.
Obeso J A et al. Physiology 2002;17:51-55

Summary of the main connections of the “motor circuit” of the basal ganglia.
Obeso J A et al. Physiology 2002;17:51-55

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

Broadly . . .
Hyperkinetic Movements Hypokinetic Movements

Or More Specifically . . .
Hyperkinetic Movements
• Tremor (ET-Most common)
• Chorea/Athetosis
• Dystonia
• Ballism
• Myoclonus
• Tics
• Ataxia
• Myokymia
• Myorrhythmia
• Restless Legs
• Hyperkplexia (startle response)
• Akathesia
Hypokinetic Movements
• Parkinsonism (2nd most
common)
• Apraxia
• Hesitant gaits
• Hypothyroid slowness
• Rigidity

 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
Physiological Involuntary movements

Bradykinesia/Akinesia
• Decreased movement
• Common to parkinsonism
• 6 Cardinal Features of Parkinsonism:
– Tremor at rest
– Bradykniesia/Hypokinesia/akinesia
– Rigidity
– Flexed posture of the neck, trunk and limbs
– Loss of postural reflexes
– Freezing

Types of Parkinsonism
• Parkinson’s Disease (most common
encountered by neurologists)
• Drug-induced Parkinsonism (probably the
most common overall)

Features Suggestive of Atypical PD

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

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

•Probably the most common movement disorder.
•Hallmark: postural and action tremor affecting the hands, head and/or
voice. Presents as a rhythmic tremor (4–12 Hz). Typically bilateral,
accentuated with goal-directed movement (Action tremor) as opposed
to rest tremor (PD) & responds to alcohol.
•Often, it is inherited as an autosomal dominant trait with the tremor
becoming apparent by middle age and sometimes as early as childhood.
•Diagnosis-Clinical grounds; No gene or identifiable pathology has been
identified for essential tremor. Abnormalities of the basal ganglia, the
cerebellum, the thalamus, the connections between these structures, or
a combination of factors may be causative.
Essential Tremor
Katherine
Hepburn

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).

•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
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?

Because……..
 They often co-exist
 Even neurologists may often not be able to agree as to
how a particular movement should be classified!

The spectrum
Ballismus DystoniaTic Chorea Athetosis
Movements become - Less violent / explosive / jerky
- Smoother and more flowing
- More sustained
They differ from tics in that they cannot be suppressed by
voluntary control
Myoclonus

Ballism
 More dramatic ballistic movements of of the arms & legs on one side
of the body (unilateral) and therefore use term “ HEMIBALLISMUS”.
 Large amplitude choreic movements of the proximal parts of the
limbs, Usually Unilateral,Violant & flinging movements
 Treatment with antipsychotics is often effective.
 Most common cause of hemi-ballism:
CVA in contralateral subthalamic nucleus
 Second most common cause of ballism:
Overdose of levodopa

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

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

•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!
•Distinguished from other involuntary movements by the ability of the patient to
suppress their occurrence, at least for a short time.
•Often-times, affected individuals have co-existing obsessive-compulsive disorder,
learning disabilities, hyperactivity/attention deficit disorder and behavioral problems.
•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.
•Treatment of tics is symptomatic with haloperidol and other neuroleptics.
•Some patients benefit from Clonidine, or Guanfancine an -2 agonist, or with
benzodiazepines.
•May be transmitted as an autosomal dominant trait
Tourette’s Syndrome (tics)

Tourette’s Syndrome
(tics)

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

Causes of Tics

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

Athetosis is a slow continuous stream of slow, sinuous, writhing movements,
typically of the hands and feet.
Most commonly seen together with chorea in dyskinetic motor fluctuations in
PD.
Also in athetoid cerebral palsy where damage occurs in the basal ganglia.
Related to excessive dopaminergic activity. In PD reducing dopaminergic drugs
alleviates.
If Athetosis becomes faster, it sometimes blends with chorea, ie
choreoathetosis/ 'choreo-athetoid' movements. Can be thought of as an
athetoid movement that “gets stuck” for a period of time; thus, a patient with
choreoathetosis may perform an involuntary movement in which his hand and
fingers are twisted behind his head. He may hold this position for a few
moments before his hand moves back in front of his body. The part of the
movement when the limb was held, unmoving, in an abnormal position would
be considered a dystonia (may occur alone).
Athetosis
• Pseudoathetosis = distal athetoid
movements of the fingers and toes
due to loss of proprioception

Dystonia
• An abnormal movement characterized by
sustained muscle contraction, frequently
causing twisting, and repetitive movements
or abnormal postures
• Tend to be patterned (in the same muscles)

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

Common types of Dystonia
 Benign paroxysmal torticollis in infancy
 Dopa responsive dystonia
 Idiopathic torsion dystonia (Oppenheim’s dystonia)
 Transient idiopathic dystonia of Infancy
 Secondary dystonia:
1.Structural brain damage :Hemidystonia
2. Metabolic dystonia: Glutaric aciduria
Wilson’s disease, Lesch Nyan syndrome
Homocystinuria.

Examples of focal dystonias
 Blepharospasm means the involuntary contraction of the eyelids,
leading to uncontrollable blinking and closure of the eyelids. Affects
women> men
 Torticollis, commonly called wry neck, is the condition of spasm
affecting the muscles of the neck, causing the head to assume
unnatural postures or turn uncontrollably. Spasmodic torticollis,
also known as cervical dystonia, is the most common of the focal
dystonias.
 Writer’s cramp:
- Dystonic posturing of arm when hand used to perform
specific tasks e.g. writing, playing piano
Blepherospams+ Oromandibular
dystonia = Meige syndrome(Orofacial
Dystonia)

 Anticholinergics (benzatropine), antihistamines
(diphenhydramine), anti-Parkinsons agents
(trihexyphenidyl), and muscle relaxers (diazepam) &
GABAB receptor agonist (baclofen).
 The drug resistant dystonias can be treated by botulinum
toxin injection to the responsible muscles, to overcome
the abnormal distribution of muscle activity for a period
of time.
 Any patient with childhood onset dystonia should
receive levodopa trial to rule out dopa responsive
dystonia.
Dystonia Treatment:

Dystonia-drug induced
 Acute dystonic reaction- occulogyric crisis, sustained upward
deviation of the eyes +/- toricollis, jaw opening, tongue
protrusion and anxiety.
 Usually a result of administering dopamine receptor
blocking drugs, generally metaclopramide (antiemetic and
gastroprokinetic agent used to treat nausea and vomiting,
and to facilitate gastric emptying) or prochlorperazine
(neuroleptic; antiemetic) in young. 50% in 48hrs, 90% in 5
days.
 Treatment anticholinergic drugs, usually benztropine 1-2mg
IV

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

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 (1–2 mg), are given 1–2 hours before bedtime45www.medicalgeek.com - vitrag24

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

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.

May be caused by active muscle contraction
- positive myoclonus
 May be caused by inhibition of ongoing muscle activity
- negative myoclonus ( eg. Asterixis )
 Generalised - widespread throughout body
 Focal / segmental – restricted to particular part of body
Hypnogogic jerk – Normal during falling sleep or waking up
Treatment:Valproic acid is drug of choice May respond to
benzodiazepines-clonazepam , piracetam, primidone.
Myoclonus

Myoclonus Classification Schemes
• Clinical
– Spontaneous
– Action
– Reflex
– Focal
– Axial
– Multifocal
– Generalized
– Irregular
– Repetitive
– Rhythmic
• Pathophysiology
Cortical
Focal
Multifocal
Generalized
Thalamic
Brainstem
Reticular
Startle
Palatal
Spinal
Segmental
Propriospinal

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

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

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

 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.
Tardive dyskinesia

DEEP BRAIN STIMULATION

Let’s Talk About History

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

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?

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

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

Observation
• Rhythmic vs. arrhythmic
• Sustained vs. nonsustained
• Paroxysmal vs. Nonparoxysmal
• Slow vs. fast
• Amplitude
• At rest vs. action
• Patterned vs. non-patterned
• Combination of varieties of movements
• Supressibility

Rhythmic vs Arrhythmic
Rhythmic
• Tremor
• Dystonic tremor
• Dystonic myorhythmia
• Myoclonus (segmental)
• Myoclonus (oscillatory)
• Moving toes/fingers
• Periodic movements of
sleep
• Tardive dyskinesia
(stereotypy)
Arrhythmic
• Akathitic movements
• Athetosis
• Ballism
• Chorea
• Dystonia
• Hemifacial spasm
• Hyperekplexia
• Arrhythmic myoclonus
• Tics

Paroxysmal vs. Nonparoxysmal
Paroxysmal
• Tics
• PKD
• PNKD
• Sterotypies
• Akathic
movements
• Moving toes
• Myorhythmia
Continous
• Abdominal
dyskinesias
• Athetosis
• Tremors
• Dystonic
postures
• Myoclonus,
rhythmic
• Tardive sterotypy
• Myokymia
• Tic status

Hyperkinesias that persist during sleep
• Secondary palatal myoclonus
• Nocturnal Myoclonus (Periodic Limb
Movement Disorder)
• Ocular myoclonus
• Oculofaciomasticatory myorhythmia
• Fasciculations
• Moving toes
• Myokymia
• Neuromyotonia
• Ressless leg syndrome

Refereces:
Dejong’s Neurological Examination – 7th Ed
DeMeyer’s Neurological Examination – 6th Ed
Localization in cinical neurology : Paul Brazis – 6th Ed
Harrison’s PRINCIPLES OF INTERNAL MEDICINE : Eighteenth
Edition
UpToDate (http://www.uptodate.com)
eMedicine (http://www.emedicine.com)
The Basal Ganglia and Disorders of Movement:
Pathophysiological Mechanisms : American Physiology
Society
Bates’ guide to physical examination & history taking – 11th Ed

That’s all folks!
Thank You for listening!

Involuntary movements

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Involuntary movements