INVOLUNTARY MOTOR MOVEMENTS

Prepared By : Khaled Hani Alkhodari

Supervisor: Dr. Iyad M Husain, PhD Neurology

INVOLUNTARY MOVEMENTS

Imagine if parts of your body moved when you didn't want them to.

Dyskinesia is abnormal uncontrolled movement and is a

common symptom of many movement disorders.

INVOLUNTARY MOVEMENTS

Are neurological conditions that affect the speed, fluency, quality, and ease of movement.

Are clinically and pathologically heterogeneous ,and are characterized by impairment of planning, control or execution of movement .

Almost all movement disorders are worsened with stress, fatigue, anxiety or concomitant illness (e.g. Pneumonia, UTI, etc.). As a rule, movement disorders

show significant or complete relief during sleep.

CLASSIFICATION

CLASSIFICATION OF HYPERKINESIA

TREMORS

PHYSIOLOGICAL

Tremor is an unintentional, rhythmic muscle movement involving to-and-fro movements (oscillations) of one or more parts of the body.

Present in almost everyone and a normal finding that usually cannot be seen unless worsened in certain situations that include:

Anxiety, fear, physical exhaustion, hypoglycemia, hyperthyroidism, alcohol withdrawal

"ESSENTIAL" TREMOR (ET)- POSTURAL

Most common involuntary movement disorder, typified by a rapid postural tremor most often of the upper extremities.

Progressive, may appear at anytime of life but most commonly >70 years.

Mainly a postural tremor (i.e Tremor when skeletal muscle holding in on position against gravity.) but if worsened, will occur at rest

Commonly bilateral and symmetric.

Classically affects head and/or speech.

Classically, tremor decreases with EtOH, worsens with stress.

Neurological exam is otherwise normal.

Differentiate from Parkinson by noting absence of resting tremor, rigidity, bradykinesia, etc.

CEREBELLAR TREMOR (INTENTION TREMOR)

Slow action tremor that most commonly occurs with purposeful movement (such as in doing finger to nose test)

Usually associated with other cerebellar findings

Seen in any process that causes damage to the cerebrellum (stroke, tumor, multiple sclerosis, chronic alcohol abuse, certain medications)

Unlike the cerebrum, cerebellar disease causes problems on the SAME SIDE of the body as the the cerebellar lesion

Titubation refers to tremor of the head (and sometimes trunk) due to cerebellar disease

PARKINSONIAN TREMOR

Most common cause of a resting tremor that usually involves the distal muscles (e.g. pill rolling tremor of the hand)

May decrease with voluntary activity (e.g. raising arm) and increase with mental activity (e.g. asking patient to do math) and increase with anxiety or emotional excitement

causes:

idiopathic parkinson's disease: usually starts in one hand then spreads to other limbs

Drugs: antipsychotic agents, metoclopramide, prochloperazine

Encephalitis (HIV/AIDS, neurosyphilis, PML, toxoplasmosis)

Chronic head trauma.

Other resting tremors include Wilson's Disease and severe essential tremor (ET is usually postural but when servere can be seen at rest or even with movement)

TREMORS

TREMORS

TREMORS

CHOREA

A state of excessive, spontaneous movements, irregularly timed, non-

repetitive, randomly distributed and abrupt in character.

These movements may vary in severity from restlessness with mild intermittent to unstable dance-like gait to a continuous flow of disabling, violent movements.

Chorea involves both proximal and distal muscles.

CAUSES OF CHOREA

Hereditary: Huntington’s disease, benign chorea

Drugs: Antiparkinsonian drugs, oral contraceptives

Toxin: alcohol, carbon monoxide poisoning

Infections: Sydenham’s chorea, encephalitis

Metabolic: hyperthyroidism, hypocalcaemia

Immunological: SLE, polyarteritis nodosa

Vascular: Hemibalism

CHOREAHUNTIGTON DISEASE

Definition: is an autosomal dominant disease that characterized by progressive chorea and dementia

Etiology: pathology in gene called “huntingtin” located in chromosome 4p 16.3.

Pathology: reduction in the striatum of γ-Aminobutyric acid-(GABA) and acetylcholine

Age: 30-40 years

CHOREAHUNTIGTON DISEASE

Symptoms and signs:

motor impersistence.

milkmaid's grip.

harlequin's tongue.

Patients often drop objects involuntarily.

Trials to mask it by semipurposeful movements

rigidity and dementia

Diagnosis: MRI, PET scaning. Genetic testing is diagnostic

Treatment: phenothiazine, haloperidole.

CHOREAHUNTIGTON DISEASE

CHOREA

SYDENHAM CHOREA

Its one of major criteria of rheumatic chorea, postinfective chorea

Age: 5-15 years

Movements are diffuse and often associated with behavioral changes

Pathology: necrotising arteritis in thalamus, caudate nucleus and putamen

Diagnosis: elevated ESR and ASOT (antistreptolysin titre)

Treatment: aspirin, penicillin, sedation

can occur in:

A tumor or an infarct in the striatum (caudate or putamen) can cause acute unilateral chorea (hemichorea).

Sydenham chorea and chorea due to infarcts of the caudate nucleus often lessen over time without treatment.

CHOREASYDENHAM CHOREA

CHOREA GRAVIDARUM

Occurs during pregnancy, often in patients who have had rheumatic fever.

Chorea usually begins during the 1st trimester and resolves spontaneously

at or after delivery.

If treatment before delivery is necessary because chorea is severe,

barbiturates are indicated because they have fewer fetal risks than other

drugs used to manage chorea.

Rarely, a similar disorder occurs in women taking oral contraceptive

OTHER CHOREAS

Neuroacanthocytosis is a rare, recessive, relentlessly progressive disorder typified by chorea coupled with erythrocyte abnormalities and possibly dystonia, tics, seizures, polyneuropathy, and self mutilation. May present at any time in life.

Similar presentation is noted in McLeod syndrome - an X-linked disorder associated with reactivity to Kell antigens, typically older patients.

Paroxysmal chorea has been described in hyper- and hypoglycemia, vascular diseases, and infections.

Benign senile chorea & benign inherited chorea of childhood have been described but are controversial. It is important to rule out HD.

SLE and less commonly other autoimmune disorders may cause chorea.

ATHETOSIS (SLOW CHOREA), CHOREOATHETOSIS

Athetosis

Is nonrhythmic, slow, writhing (ie, squirming, twisting, or

snakelike), sinuous movements

Predominantly in distal muscles.

Often alternating with postures of the proximal limbs.

May be due to ↑ dopamine

Choreoathetosis:

Bit more rapid than the usual athetosis, slower than the usual chorea, or

A mingling of chorea and athetosis within the same patient at different times or in different limbs

The only difference between chorea, choreoathetosis, and athetosis is the speed of movement

ATHETOSIS

HEMIBALLISMUS & BALLISMUS (SEVERE FORM OF CHOREA)

Continuous, violent, coordinated involuntary activity involving the axial and proximal appendicular musculature such that the limbs are flung about.

Vascular type of chorea, due to stroke of corpus luisii in subthalamus (posterior cerebral artery territory)

Hemiballismus is unilateral

Ballismus movement occurs bilaterally.

HEMIBALLISMUS & BALLISMUS

TIC

A “tic” is an involuntary movement or vocalization that is usually sudden onset, brief, repetitive, stereotyped but non rhythmical in character, can be suppressed.

TICTYPES

Motor tics are associated with movements. Categorized as simple or complex. Simple motor tics involve only a few muscles usually restricted to a specific body part.

Examples of simple motor tics include: eye blinking, shoulder shrugging, facial grimacing, neck stretching, mouth movements, jaw clenching and spitting.

Vocal tics are associated with sound

Simple vocal tics consist of sounds that do not form words, such as: throat clearing, grunting, coughing, and sniffing

TICSSUBTYPES

Transient Simple: common in children

Chronic: any age

Persistent Simple or Multiple: onset before 15 yr age, resolve in

adults

Chronic Multiple: Tourette’s syndrome

TIC

TICSSOME DEFINITIONS

Common complex vocal tics include:

Repeating words or phrases out of context,

Coprolalia : use of socially unacceptable words, frequently obscene.

Palilalia : repeating one's own sounds or words.

Echolalia : repeating the last-heard sound, word, or phrase.

Echokinesis : imitation of someone else's movements.

TICSGILLES DE LA / TOURETTE DISORDER

Cause: unknown

Common in male in childhood or adolescence.

Treatment: difficult but patients may respond to clonidine, benzodiazepines, halidol, risperdal, clozapine,

Surgery for drug-resistant tics, but not shown effective in Tourette’s

TICS

TICS

MYOCLONUS

Definition: These are sudden, rabid,

involuntary, jerky, ‘shock-like’

movement of a single muscle or a

group of muscles.

Epileptic myoclonus

Seizures without significant

encephalopathy

Cortical origin

Benign essential myoclonus

Nocturnal myoclonus

Paramyoclonus multiplex

Segmental myoclonus

Brainstem

Spinal

NON EPILEPTIC MYOCLONUSENCEPHALOPATHY WITHOUT SIGNIFICANT EPILEPSY

Basal ganglia degenerations

Wilson’s

Huntingtons

Dementias

Alzheimer’s

CJD

Malabsorption

Hypoxia

Spinocerebellar degeneration

Focal

Tumour

Stroke

Trauma

Metabolic

Hepatic failure

Renal failure

Paraneoplastic

Toxic

DYSTONIA (ALTERED MUSCLE TONE)

Refers to a syndrome of involuntary sustained or spasmodic muscle contractions involving co-contraction of the agonist and the antagonist.

The movements are usually slow and sustained, and they often occur in a repetitive and patterned manner

They can be unpredictable and fluctuate.

DYSTONIATYPES

Generalized:

Dystonia muscularum deformans (idiopathic torsion dystonia):Dystonia affecting gait and

posture, common in childhood

Dopamine responsive Dystonia: The disorder presents in childhood and generally

involves the legs only.

Drug-induced dystonia (metoclopramide,phenothiazine, haloperidol, largactyl)

Symptomatic dystonia (after encephalitis, Wilson’s disease)

DYSTONIATYPES

Partial or focal:

Spasmodic torticollis

Blepharospasm

Oromandibular dystonia

Writer’s cramp

Hemiplegic dystonia after stroke

SPASMODIC TORTICOLLIS

Unilateral deviation of the head

Dystonic contraction of left

sternomastoid muscle produces head

turning to the right

Treatment: anticholinergics and

local botolinum toxin injection.

BLEPHAROSPASM & OROMANDIBULAR DYSTONIA

Involuntary prolonged tight eye closure (blepharospasm) is associated with dystonia of mouth, tongue or jaw muscles (jaw clenching and tongue protrusion)

WRITER'S CRAMP = MOGIGRAPHIA = SCRIVENER'S PALSY

Symptoms usually appear when a person is trying to do a task that requires fine motor movements such as writing or playing a musical instrument.

The symptoms may be isolated to only those tasks or affect the muscles in a more general way and spread to affect many tasks.

Common symptoms include, for example, excessive gripping of a pen or utensil, flexing of the wrist, elevation of the elbow, and occasional extension of a finger or fingers causing the utensil to fall from the hand.

WRITER'S CRAMP = MOGIGRAPHIA = SCRIVENER'S PALSY

MYOKYMIA

Myokymia, a form of involuntary muscular movement, usually can be visualized on the skin as vermicular or continuous rippling movements.

The word myokymia was used first more than 100 years ago, when Schultzedescribed continuous, slow, undulating muscular contractions in small muscles of hands and feet

MYOKYMIA

AKATHESIA

Akathisia is a movement disorder characterized by a feeling of inner restlessness and a compelling need to be in constant motion, as well as by actions such as:

rocking while standing or sitting.

lifting the feet as if marching on the spot.

crossing and uncrossing the legs while sitting.

People with akathisia are unable to sit or keep still, complain of restlessness, fidget, rock from foot to foot, and pace.

Akathisia is frequently associated with the use of dopamine receptor

antagonist antipsychotic drugs

NEUROLEPTIC MALIGNANT SYNDROME

Clinical features:

Drowsiness, fever, tremor and rigidity

Muscle necrosis (rhabdomyolisis), and

renal failure.

Elevation of serum creatine kinase

activity and sometimes

Myoglobinuria

Treatment:

Dopamine receptor agonist

(Bromocriptine), Baclofen

Muscle relaxants (Dantrolene)

Associated with prescribing dopamine antagonist and long acting neuroleptic therapy

AKATHESIA

RESTLESS LEGS SYNDROME“EKBOM’S SYNDROME”

Restless legs syndrome refers to

symptoms of spontaneous, continuous

leg movements associated with

paresthesia.

These sensations occur only at the rest

and relieved by movement.

Causes:

Familial

Lumbar root disease

Polyneuropathy

Renal failure

Iron deficiency

Psychiatric

Treatment: Dopaminergic agent,

clonozepam, gabapentine.

ASSOCIATED MOVEMENTS “MIRROR MOVEMENTS”

Mirror movements are characterized by involuntary movements on one side of the body mirroring voluntary movements of the other side.

This mostly affects the top half of the body but can also affect the bottom half of the body.

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