BASIC APPROACH TO MOVEMENT

DISORDERS
DR GANESHGOUDA MAJIGOUDRA
CONSULTANT NEUROLOGIST
NANJAPPA HOSPITALS DAVANAGERE
9380906082
ganeshgoudam4@gmail.com
 DEFINITION

• Movement disorders can be defined as neurologic syndromes in which there is

either an excess of movement or a paucity of voluntary and automatic
movements, unrelated to weakness or spasticity

Sir William Osler

PATHOPHYSIOLOGY OF MOVEMENT DISORDERS
PATHOGENESIS OF MOVEMENT DISORDERS
PATHOGENESIS OF MOVEMENT DISORDERS
ANOTOMICAL LOCALIZATION
ANOTOMICAL
LOCALIZATION
FOR EVERY MOVEMENT DISORDERS
FOR EVERY MOVEMENT DISORDERS
FOR EVERY MOVEMENT DISORDERS
 INTRODUCTION

Major categories:
1. Hyperkinetic movement disorders
• Excessive, often repetitive, involuntary movements that intrude into the normal flow of
motor activity
• Includes chorea, dystonia, myoclonus, stereotypies, tics, and tremor.

2. Hypokinetic movement disorders

• Refers to akinesia (lack of movement), hypokinesia (reduced amplitude of movement),
bradykinesia (slow movement), and rigidity.
• Parkinsonism is the primary hypokinetic movement disorder.
 TYPES OF MOVEMENT DISORDERS

• Can also be categorized into primary and secondary disorders.

1. Primary movement disordersthe abnormal movement is the primary manifestation
of the disorder

2. Secondary movement disorders broader structural, toxic, metabolic, or inherited

etiologic factor is responsible for the symptoms.
TYPES OF MOVEMENT DISORDERS
TYPES OF MOVEMENT DISORDERS
MIXED MOVEMENT DISORDERS
HYPOKINESIAS
 AKINESIA/BRADYKINESIA

• Akinesia absence of movement

• Bradykinesia slowness of movement

• Hypokinesia decreased amplitude of movement

•
• All three terms commonly grouped together & referred to as bradykinesia

• Bradykinesia a necessary sign for the diagnosis of PD (UK parkinson disease society’s
brain bank criteria)
 CATATONIA, PSYCHOMOTOR DEPRESSION
AND OBSESSIONAL SLOWNESS

• Catatonia
1. Catalepsy (abnormal maintenance of posture or physical attitudes)
2. Waxy flexibility (retention of the limbs for an indefinite period in the positions in which they
are placed)
3. Negativism
4. Mutism
5. Bizarre mannerisms

• Patients with catatonia can remain in one position for hours and move exceedingly slowly to
commands, usually requiring the examiner to push them along
• However, when moving spontaneously, they move quickly, such as when scratching themselves
 CATATONIA, PSYCHOMOTOR DEPRESSION
AND OBSESSIONAL SLOWNESS

• Depression commonly associated with a general slowness of movement and of thought

psychomotor retardation
• Catatonia can be considered an extreme case of this problem.
• Obsessional slowness (extreme slowness of movement) seen in some patients of obsessive-
compulsive disorder (OCD)
1. Difficulty initiating goal-directed action+many suppressive interruptions and perseverative
behavior.
2. May have cogwheel rigidity, decreased arm swing when walking, decreased spontaneous
movement, hypomimia, and flexed posture.
3. However no decrementing of either amplitude or speed with repetitive movements, no
tremor, and no micrographia. Also no freezing or loss of postural reflexes
 FREEZING

• Transient periods, usually lasting several seconds, in which the motor act is halted, being stuck in
place
• Types
1. Start-hesitation (freezing when gait is initiated)
2. Turning-hesitation (freezing when turning)
3. Destination-hesitation (freezing when approaching the target)
4. Freezing when a physical or a temporal “obstacle” is encountered
5. Freezing when movement is time-dependent (e.g., entering or leaving elevator before door
closes)
6. Spontaneous sudden transient freezing
7. Palilalia or freezing of speech
 HESITANT GAITS(CAUTIOUS GAITS)

• Common in elderly or in patients who have experienced a fall due to some cause

• Patients become cautious due to anxiety that they will fall

• Gait is like what it would be for normal people if they were to walk on ice
Clinical
features
associated
with various
hypokinetic
syndromes
 HYPOTHYROID SLOWNESS

• Hypothyroidism
1. Decreased metabolic rate
2. Cool body temperature
3. Bradycardia
4. Myxedema
5. Loss of hair
6. Hoarseness
7. Myotonia
8. Severe hypothyroidism can also feature motor slowness, weakness, and lethargy.
• Need to differentiate with PD on symptom complexes
 STIFF MUSCLES

• It is the result of continuous muscle firing without muscle disease and not rigidity or
spasticity.

• There are four major clinical categories of stiff-muscle syndromes

1. Continuous muscle fiber activity or neuromyotonia
2. Encephalomyelitis with rigidity
3. Stiff-limb syndrome
4. Stiff-person syndrome
HYPERKINESIAS
CHARACTERSTICS OF HYPERKINETIC DISORDERS
 DYSKINESIAS
• All hyperkinetic movements are technically dyskinesias,

• But the term is often used to encompass complex involuntary movements that do not neatly fit into
another category

• Dyskinesia is used most often to refer to abnormal involuntary movements related to drugs.

• Dyskinesias are a common dose-related complication of the treatment of PD with levodopa and
dopamine agonists.

• In some disorders, the dyskinesias occur paroxysmally.

 AKATHISIA

• Greek word meaning “unable to sit still”

• Feeling of inner, general restlessness that is reduced or relieved by moving about.
• The typical akathitic patient, when seated
Cross and uncross the legs
Rock the trunk
Twist
Get out of the chair often to pace back and forth
Even make noises such as moaning
• Carrying out these motor acts brings relief from the sensations of akathisia.
• Akathitic movements are complex and usually stereotyped; the same type of movements are employed
over and over
 ATAXIA/ASYNERGIA/DYSMETRIA

• Interchangeable terms  decomposition of movement as a result of breakdown of normal

coordinated execution of a voluntary movement

• Ataxia  cardinal clinical features of cerebellar disease or of pathways to and from cerebellum.
Rather than a smooth, continuous movement; the limb wanders off its trajectory attempting to a
reach a target, with corrective maneuvers that resemble oscillations of the limb

• Dysmetria  During Finger nose finger test here the limb usually misses the target

• Dysmetria with cerebellar dysfunction is due to overshooting (hypermetria) and undershooting

(hypometria) of the target.
 ATHETOSIS

• Used in two senses

1. To describe a class of slow, writhing, continuous, involuntary movements
2. To describe the syndrome of athetoid cerebral palsy (a result of injury to the basal ganglia
in the prenatal or perinatal period or during infancy)
• Affect the limbs, especially distally, but can also involve axial musculature, including neck,
face, and tongue
• Overflow-
• When not present in certain body parts at rest, it can often be brought out by having the patient
carry out voluntary motor activity elsewhere in the body.
• Like speaking can induce increased athetosis in the limbs, neck, trunk, face, and tongue
 ATHETOSIS

• Athetosis often is associated with sustained contractions producing abnormal posturing. So it blends with
dystonia.
• Although the speed of athetosis is usually slow, sometimes it is fast enough to blend in with the speed of
chorea, and the term choreoathetosis is used.
• Athetosis resembles “slow” chorea in that the direction of movement changes randomly and in a flowing
pattern
• Pseudoathetosis
1. Distal athetoid movements of the fingers and toes
2. Result of loss of proprioception
3. Either to sensory deafferentation (sensory athetosis) or to central loss of proprioception
 BALLISM

• Very-large-amplitude chorea movements of the proximal parts of the limbs

• Causing flinging and flailing limb movements

• Most frequently unilateral, in which case it is referred to as hemiballism

• Rarely ballism occurs bilaterally (biballism) and is due to bilateral basal ganglia involvement
 CHOREA

• They are
1. Involuntary
2. Irregular
3. Purposeless
4. Nonrhythmic
5. Abrupt
6. Rapid
7. Unsustained movements
8. That seem to flow from one body part to another.
• A characteristic feature of chorea is that the movements are unpredictable in timing, direction, and
distribution (i.e., Random).
CHOREA
CHOREA
 DYSTONIA

• Movements
1. Tend to be sustained at the peak of the movement
2. Are usually twisting and frequently repetitive
3. Often progress to prolonged abnormal postures
• These movements repeatedly involve the same group of muscles—that is, they are patterned
(Differ from chorea)
• Agonist and antagonist muscles contract simultaneously (cocontraction) to produce the
sustained quality of dystonic movements.
• Speed may vary widely from slow (athetotic dystonia) to shock-like (myoclonic dystonia).
 DYSTONIA

• Time
1. Lasting less than a second Dystonic spasms.
2. Sustained for several seconds Dystonic movements.
3. Lasting minutes to hoursDystonic postures. If present for
weeks or longer, the postures can lead to permanent fixed
contractures
• Sensory tricks (gestes antagoniste)
1. One of the characteristic and almost unique feature of dystonic
movement
2. They can often be diminished by tactile or proprioceptive
stimulus.
3. Like touching the involved body part or an adjacent body part
can often reduce the muscle contraction
DYSTONIA A&B-Oromandibular dystonia.
C-jaw opening dystonia
D-Image shows focal idiopathic cervical dystonia with
laterocollis
E- sensory trick of wearing a scarf and applying tension to the
posterior neck.
F-Focal truncal dystonia with Pisa syndrome.
G-Segmental dystonia
H, Segmental dystonia with idiopathic Meige syndrome
I-Idiopathic left hemifacial spasm is shown,
J- abnormal left lower facial posturing related to lower facial.
K-Hand dystonia
L- Multifocal bilateral arm dystonia is shown with notable
abnormal wrist extensor posturing and right shoulder
adduction
M-Task-specific focal hand dystonia is shown in a violinist
who exhibits flexion dystonia of the left little greater than
ring fingers.
N-Focal left foot dystonia involves excessive plantarflexion
in a patient with task-specific walking/ running dystonia.
O-Left foot dystonia with toe curling is shown in a patient
with generalized dystonia
DYSTONIA
DYSTONIA
 HEMIFACIAL SPASM

• Unilateral facial muscle contractions. Example of peripherally induced movement disorder

• Generally continual rapid, brief, repetitive spasms (clonic form of HFS)
• Also can be more prolonged sustained tonic spasms (tonic form) mixed with periods of
quiescence
• Often the movements can be brought out when the patient voluntarily and forcefully contracts
the facial muscles; when the patient then relaxes the face, the involuntary movements appear.
• HFS usually affects both upper and lower parts of the face ipsilaterally, but patients are
commonly more concerned about closure of the eyelid than about the contractions of the cheek
or at the corner of the mouth.
 HYPNOGENIC DYSKINESIAS: PERIODIC MOVEMENTS
IN SLEEP AND REM SLEEP BEHAVIOR DISORDER

• REM sleep behavior disorder (RBD), here there is lack of somatic muscle atonia, thus enabling
such individuals to move while they dream (acting out their dreams).

• The affected individual is unaware of these movements unless awakened by falling out of bed or by
the bed partner who might have been struck or kicked by the abnormal movements and then awakens
the person to stop the movements.

• RBD may precede by several years the development of a subsequent synucleinopathy but may
instead develop after the onset of the synucleinopathy, and not all individuals with RBD will develop
a synucleinopathy .
 ASTERIXIS

• Is an inability to sustain normal muscle tone.

• With the arms outstretched and wrists extended, “like stopping traffic,” the lapse in postural tone
may cause the hands to suddenly fl op downward, then quickly recover, causing a slow and irregular
flapping motion that led to the term “liver flap.”
• When severe, the entire arm may drop.
• Other body parts may exhibit the phenomenon (e.g., inability to keep the foot dorsiflexed [foot
flap]).
• Unilateral asterixis may occur with focal brain lesions, particularly involving the contralateral
thalamus.
• In metabolic encephalopathy, there may be a high-frequency postural tremor that begins after a
latent period of 2 to 30 seconds and attenuates on movement (mini-asterixis, metabolic tremor).
• In unresponsive patients, asterixis at the hip joints can sometimes be brought out by passively
flexing and abducting to hips so that the thighs form a “V.” In this position, the knees may flap up
and down
 PAINFULL MOVING TOES AND
FINGERS

• Here the toes of one foot or both feet are in continual flexion–extension with some
lateral motion, associated with a deep pain in the ipsilateral leg

• The constant movement has a sinusoidal quality

• The movements and pain are continuous, and both occur even during sleep, though
they may be reduced and the normal sleep pattern may be altered.

• The leg pain is much more troublesome to the patient than are the constant movements.
 MYOCLONUS

• Sudden, brief, shock-like involuntary movements caused by

1. Muscular contractions (positive myoclonus) or
2. Inhibitions (negative myoclonus) like asterixis
• Myoclonic jerks are usually irregular (arrhythmic) but can be rhythmic (such as in palatal
myoclonus)
MYOCLONUS
MYOCLONUS
MYOCLONUS
 MYOKYMIA AND SYNKINESIS

• Myokymia fine persistent quivering or rippling of muscles (sometimes called live flesh by patients).

• Myokymia occurs most commonly in facial muscles Some causes

1. Pontine lesions like Multiple sclerosis
2. Less often to pontine glioma
• Synkinesis Aberrant reinnervation of the facial nerve after denervation, such as from Bell palsy, is
manifested by occurrence of involuntary movements in one part of the face accompanying voluntary
contraction of another part.
• For example, moving the mouth in a smile may cause the eyelid to close or closing the eyelid may
cause contraction of the ipsilateral lower facial muscles
 MYORHYTHMIA

• Earlier refer to the somewhat rhythmic movements in patients with torsion dystonia. Today, these
are simply called dystonic movements and do not distinguish between the movements that are
repetitive and those that are not
• Now it is defined as somewhat slow frequency (<3 Hz) prolonged, rhythmic or repetitive
movement in which the movement does not have the sharp square wave appearance of a
myoclonic jerk
• Seen in Whipple disease to describe slow-moving, repetitive, synchronous, rhythmic contractions
in ocular, facial, masticatory, and other muscles, so-called oculofaciomasticatory myorhythmia
 PAROXYSMAL DYSKINESIAS

• Here there are various types of dyskinetic movements, particularly choreoathetosis and dystonia,
that occur out of the blue and then disappear after being present for seconds, minutes, or hours
• The patient can remain normal for months between attacks, or there can be many attacks per day
• They are usually familial
• Paroxysmal kinesigenic dyskinesia characteristically triggered by a sudden movement, and the
abnormal movements last only seconds to a few minutes
• Paroxysmal nonkinesigenic dyskinesia can be hereditary or symptomatic; is triggered by
stress, fatigue, caffeine, or alcohol; and can last minutes to hours
 STEREOTYPY

• Non–goal-directed movement or vocalization pattern that is repeated continually for a period of

time in the same form and on multiple occasions and is typically distractible
• There may be long periods of minutes between movements, or the movements may be very
frequent.
• When they occur at irregular intervals, stereotypies may not always be easily distinguished
from motor tics, compulsions, gestures, and mannerisms.
 TICS
• Can consist of
1. abnormal movements (motor tics)
2. abnormal sounds (phonic tics)
• When both types of tics are present, the designation of Gilles de la Tourette syndrome or Tourette
syndrome
• Tics frequently vary in severity over time and can have remissions and exacerbations.
• Sensory urge
1. Like akathitic movements, tics are usually preceded by an uncomfortable feeling or sensory
urge that is relieved by carrying out the movement, like “scratching an itch.”
2. Thus, the movements and sounds can be considered “unvoluntary.”
3. Unless very severe, tics can be voluntarily suppressed for various periods of time.
4. But when they are suppressed, inner tension builds up and is relieved only by an increased
burst of more tics
TICS
 TREMOR

• Oscillatory, typically rhythmic and regular, movement that affects one or more body parts, such
as the limbs, neck, tongue, chin, or vocal cords
• Jerky, irregular “tremor” is usually a manifestation of myoclonus or dystonia
• Tremor is produced by rhythmic alternating or simultaneous contractions of agonists and
antagonist muscles.
• The rate, location, amplitude, and constancy vary depending on the specific type of tremor and
its severity
 TREMOR

• Tremors can be classified as

1. tremor-at-rest (with the patient sitting or lying in repose)
2. Postural tremor (with the arms or legs extended in front of the body)
3. Action tremor (such as writing or pouring water)
4. Intention tremor (with intention maneuvers like bringing the finger to touch the nose)
• Phenomenology
1. most common cause of a rest tremor parkinsonism particularly PD.
2. most common cause of postural and action tremor  essential tremor
3. most common cause of intention tremor  lesion in the cerebellar outflow pathway.
4. A combination of rest tremor and a worse action and intention tremor manifestation of a
lesion in the midbrain commonly mislabeled as “rubral” tremor, but is more appropriately
called midbrain tremor or Holmes tremor because of involvement of both the nigrostriatal and
dentato-rubro-thalamic pathway
TREMOR
TREMOR
TREMOR
TREMOR
 FASCICULATIONS

• Fine, rapid, flickering or vermicular twitching movements due to contraction of a bundle,

or fasciculus, of muscle fibers.
• They are usually not extensive enough to cause movement of joints, except occasionally
the digits.
• They vary in size and intensity, from so faint and small as to only slightly ripple the surface
of the overlying skin, to coarse and impossible to overlook.
• They are random, irregular, fleeting, and inconstant. At times, they are abundant; at other
times, they require a careful search.
FASCICULATIONS
 PSYCHOGENIC MOVEMENT
DISORDERS

• Can simulate virtually any type of movement disorder.

• Psychogenic disorders do not correspond to any of the organic types of abnormal involuntary
movement; they are bizarre, change in type from time to time, and are influenced by emotional
state and suggestion.
• Onset is often sudden. If a movement disorder is bizarre and defines classification, the
possibility that it may be psychogenic should be borne in mind.
• Peculiar motor behaviors occur frequently in major psychiatric illnesses such as schizophrenia.
• However, being bizarre and difficult to characterize does not necessarily mean a movement
disorder is psychogenic
 WHEN TO SUSPECT A MOVEMENT DISORDER WITH
NEURONAL ANTIBODIESCHARACTERISTIC SYNDROMES

• Characteristic Syndromes
• Faciobrachial dystonic seizures-antiLGI1
• Leg myoconus-antiCaspr
• Orofacial and limb dyskinesias in states of reduced consciousness-antiNMDAR
• Finalistic movements in NREM sleep-antilglONS
• Painful tonic spasms-antiAQP4
 WHEN TO SUSPECT A MOVEMENT DISORDER WITH
NEURONAL ANTIBODIESCHARACTERISTIC SYNDROMES

• Red Flags
• Hyponatraemia episodic bradycardia-antilGI1
• Sleep disorders /breathing disorders /dysphagia-antilglONS
Longlasting diarrhea-antiDPPX
• Headache and blurred vision-antiGFAP
• New symptoms after recent herpes simplex encephalicis
• Other
• Unilateral signs without lesion on imaging
• Rapid evolution
• Inflammatory CSF or MRI
• History of malignacy
BASIC INVESTIGATIONS
INVESTIGATIONS
INVESTIGATIONS
INVESTIGATIONS
INVESTIGATIONS
TREATMENT ASPECT
DYSTONIA
TREMOR
 TREATMENT OF CHOREA

• Therapy should be directed at the underlying cause,

• But in general, treatment of chorea is symptomatic.
• Decreasing dopamine neurotransmission is a major therapeutic mechanism- achieved through postsynaptic
blockade, ideally with atypical neuroleptics, or with presynaptic depletion, using tetrabenazine or reserpine
• Monitor for the side effects of depression, parkinsonism, and akathisia
• Glutamate NMDA receptor antagonists, such as amantadine, may be helpful in HD,
• Anticonvulsants may be tried, particularly levetiracetam, valproic acid, and carbamazepine. The mechanism
of action of these agents in chorea is unclear
• Deep brain stimulation or ablative procedures, with mixed outcomes the customary target is the internal
segment of the globus pallidus; however, the subthalamic nucleus and the motor thalamic nuclei have also
been targeted
 TREATMENT OF MYOCLONUS
• The ideal way to control myoclonus is to treat the underlying disorder, as this may partly or totally
reverse certain types of myoclonus.
•
Examples include myoclonus caused by an acquired abnormal metabolic state, a removable
medication or toxin, an excisable lesion, or a psychogenic etiology .
•
With most types of myoclonus, however, treatment of the underlying disorder is impossible or
ineffective. In these cases, symptomatic treatment is justified if the myoclonus is disabling.

Unfortunately, there is sparse evidence from controlled clinical trials to direct myoclonus therapy, in
part because no drug has been designed or marketed specifically for the purpose of treating
myoclonus.
•
Nearly all of the evidence comes from observational case reports and case series
•
Valproate, piracetam,leveteracetam,valproate and clonazepam beneficial
TICS
 CONCLUSION

• There are eight predominant movement disorders commonly encountered

1. Akinesia/bradykinesia
2. Rhythmic tremor and other rhythmic movements
3. The sustained contractions of dystonia/athetosis
4. Three types of usually fast movements—myoclonus, chorea/ballism, and tics
5. Stereotypies/ compulsions
6. Paroxysmal dyskinesias
7. Ataxia/asynergia
8. Hypnogenic dyskinesias
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