Movement Disorders 101

Paul W. Brazis MD
Mayo Clinic - Jacksonville

Movement Disorders

Bradykinesia Poverty of movement

Hyperkinesia Excess of movement
Both arise from abnormalities of
basal ganglia

Hyperkinesias result from

disinhibition of thalamus which
results in increased drive of cortex

Chorea
Sudden, brief, spontaneous, involuntary,
purposeless, continuous, irregular, and
unpredictable jerks

Randomly involve the appendicular, facial,

or truncal musculature

Occur at rest or during volitional acts and

interfere with activities of daily living

Cease during sleep

Intensify during stress
Often are camouflaged by the patient

through a superimposed purposeful act

(parakinesia)

Chorea Common Causes

Huntingtons disease
Autosomal dominant
Symptoms often begin insidiously in the

third through the fifth decades

Progressive chorea, dystonia, eye
movement abnormalities, behavioral
changes, and progressive dementia

Benign hereditary chorea

Autosomal dominant
Often presenting with childhood-onset
chorea, no dementia, and little or no
progression

Chorea - Common Causes

Sydenham's chorea (rheumatic chorea or St Vitus dance)
Related to group A beta-hemolytic streptococcal infection
Occurs in childhood and adolescence
Medications (e.g., L-dopa, neuroleptics, metoclopramide,

anticonvulsants, propofol, pemoline, contraceptives,

corticosteroids, lithium, digoxin) and drugs of abuse (e.g.,
cocaine-induced choreoathetosis or "crack dancing"
amphetamines).
Systemic disorders - SLE, Henoch-Schonlein purpura,
sarcoidosis, vasculitis, multiple sclerosis, Behets disease,
hyperthyroidism, hypoparathyroidism, renal failure,
polycythemia vera, hypo- or hypercalcemia, hypo- or
hypernatremia, hypo- or hyperglycemia, mercury poisoning,
carbon monoxide poisoning, antiphospholipid antibody syndrome
In children as a sequelae of cardiac surgery (postpump
chorea), especially associated with prolonged time on the
pump, deep hypothermia, or circulatory arrest
Pregnancy - chorea gravidarum

Athetosis
Slow uncoordinated, writhing involuntary
movements of wide amplitude

Involve predominantly distal appendicular

muscles, although facial and axial muscles
may be involved

May be unilateral or bilateral and often

associated with muscular hypertonia or with
chorea (choreoathetosis)

May be hereditary, congenital, degenerative

(Wilsons disease, perinatal anoxia)

Ballismus

Often confined to one half of body

(hemiballismus)

Flinging, throwing movements of arm

and leg contralateral to lesion of
subthalamic nucleus of Luys

Movement ceases with sleep

Most often hemorrhage or infarct

Dystonia
Characterized by slow, long-sustained

contorting involuntary movements and

postures involving proximal appendicular and
axial muscles

Typically slow and wrapping (athetotic

dystonia) although may demonstrate

superimposed rapid involuntary jerks
(dystonic spasms or myoclonic dystonia)

Dystonic posturing results in disabling and

abnormal attitudes (dystonic postures)
torticollis, lordosis, inversion of hands and
forearms, equinovarus

Dystonia
May be generalized and idiopathic (dystonia

musculorum deformans) or secondary (drug-induced,

Wilsons disease, gangliosidoses, cortical-basal
ganglionic degenerations)
Segmental or focal dystonias often helped by
Botox
Spasmodic torticollis
Oromandibular dystonia
Task-specific
Writers cramp (graphospasm)
Musicians cramp
Spasmodic dysphonia
Blepharospasm
Meige syndrome combination of
blepharospasm with oromandibular dystonia
Orofacial dyskinesia

Therapy With Botulinum Toxin

Torticollis

After Injection

Before Injection
Courtesy of Dr. R. Uitti, Mayo Clinic
Jacksonville

Blepharospasm

Characterized by spontaneous forceful

eye closure that may render patient
functionally blind

Usually idiopathic but may be seen with

Parkinsons disease, torsion dystonia, or
as side effect of neuroleptics

Meige syndrome blepharospasm +

oromandibular dystonia

Blepharospasm
Meige Syndrome
Abnormal,
involuntary,
usually
intermittent but
may be
sustained,
forced eyelid
closure

Writers Cramp

Action
induced
involuntary
muscle
spasms of the
hand or
forearm

Courtesy of Dr. R. Uitti, Mayo Clinic Jacksonville

Writers Cramp

Action
induced
involuntary
muscle
spasms of the
hand or
forearm

Courtesy of Dr. R. Uitti, Mayo Clinic Jacksonville

Spasmodic Dysphonia
Characterized by tremulous, forced voice with
a low tone and volume often associated with
facial grimacing

Three subtypes: Pitch breaks, hoarseness,

limited intensity range, and poor intensity
control are present in all 3
Adductor SD involuntary hyper
adduction of vocal cords results in
strained or strangled voice quality
Abductor SD involuntary abductions
result in intermittent bursts of breathy
phonation
Mixed SD full range of both
characteristics

Spasmodic Dysphonia

Therapy of Dystonias
Behavioral modifications and physical
methods

Pharmacotherapy: anticholinergics,
benzodiazepines, muscle relaxants,
levodopa, and others

Botulinum toxin
Surgical approaches: myectomy, recurrent
laryngeal nerve crush, selective denervation,
cervical cord stimulation, cryothalamatomy

DOPA-Responsive Dystonia
(Segawa Disease)

Autosomal dominant
Presents in childhood
Dystonic movements and postures
Often the dystonias have marked diurnal
variations, being more pronounced in late
afternoon, evening, and night

May have parkinsonism features

Remarkably responsive to low doses of
levodopa

Hemifacial Spasm
Unilateral, involuntary painless arrhythmic
spasms of facial muscles

Often starts in one muscle (e.g.,

orbicularis oculi) and later spreads to all

facial muscles

Usually lesion anywhere along course of

facial nerve (CN VII) tumors, aberrant
vessels, AVM, etc)

May follow Bells palsy

Needs evaluation (MRI and MRA)

Hemifacial Spasm
Disorder of the
facial nerve
(segmental
myoclonus)

Initially, unilateral
clonic twitching of
either the upper or
lower facial
musculature

Later on, long

lasting sustained
tonic muscle
contractions

Courtesy of Dr. D. Bennett, University of Nebraska

Myoclonus
Movement disorder characterized by

unexpected, brisk, shock-like involuntary

repetitive synchronous or asynchronous
contractions of a muscle or group of axial or
appendicular muscles

May be focal, multifocal, or generalized

May be spontaneous, occur with attempted

movement (action myoclonus e.g., due to

cerebral hypoxia Lance-Adams
syndrome), or precipitated by sudden
stimulus

Myoclonus
Four major etiologies:
Physiologic
Sleep induced
Essential Hereditary or sporadic
Epileptic patients with epilepsy
Symptomatic Multiple etiologies
Basal ganglia degenerative diseases
Storage diseases
Encephalitis
Metabolic disease (hepatic, renal,

etc)
Dementing illnesses (J-C disease)

Palatal Myoclonus (Palatal Tremor)

Rhythmic contraction affecting palatal

and pharyngeal structures often with
synchronous movements of ocular
muscles, diaphragm, head, and neck

Persists in sleep
Lesion (usually vascular, trauma, tumor,

or demyelinating) of pathway between

red nucleus, inferior olivary nucleus, and
cerebellar dentate nucleus (GuillainMollaret triangle)

Tics

Sudden rapid usually stereotyped,

predominantly clonic hyperkinesias

May be will fully suppressed for short

periods and disappear in sleep

Usually start around eyes or mouth

but may spread to neck or shoulders
or become generalized

Gilles de la Tourette syndrome

Begins in childhood
Often behavioral problems
Obsessive-compulsive disorder
Lack of impulse control
Attention deficit disorder
Multiple or single motor tics
Often associated with vocalizations (grunts, snorts,

sniffs, bark, throat clearing, spitting, coughing)

Occasionally associated with more complicated motor
activity
Coprolalia (obscene language), copropraxia
(obscene gesturing), or echolalia (tendency to
repeat words or sentences recently spoken to
patient) in <50%
Echopraxia (imitation of acts), jumping, kicking

Tremor
Definition - rhythmic, oscillatory
movement about a joint produced by
alternating or synchronous contraction of
reciprocally innervated agonist/antagonist
muscles

The most common form of involuntary

movement, but only a small fraction who
shake seek medical attention

Classification of Tremor
Postural tremor - occurs while maintaining body part
in position against gravity e.g., Essential Tremor

Kinetic tremor - occurs during voluntary movement

e.g. Cerebellar disease

Rest tremor - occurs when body part not voluntarily

activated and is supported against gravity e.g.
Parkinson disease

Task-specific tremor - kinetic tremor that appears or

is exacerbated during specific skilled movements

Orthostatic tremor - form of task-specific tremor very rapid (14Hz) tremor of lower extremities that
occurs while standing in place

ET - Clinical Features

Typically mixed postural and kinetic

tremor

Often most evident at end of goaldirected movements

4-12 Hz
Upper extremities and head more than
legs and voice

Parkinsonism Tremor
Most often at rest
Tremor of face, tongue, and jaw likely due to
PD while head tremor more typical of ET or
cervical dystonia

PD may have re-emergent tremor - when

hands raised to assume postural position,
there will be brief period with no tremor
followed by crescendo increase in tremor until
appears typical rest tremor

Cerebellar Tremor
Usually intention tremor = kinetic tremor
markedly augmented when intended target
approached

Usually accompanying signs of cerebellar

dysfunction - ataxia, etc

Rubral or midbrain tremor - mixed rest,

postural, and intention tremor accompanied
by other signs of brainstem or cerebellar
dysfunction

Wilsons Disease
Autosomal recessive
Triad
Liver dysfunction
Behavioral abnormalities cognitive impairment,

psychosis, dementia
Abnormal movements

Often wing-beating tremor

Also postural or kinetic tremor, dysarthria, drooling,
clumsiness, gait changes, parkinsonism, dystonia,
ataxia

Oph abnormalities Kayser-Fleischer rings,

sunflower cataracts, slow saccadic eye movements

Orthostatic Tremor
High frequency (20 Hz) postural tremor in
torso and lower limbs - Shaky legs
syndrome

Also may occur in upper limbs

Characterized by subjective feeling of
unsteadiness while standing

Suppressed by walking
Rarely fall and do not have problems sitting or
lying

Treatment of ET - Alcohol
Effective in reducing tremor in 3/4 patients
Response dramatic but transient - tremor
returns in about 2 hrs sometimes with
rebound augmentation

Single glass of wine or other drink prior to

social function may be suggested for
temporary amelioration

Treatment of ET

Beta blockers
Primidone (Mysoline)
Gabapentin (Neurontin)
Topiramate (Topamax)

Treatment of ET Other Agents

Clonazepam (Klonopin)
Alprazolam (Xanax)
Levetiracetam (Keppra)
Miratazapine (Remeron)
Low dose theophylline
Trazadone
Clonidine
Zonisamide (Zonogram)
Nimodipine (Nimotop)

Treatment of ET - Surgery
Electrical stimulation of VIM nucleus of
thalamus (VIM STIM)

Very effective in patients with severe,

medication resistant ET

Adverse effects - paresthesias, dysarthria,

gait disorder

Treats tremor contralateral to site of

stimulation

Bilateral stimulation - increased risk of

dysarthria and gait disorders