D(ix). APPROACH TO INVOLUNTARY MOVEMENTS

MOVEMENT DISORDERS

Dyskinesia is abnormal uncontrolled movement and is a common symptom of many movement

disorders [Flowcharts 6D(ix).1 and 6D(ix).2].

Movement disorders disrupt motor function by:

Abnormal, involuntary, unwanted movements (hyperkinetic movement disorders).

Curtailing (restricting) the amount of normal free flowing, fluid movement (hypokinetic movement

disorders).

Flowchart 6D(ix).1: Categorization of movement disorders.

Flowchart 6D(ix).2: Systematic approach to movement disorders.

Site of Lesion

Parkinsonism → Contralateral substantia nigra

Unilateral hemiballismus → contralateral subthalamic nucleus

Chronic chorea → Caudate nucleus/putamen

Athetosis, dystonia → Contralateral putamen or thalamus

Myoclonus → Cerebellar cortex/thalamus

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Rhythmic palatal/facial myoclonus → Central tegmental tract, inferior olivary nucleus, olivodentate

fibers.

TREMOR

Tremor: Series of involuntary, relatively rhythmic, purposeless, oscillatory movements due to intermittent

muscle contractions:

Simple tremor involves only a single muscle group

Compound tremor involves several muscle groups

Several elements in combination

Resulting in a series of complex movements

May be unilateral or bilateral

Most commonly involves distal parts of the extremities—fingers or hands

May also affect the arms, feet, legs, tongue, eyelids, jaw, and head

May occasionally involve the entire body

Rate may be slow, medium, or fast

Slow: Oscillations of 3 to 5 Hz

Rapid: Oscillations of 10 to 20 Hz

Amplitude may be fine, coarse, or medium

The relationship to rest or activity is the basis for classification into two primary tremor types:

Resting

Action

Resting (Static)

Tremors are present mainly during relaxation (e.g. with the hands in the lap)

Attenuate when the part is used

Rest tremor is seen primarily in PD and other Parkinsonian syndromes

Action Tremors

Postural tremors

become evident when

the limbs are:

Maintained in an

antigravity position

(e.g. arms

outstretched)

Types of postural

tremor:

Enhanced

physiological tremor

(EPT)

Essential tremor

(ET)

Kinetic tremor: Appears when making a voluntary movement

May occur at the beginning, during or at the end of the movement. For example, intention (terminal) tremor

seen primarily in cerebellar disease

Task specific tremor: Occurs when

performing highly skilled, goal-oriented tasks.

For example, while writing or speaking

CHOREA

Characterized by involuntary, irregular, purposeless, random, nonrhythmic hyperkinesias.

Movements are spontaneous, abrupt, brief, rapid, jerky, and unsustained.

Movements are actually random and aimless:

Rather than disrupting a voluntary task, it appears as if fragments of movements intrude; in some

cases, there is loss of motor tone, known as “motor impersistence”, which appears due to

lapses in the ability to perform desired action.

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When asked to hold the hands outstretched, there may be constant random movements of individual

fingers (piano-playing movements).

If the patient holds the examiner’s finger in her fist, there are constant twitches of individual fingers

(milkmaid grip):

“Jack in the box” tongue/ harlequin’s tongue: Patient is unable to maintain tongue in protruded

state and the tongue moves in and out.

Blink rate is increased.

Causes

Hereditary: Huntington’s disease, benign chorea

Drugs: Antiparkinsonian drugs, oral contraceptives

Toxin: Alcohol, carbon monoxide poisoning

Infections: Sydenham’s chorea, encephalitis

Metabolic: Hyperthyroidism, hypocalcemia

Immunological: SLE, polyarteritis nodosa

Vascular

Pregnancy (Chorea gravidarum)

ATHETOSIS

Involuntary, irregular, coarse, somewhat rhythmic, and writhing or squirming in character (twisting).

Hyperkinesias are slower, more sustained, and larger in amplitude than those in chorea.

May involve the extremities, face, neck, and trunk.

In the extremities, they affect mainly the distal portions, the fingers, hands, and toes:

Affected limbs are in constant motion (athetosis means “without fixed position”)

Choreoathetosis refers to movements that lie between chorea and athetosis in rate and

rhythmicity, and may represent a transitional form.

Causes

Cerebral palsy

Congenital due to perinatal injury to the basal ganglia.

HEMIBALLISMUS

Dramatic neurologic syndrome of wild, flinging (forceful), incessant (uninterrupted or continuous)

movements that occur on one side of the body.

Due to infarction or hemorrhage in the region of the contralateral subthalamic nucleus.

More rapid and forceful

Involve the proximal portions of the extremities. When fully developed, there are continuous, violent, swinging, flinging, rolling, throwing, flailing

(thrashing) movements of the involved extremities.

They are usually unilateral, and involve one entire half of the body.

Rarely, they are bilateral (biballismus or paraballismus) or involve a single extremity (monoballismus).

MYOCLONUS

Single or repetitive, abrupt, brief, rapid, lightning-like, jerky, arrhythmic, asynergic, involuntary

contractions, involving portions of muscles, entire muscles, or groups of muscles.

Seen principally in the muscles of the extremities and trunk, but the involvement is often multifocal,

diffuse, or widespread.

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May involve the facial muscles, jaws, tongue, pharynx, and larynx.

Myoclonus may appear symmetrically on both sides. Such synchrony may be an attribute unique to

myoclonus.

Myoclonus has been classified in numerous ways including the following:

Positive versus negative;

Epileptic versus nonepileptic;

Stimulus sensitive (reflex) versus spontaneous;

Rhythmic versus arrhythmic;

Anatomically (peripheral, spinal, segmental, brainstem, or cortical)

By etiology (physiologic, essential, epileptic, and symptomatic)

Encephalitis

Juvenile myoclonic epilepsy (JME, Janz syndrome)

Drug overdose

Hypnic jerks (appear during the process of falling asleep)

Hiccup

Creutzfeldt–Jakob disease

Subacute sclerosing panencephalitis (SSPE)

Anoxic encephalopathy (Lance-Adams syndrome)

TIC

A “tic” is an involuntary movement or vocalization that is usually sudden onset, brief, repetitive,

stereotyped but nonrhythmical in character, can be suppressed.

Types

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/phonic tics are associated with sound

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

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.

Gilles de la Tourette syndrome—associated with chronic motor and phonic tics.

DYSTONIA

Refers to a syndrome of involuntary sustained or spasmodic muscle contractions involving

cocontraction 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.

Partial or focal Generalized

Spasmodic torticollis

Blepharospasm

Oromandibular dystonia Writers cramp

Hemiplegic dystonia after stroke

Dystonia musculorum deformans (idiopathic torsion dystonia)

Dopamine responsive dystonia: In childhood and generally involves the legs only.

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

Symptomatic dystonia (after encephalitis, Wilsons disease)

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Blepharospasm and 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.

MYOKYMIA

Myokymia, a form of involuntary muscular movement, usually can be visualized on the skin as

vermicular or continuous rippling movements.

AKATHISIA

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.

RESTLESS LEGS SYNDROME/“EKBOM’S SYNDROME”

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, and iron deficiency.

SYNKINESIS/MIRROR MOVEMENTS

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

voluntary movements of the other side.

FASCICULATIONS

Fasciculations are visible, fine and fast, sometimes vermicular contractions of fine muscle fibers that

occur spontaneously and intermittently but usually do not generate sufficient force to move a limb.

Described as verminosis, because they look like worms moving below the dermis.

Involuntary contraction of the muscle fibers innervated by a motor unit.

Causes of Fasciculations

Fasciculations in healthy

subjects

Coffee; exhaustive physical activity/fatigue; stress; benign fasciculations

Fasciculations associated with movement disorders

Spinocerebellar degeneration-type 3; spinocerebellar degeneration-type 36; Parkinsonism

(multiple system atrophy, ALS-plus syndromes)

Motor neuron diseases Amyotrophic lateral sclerosis; progressive spinal muscular atrophies; benign monomelic

amyotrophy; postpolio syndrome; Kennedy disease

Systemic diseases Hyperthyroidism; hypophosphatemia, calcium disorders secondary to hyperparathyroidism,

paraneoplastic myopathy

Drugs and/or intoxications by

heavy metals pollutants

Organophosphorus poisoning; neostigmine; corticosteroids; succinylcholine; elemental mercury intoxication; atropine, lithium, nortriptyline; flunarizine; isoniazid

D(x). MENINGEAL SIGNS, SKULL, AND SPINE

SIGNS OF MENINGEAL IRRITATION

Nuchal Rigidity/Meningeal Stiffness

Meningeal tightness is a contracture of the paravertebral muscles, a defense against the secondary pain

stemming from inflammation of the meninges.

Painful and permanent, it sometimes presents with the subject lying down, curled up with his or her

back to the light, head back, and extremities half-bent. All attempts to flex the head provoke

insurmountable and painful resistance. There is extreme neck stiffness; rotational and side-to-side

movements are possible but aggravate the headache [Fig. 6D(x).1].

Fig. 6D(x).1: Examination of neck stiffness.

In Kernig’s sign, patient is kept in supine position, hip and knee are flexed to a right angle, and then

knee is slowly extended by the examiner. The appearance of resistance or pain during extension of the

patient’s knees beyond 135° constitutes a positive Kernig’s sign [Figs. 6D(x).2 and 6D(x).3].

Brudzinski’s Sign

Josef Brudzinski described 4 maneuvers for the clinical diagnosis of meningitis: The cheek sign,

symphyseal sign, Brudzinski’s leg sign/reflex, and Brudzinski’s neck sign.

1 The cheek sign A positive cheek sign is elicited by applying pressure on both cheeks inferior to the zygomatic arch that

leads to spontaneous flexion of the forearm and arm

2 Symphyseal

sign [Fig.

6D(x).4]

A positive symphyseal sign occurs when pressure applied to the pubic symphysis elicits a reflex hip and

knee flexion and abduction of the leg

3 Brudzinski’s

leg sign/reflex

[Fig. 6D(x).5]

Brudzinski’s contralateral reflex sign consists of reflex flexion of a lower extremity after passive flexion of

the opposite extremity

4 Brudzinski’s

neck sign

[Figs. 6D(x).6

and 6D(x).7]

Brudzinski’s neck sign is performed with the patient in the supine position. The examiner keeps one hand

behind the patient’s head and the other on chest in order to prevent the patient from rising. Reflex flexion of

the patient’s hips and knees after passive flexion of the neck constitutes a positive Brudzinski’s sign

Fig. 6D(x).2: Demonstration of Kernig’s sign.

Fig. 6D(x).3: Illustration of Kernig’s sign.

Fig. 6D(x).4: Symphyseal sign.

Fig. 6D(x).7: Brudzinski’s neck sign.

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