Phenomenology of

Movement Disorder

Santoso
Movement Disorders is….
• Neurological syndromes:
• Excess of movement
• Paucity of voluntary and autonomic movement
• Unrelated to weakness or spasticity
• Excessive movements
• Hyperkinesia
• Dyskinesias (unnatural movements) :
• Chorea
• Dystonia
• Myoclonus
• Tics
• Tremor
• etc
• Abnormal involuntary movement
• The paucity movement
• Hypokinesia (decreased amplitude of movement)
• Bradykinesia n(slowness of movement)
• Akinesia (loss of movement)
• Movement disorder basically divided:
• Parkinsonism
• Others
Categories of movement
• Automatic
• Learned motor behavior that are performed without conscious effort
• e.g : Walking an accustomed route, tapping of the fingers when thinking about
something else
• Voluntary
• Intentional (planned or self-initiated) or externally triggered (in response to
some external stimulus)
• e.g : Turning the head toward a loud noise or withdrawing a hand from hot plate
• Semivoluntary (unvoluntary)
• Involuntary
Categories of movement
• Automactic
• Voluntary
• Semivoluntary (unvoluntary)
• Induced by an inner sensory stimulus or by an unwanted feeling or compulsion
• e.g: need to “stretch” a body part, need to scratch an itch, compulsive
touching or smelling), tics, akathisia, restless leg syndrome
• Usually suppressible
• Involuntary
• Non-suppressible or partially suppressible
• Tremor, myoclonus, chorea, dystonia, stereotypies, tics
Origins of abnormal movement
• Associated with pathologic alteration in the basal ganglia or their
connections, cerebellum or its pathways, and others
• Basal ganglia:
• Caudate nucleus
• Putamen (Lying deep within the cerebral hemispheres)
• Pallidum
• Subthalamic nucleus (dienchephalon)
• Substantia nigra (mesenchephalon)
• Pedunculopontine nuclei (the mesencephalic-pontine junction)
• The pathology of the cerebellum and its pathways results in:
• Impairment of coordination (asynergy, ataxia)
• Misjudgment of distance (dysmetria)
• Intention tremor
• Myoclonus and many forms of tremors can be resulted from the:
• Cerebral cortex  cortical reflex myoclonus
• Brainstem  cerebellar outflow tremor, reticular reflex myoclonus, hyperekplexia,
rhythmical brainstem myoclonus such as palatal myoclonus, ocular myoclonus
• Spinal cord  rhythmical segmental myoclonus and non-rhythmic propriospinal
myoclonus
• Cerebellum  Ramsay hunt syndrome of progressive myoclonic ataxia
• The peripheral nervous system  painful legs-moving toes syndrome
Etiology
• Genetics
• Parkinson disease or variants of classic PD  PARK4 (triplication of the
normal alfa synuclein gene), PARK1
• etc
• Others
Evaluation
• Traditional approach in neurology:
• Where is the lesion
• What is the lesion
• How do we treat this problem
• In the movement disorders:
• Is any abnormality involuntary movement actually present
• Determine the category of the involuntary movement (nature of the involuntary movement)
• Rhythmicity, speed, duration, pattern, induction, complexity of the movements, suppressibility by
volitional attention or by sensory tricks, whether the movements are accompanied by sensations such as
restlessness or urge to make a movement that can release a built-up tension, which body part are
involved
• Determine the etiology of the abnormal involuntary movement
• How best to treat the movement disorder.
Akinesia/Bradykinesia
Akinesia/Bradykinesia
• Bradykinesia refers to “absence, slowness, and decreased amplitude of movement
• Akinesia refers to lack of movement
• The prominent and most important feature of parkinsonism
• Parkinsonisms:
• Tremor at rest
• Bradykinesia/hypokinesia/akinesia
• Rigidity
Cardinal features
• Flexed posture of neck, trunk and limbs
• Freezing
• Loss of postural reflexes
• Diagnosis criteria : at least two of cardinal features, one of them being tremor at rest, and
bradykinesia
• Cause of parkinsonism:
• Primary/idiopathic  Parkinson disease
• Secondary  vascular parkinsonism, drug-induced parkinsonism
• Parkinsonism-plus syndromes
• Heredodegenerative disorders
Parkinson Disease
• Idiopathic parkinsonism
• Tremor : present in the distal part of the extremities and the lips, “at
rest”, pill-rolling. If the tremor occurred in the cranial structures
consider other diagnosis such as essential tremor, cerebellar tremor,
dystonic tremor
• Akinesia/bradykinesia/hypokinesia: present in the cranial structures
such as masked facies (hypomimia), decreased frequency of blinking,
impaired upgaze, impaired ocular convergence, soft speech
(hypotonia), loss of inflection (aprosody), drooling of saliva due to
decreased spontaneous swallowing.
• How to examine akinesia/bradykinesia/hypokinesia (cranial structures)
• Repetitive tapping of the glabella  non-suppression of blinking (Myerson sign)
• Ask to open the eyelid after the eyelids were forcefully closed
• Normal in PD
• Abnormal in progressive supranuclear palsy  apraxia of eyelid opening
• Ask to see or look straight a head
• Quiet in PD
• Abnormal (there is wave jerk) in some parkinsonism-plus syndrome such as progressive
supranuclear palsy
• Check the saccadic ocular movement
• Normal in PD (some impaired for upgaze and convergence)
• Abnormal (impaired downgaze)  some parkinsonism-plus syndrome such as progressive
supranuclear palsy or cortico-basal ganglionic degeneration
• How to examine akinesia/bradykinesia/hypokinesia (distal structures)
• Arms
• Slowness in shrugging or relaxing the shoulder
• Slowness in raising the arm
• Loss of spontaneous movement such as gesturing, smallness and slowness of
handwriting (micrographia)
• Slowness and decrementing amplitude of repetitively opening and closing the hands,
tapping a finger and twisting the hand back and forth
• Difficulty with hand dexterity for shaving, brushing teeth, putting on makeup,
• Decreased armswing when walking
• How to examine akinesia/bradykinesia/hypokinesia (distal structures)
• Legs
• Slowness and decrementing amplitude in repetitively stomping the foot or tapping the
toes
• Slowness in making the number 8 with the foot
• Slow, short-strided, shuffling gait with reduced heel strike when stepping forward
• Trunks
• Difficulty rising from a chair, getting out of automobiles and turning in bed
• Bradykinesia : loss of automatic movements as well as slowness in
initiating movement on command and reduction in amplitude of the
voluntary movement
• Early feature : reduction of amplitude is the decrementing of the
amplitude with repetitive finger tapping or foot tapping, impaired rhythm
of the tapping
• Carrying out two activities stimultaneously is impaired
• Conclusion: Bradykinesia : decreased rapid successive movement both in
amplitude and speed.
• To overcome : stimulate the sufficient sensory input  kinesia paradoxica
• Rigidity
• In distal limbs
• A ratchety “give” when a joint is passively moved throughout its range of motion 
cogwheel rigidity
• Proximal joint
• Can be found by swing the shoulders (Wartenberg sign), rotating the hips
• Complain from patient : stiffness of the neck
• Flexed posture in the neck, thorax, elbows, hips and knees
• Camptocormia (an abnormal flexion of the trunk appearing in standing
position, increasing while walking and abating in the supine position)
• Pronounced kyphoscoliosis with truncal tilting
• Loss of postural reflexes
• Occurs later in the disease
• Difficulty righting himself or herself after being pulled off balance
• How to examine : pull test
• Examiner stand behind the patient
• Give a firm tug on the patient’s shoulders towards the examiner
• Explaining the procedure in advance
• Directing that the patient should try to maintain his balance by taking a step backward
• Response:
• Normal : recover within two steps
• Abnormal :
• Require several steps to recover balance  mild
• Greater degree of retropulsion  moderate
• The patient would fall if not caught by examiner  severe
• Combination of loss of postural reflexes and stooped posture  festination
• The patient walk faster and faster, trying to catch up with his or her center of gravity to prevent falling
• Freezing phenomenon
• Not akinesia
• The feet take short, sticking, shuffling steps when the patient initiates
walking, or turning-hesitation while walking
• Feet become “glued to the ground”
• Destination-freezing : stopping before reaching the final destination
• Bradyphrenia
• Mental slowness, analogous to the motor slowness of bradykinesia
• Slowness in thinking or responding to questions
• Tip-of-the-tounge phenomenon
• Patient know the correct answer
• Age onset of Parkinson disease
• Usually above the age of 40
• Young onset Parkinson disease (20-40 yo)
• Juvenile parkinsonism (before 20 yo)  consider Wilson disease, the
Westphal variant of Huntington disease, familial and sporadic primary
juvenile parkinsonism
Apraxia
Apraxia
• Disorder of voluntary movement that cannot be explained by weakness,
spasticity, rigidity, akinesia, sensory loss or cognitive impairment
• Lesion of cerebral cortex
• The voluntary movement cannot be executed
• Simple test :
• Ask the patients to copy a series of hand postures shown to them by examiner
• Ideomotor and limb-kinetic apraxia are found in number of movement
disorders
• Cortico-basal ganglionic degeneration
• Progressive supranuclear palsy
Categories of apraxia
• Ideational apraxia
• The concept or plan of movement cannot be formulated by patients
• Due to parietal lobe lessions
• How to examine:
• Asking the patient to perform a series of sequential movement
• Filling a pipe – lighting it – then smoking
• Putting a letter into an envelope – sealing it – then affixing a stamp

• Ideomotor apraxia
• Limb-kinetic apraxia
Categories of apraxia
• Ideational apraxia
• Ideomotor apraxia
• The concept or plan of movement is intact, but the individual motor engrams
or programs are defective
• Due to lesion of the dominant hemisphere (parietotemporal region, the
arcuate fasciculus, frontal lobe or corpus callosum)
• How to examine:
• Asking the patient to undertake specific motor acts to verbal or written commands such
as waving goodbye, saluting like soldier, combing their hair, using hammer to fix a nail
• Improve when the examiner shows them what to do or given the object or tool to use
• Limb-kinetic apraxia
Categories of apraxia
• Ideational apraxia
• Ideomotor apraxia
• Limb-kinetic apraxia
• The least understood type
• A higher-order motor deficit in executing motor acts that cannot be explained
by simple motor impairments
• Due to lesion of premotor regions in the frontal lobe (supplementary motor
area)
Blocking (holding) tics
• Is seen occasionally in patients with tics
• A brief interference of social discourse and contact
• No loss of consciousness
• Appear in two situations:
• Accompanying feature of some prolonged tics (intrusion)
• The interruption of activity is due to a positive motor phenomenon  interferes with
other motor activities
• Specific tic phenomenon in the absence of an accompanying obvious motor
or vocal tic (negative)
Cataplexy and Drop Attack
Drop attacks
• Sudden falls with or without loss of consciousness due either to
collapse of postural muscle tone or to abnormal muscle contractions
in the legs
• Can be neurologic and non-neurologic causes
• Neurologic cause : e.g leg weakness, sudden falls in Parkinsonian
syndrome (due to freezing), transient ischemic attack, epilepsy,
myoclonus, startle reactions (hyperekplexia), paroxysmal dyskinesias,
structural central nervous system lesions and hydrocephalus
• Non-neurologic cause: e.g syncope and cardiovascular disease
Cataplexy

• No loss of consciousness, but there is inability to speak during an

attack
• Precipitating trigger: laughter, sudden emotional stimulus
• Muscle tone is flaccid, and remains for many seconds
• One of features of narcolepsy syndrome
 Catatonia, Psychomotor
Depression and Obsessional
Slowness
Catatonia

• A syndrome characterized by catalepsy (abnormal maintenance of

posture or physical attitudes), waxy flexibility (retention of the limbs
for an indefinite period of time in the positions in which they are
placed), negativism, mutism and bizarre mannerisms
• Occurs in Schizophrenia, severe depression, conversion hysteria,
dissociative states, and organic brain disease
Depression
• Characterized by:
• General slowness of movement as well as of thought (psychomotor
retardation)
• Dysphoria
• Anorexia
• Insomnia
• Somatization
• Tearfulness
• There is no Myerson sign, snout reflex, palmomental reflexes
Obsessive-compulsive Disorder
• There is obsessional slowness, cogwheel rigidity, decreased armswing
when walking, decreased spontaneous movement, hypomimia, flexed
posture
• There is no decrementing of either amplitude or speed with repetitive
movements, tremor, micrographia, freezing, or loss of postural
reflexes
• From imaging with fluorodopa PET scan : no abnormality of dopa
uptake, hypermetabolism in orbital, frontal, premotor, and midfrontal
cortex (excessive neural activity)
Freezing
• Transient periods, usually lasting several seconds, in which the motor
act is halted, being stuck in place (motor block)
• Pure akinesia, gait ignition failure
• It can be no apparent attempt to move or the voluntary motor activity
being attempted is halted because agonist and antagonist muscles are
simultaneously and isometrically contracting  preventing normal
execution of voluntary movement
• There is no lack of muscle activity, but analogous to being glued to a
position so that the patient exerts increased effort to overcome the
block and muscle force (isometric) is being exerted.
Types of Freezing phenomena
• Start-hesitation (freezing when gait is initiated)
• Turning-hesitation (freezing when turning)
• Destination-hesitation (freezing when approaching the target)
• Freezing when a physical or temporal “obstacle” is encountered
• Spontaneous sudden transient freezing
• Palilalia or freezing of speech
• “apraxia” of eyelid opening or levator inhibition
• Freezing of limbs
Hesitant Gait
• Cautious gait
• Fear of falling
• The senile gait disorder
• Frontal gait disorder
• Severe disequilibrium because of frontal cortex and deep white
matter lesions (frontal disequilibrium), thalamic and midbrain lesions
(subcortical disequilibrium)
Hypothyroid Slowness
Hypotiroidism
• Decreased of metabolic rate
• Cool temperature
• Bradycardia
• Myxedema
• Loss of hair
• Hoarseness
• Myotonia
• Motor slowness
Rigidity
• Increased muscle tone to passive motion
• Rigidity can be smooth (lead-pipe), or jerky (cogwheel)
• Cogwheel rigidity occurs due to superimposition of tremor rhythm 
more often in Parkinsonisms (nigral lesion)
• Lead-pipe rigidity occurs due to other central nervous system lesions
involving the corpus striatum, cortical basal (ganglionic rigidity),
midbrain (decorticate rigidity), medulla (decerebrate rigidity) and
spinal cord (tetanus)
• Gegenhalten sign  more force applied by the examiner is met with
more resistance by the patient
• Rigidity is more easily treated with levodopa therapy or by
stereotactic thalamotomy or stimulation of the subthalamic nucleus
• Steele-Richardson-Olszewski syndrome  extended neck due to
rigidity (versus dystonia)
• Can occur in the neuroleptic malignant syndrome and sudden
discontinuation of levodopa therapy
Stiff Muscles
• Due to continuos muscle firing without muscle disease and not to
rigidity or spasticity
• Categorized to:
• Continuous muscle fiber activity (neuromyotonia)
• Encephalomyelitis with rigidity
• The stiff-limb syndrome
• The stiff-person syndrome
• Continuous muscle fiber activity (neuromyotonia)
• Syndrome of myotonic failure of muscle relaxation plus myokymia and
fasciculations
• Manifested by continuous muscle activity causing stiffness and cramps
• e.g Isaacs syndrome
• Encephalomyelitis with rigidity
• The stiff-limb syndrome
• The stiff-person syndrome
• Continuous muscle fiber activity (neuromyotonia)
• Encephalomyelitis with rigidity
• Marked rigidity and muscle irritability with increased response to tapping the
muscles along with myoclonus
• Respond to steroid therapy
• The stiff-limb syndrome
• The stiff-person syndrome
• Continuous muscle fiber activity (neuromyotonia)
• Encephalomyelitis with rigidity
• The stiff-limb syndrome
• The stiff-person syndrome
• Autoimmune disease : antibodies against the GABA-synthesizing enzyme, glutamic acid
decarboxylase, antibodies against insulin
• Many somatic muscles are continuously contracting isometrically, resembling “chronic
tetanus”
• Usually forceful and painful, and most frequently involve the trunk and neck musculature
• Respond to benzodiazepines and valproate
• Variant : interstitial neuronitis  encephalomyelitis with rigidity and myoclonus, stiff-baby
syndrome
Dyskinesia
Differential Diagnosis
• Depends primarily on their clinical
features
• Abdominal dyskinesia • Jumpy stumps
• Akathitic movements • Moving toes and fingers
• Ataxia/asynergia/dysmetria • Myoclonus
• Athetosis • Myokymia and synkinesis
• Balism
• Myorhythmia
• Chorea
• Dystonia • Paroxysmal dyskinesias
• Hemifacial spasm • Restless legs
• Hypereksplexia and jumping • Stereotypy
disorders • Tics
• Hypnogenic dyskinesias
• Tremor
Abdominal dyskinesia

• Continuous movements of abdominal wall or sometimes the

diaphragm  belly dancer’s dyskinesia
• Maybe associated with abdominal trauma, segmental abdominal
myoclonus, tardive dyskinesia
Akathitic movement
• Feeling of inner, general restlessness that is reduced or reduced or
relieved by moving about
• The patient, when seated, may caress his or her scalp, cross and
uncross the legs, rock the trunk, squirm, get out of the chair often to
pace back and forth, make noises such as moaning