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movement disorders
1.
2. Movement Disorders
0 Movement disorders (MDs) typically result from
abnormalities of the extrapyramidal system.
0 The core structures of the extrapyramidal system include
the caudate nucleus, globus pallidus, substantia nigra, red
nucleus, thalamic nucleus, subthalamic nucleus and their
connections to the cerebral cortex, brain stem, and
cerebellum, with the final output being pyramidal tract.
0 The main transmitter substances are dopamine,
acetylcholine, GABA and glutamate.
0 MDs can be hyperkinetic, hypokinetic, or dyskinetic.
3. Pattern Recognition
0 Tremor: rhythmic involuntary oscillating of a body part.
Usually involves distal extremities. Tremor is subclassified
according to position of the limb (rest, action, intention,
postural).
0 Chorea: rapid, continuous, irregular, brief, purposeless
movements, often involve distal limb.
0 Athetosis: slow, irregular undulating, writhing-type
movement, often associated with chorea.
0 Tics: stereotyped, irregular simple or complex movements.
0 Dystonia: sustained, abnormal contraction of a group of
muscles, resulting in abnormal posture of the limb.
4. 0 • Ballismus: violent, flinging movements often involving
proximal limb, usually unilateral (hemiballismus), but can be
bilateral lower or upper extremities because of contralateral
subthalamic nucleus lesion.
0 • Myoclonus: sudden, irregular shock-like contraction of the
muscle or group of muscles.
0 • Tardive dyskinesia: stereotyped movements often involving
the facial and oral muscles, manifesting as tongue protrusion,
chewing, lip smacking, and facial grimacing. The trunk and
extremities are often also involved. This condition is seen after
chronic use of dopamine-blocking antipsychotic or antiemetic
drugs.
0 • Rigidity: increased muscle tone throughout the passive range
of motion of the limb. When there is coexistence of tremor, the
examiner detects a ratchet like resistance, referred to as
“cogwheel rigidity.”
6. 1. Rest tremor.
0 Tremor that occurs in a body part that is not voluntarily activated
and is completely supported against gravity.
0 Rest tremor increases with mental stress (counting backwards),
or when movements of another body part are performed
(especially walking). It is mostly found in Parkinson's disease, but
also in other parkinsonian syndromes, including drug-induced
parkinsonism.
0 Its presence indicates dysfunction of the nigrostriatal dopamine
pathway or its efferent projections to basal ganglia
thalamocortical circuits.
7. 2. Action tremor.
0 Postural tremor. Tremor present while voluntarily maintaining a
position against gravity. This tremor is usually documented by
having the patient outstretch the arms.
0 Simple-kinetic tremor. Tremor that occurs during voluntary action
that is not target-directed.
0 Intention tremor. Action tremor in which amplitude increases
substantially during the pursuit of a target or goal. Its presence
suggests a disturbance of the cerebellum or its afferent/efferent
pathways.
0 Task-specific kinetic tremor. Tremor occurring during specific
activities, such as the primary writing tremor and occupational
tremors. These tremors are often associated with dystonia.
12. Treatment (ET)
a) β-Blockers: propranolol (Inderal) studied most extensively
i) Inderal LA (long-acting) may be started at 40 mg daily, and increased in small increments to
optimal doses 240-320 mg/day, as tolerated and if needed
ii) Tremor reduction up to 60%
iii) Contraindicated if asthma, diabetes mellitus, marked bradycardia, second- or third-degree
atrioventricular block, heart failure
b) Primidone (Mysoline)
i) As effective as propranolol but more adverse effects
ii) Converted to phenylethylmalonamide and phenobarbital, the latter with longer half-life
iii) To minimize the adverse effects, start with small doses of 50 mg/day and with subsequent
titration, may be divided as 3-times-daily regimen
iv) May be titrated up to maximum of 750 mg/day (250 mg 3 times daily)
c) Second-line agents: carbonic anhydrase inhibitors
(e.g., methazolamide), gabapentin, benzodiazepines
d) Surgical treatment
i) Thalamotomy
ii) Thalamic stimulation: high success rate (tremor reduced in up to 90% and abolished in 50%
of cases); bilateral procedures have lower complication rates than thalamotomy
14. Chorea
0 a. Unsustained, nonstereotypic movements with variable
speed and direction, may be rapid or brief, may flow from
one extremity to the next.
0 b. Choreoathetosis: chorea occurring concurrently with
athetosis or dystonic movements
0 c. Ballismus (ballism): large-amplitude random movements,
most prominent at the proximal limbs
0 d. Hemichorea or hemiballismus: refers to hemi body
distribution of the movement disorder
15. Clinical features
a. Mild chorea may appear as restlessness or uneasy
movements
b. Parakinesia: chorea that may seem semipurposeful and
may “blend into” purposeful movements
c. Inability to maintain tone: maintaining handgrip
(“milkmaid’s grip”) or maintaining protrusion of tongue
(“flycatcher’s tongue”)
16.
17. Treatment and management
a) Dopamine receptor blockers and dopamine-depleting agents for
chorea, psychosis, and behavioral symptoms
i) Typical antipsychotics are not well tolerated and can induce tardive dyskinesias
ii) Atypical antipsychotics better tolerated and do not induce tardive dyskinesias
iii) Atypical antipsychotic—clozapine: effective for psychosis but not for chorea;
disadvantage is high cost and risk of agranulocytosis
iv) Atypical antipsychotic—olanzapine: effective for both chorea and psychiatric
symptoms (may increase stroke risk in elderly)
b) Riluzole: corticostriatal glutamate release inhibitor; may potentially improve
chorea
c) Remacemide: glutamate/NMDA receptor antagonist, may marginally improve
chorea .
d) Coenzyme Q10: mitochondrial complex I enhancer, possible decline in
measure of disability (not statistically significant), possible benefit on behavioral
symptoms
e) Anticonvulsants (e.g., valproate): may be helpful for chorea and psychiatric
and behavioral symptoms, including aggression and irritability
19. Dystonia
0 syndrome of sustained muscle contractions producing
abnormal postures or repetitive movements involving
different distributions
0Classification according to distribution:
1) Focal: one body region
2) Multifocal: at least two noncontiguous body regions
3) Hemidystonia: at least two ipsilateral body regions
4) Segmental: adjacent body regions
5) Generalized: involvement of both lower extremities or one
lower extremity and trunk and another body region
20. 0 A characteristic feature of dystonic movements is that they
may be diminished by sensory tricks such as gently
touching the affected body part (geste antagoniste).
0 Dystonic movements tend to be exacerbated by
fatigue, stress, and emotional states and may be suppressed
by relaxation and sleep.
0 Dystonia typically worsens during voluntary movement. At
the time of onset, dystonia may only be present during a
specific movement (action dystonia).
0 With progression, however, the dystonia may emerge with
other movements and eventually may be present at rest.
21. Causes
1) Idiopathic torsion dystonia is a primary dystonia and may
occur as a familial condition. The spectrum of the disorder is
broad and includes focal (blepharospasm, torticollis,
spasmodic dysphonia, writer's cramp), segmental, and
generalized forms. The gene for the autosomal dominant
familial form has been located on chromosome 9q,
2) Secondary causes of dystonia include metabolic disorders
(e.g., Wilson's disease) degenerative diseases (parkinson's
disease, progressive supranuclear palsy, corticobasal
ganglionic degeneration, Huntington's disease, multiple
system atrophy), and nondegenerative central nervous
system disorders (anoxia, head or peripheral trauma, prior
stroke, multiple sclerosis, or drug-induced)
22. Treatment
1)Botulinum toxin A (BTX)
2) Medical treatment
0 a) Dopaminergic (Sinemet)
0 b) Dopaminergic antagonists
0 c) Dopamine-depleting agents
0 d) Anticholinergic (e.g., trihexyphenidyl, benztropine): may be effective in up to 40% of
patients
0 e) Baclofen: may be effective in up to 20%
0 f) Clonazepam: may be effective in up to 15%
0 g) Anticonvulsants (e.g., carbamazepine, gabapentin)
3) Surgical treatment
0 a) Peripheral denervation procedures: bilateral anterior cervical rhizotomy,
microvascular decompression, ramisectomy, peripheral nerve lysis, myectomy
0 b) Deep brain stimulation
0 c) Unilateral thalamotomy
23. ACUTE DRUG-INDUCED
DYSTONIA
Acute drug-induced dystonia presents with abnormal
tongue or jaw postures and neck dystonia, occurring
within the first 3 days of starting a neuroleptic.
The treatment is benzotropin (Cogentin), 2 mg
intravenously or intramuscularly, or 50 mg
diphentrydramine (Benadryl) intravenously.
25. 0 Myoclonus is a sudden Iightning-like movement produced by
abrupt and brief muscle contraction.
0 The four etiologic categories are essential, physiological,
epileptic, and symptomatic.
0 Essential myoclonus is a non physiological variety that occurs
in isolation without evidence of other neurologic symptoms or
signs. It may occur in familial and sporadic forms. Some
patients may note a striking improvement with small
quantities of alcohol.
29. 0 Tics are abrupt, stereotyped, coordinated movements or
vocalizations. They may vary in intensity and be repeated at
irregular intervals. Tics may be exacerbated by stress and
relieved by distraction.
0 Tics may be motor or vocal and are classified as being either
simple or complex. Examples of simple motor tics include eye
blinking, shoulder shrugging, and toe curling. Spitting and finger
cracking are examples of complex motor tics. Simple vocal tics
may take the form of sniffing, throat clearing, snorting, or
coughing.
0 Complex phonic tics are meaningful syllables, words, or phrases
(“okay,” “shut up”), and include palilalia (repeating the last
syllables of one's words), echolalia (repeating someone else's
words
0 Sensory tics are uncomfortable sensations
(pressure, cold, warmth, or paresthesias) localized to certain
body parts that are relieved by the performance of an intentional
act in the affected area.
30. 0 Tics may also be classified as idiopathic (the majority) or
secondary. Secondary causes include head
trauma, encephalitis, stroke, and various drugs.
0 For treatment of tics, dopamine antagonists (haloperidol or
the atypical antipsychotics) are most effective;
however, because of the adverse effect profile of these
agents, less potent drugs such as clonazepam and clonidine
should be tried first
31. Q.1. A 45-year-old man with frequent but vague
gastrointestinal complaints has been doing well on a
combination of added fiber, ranitidine, and metoclopramide.
He has no new complaints. He appears to be chewing
gum, but examination reveals nothing in his mouth, and he
does not appear to be able to suppress the movements. He
is unaware of the movements, but his spouse noted their
progressive appearance during the past six months. The
most likely cause of his problem is
A. Whipple’s disease
B. Chronic oral tic disorder
C. Drug-induced tardive dyskinesia
D. Normal aging
32. Q.2. An 86-year-old woman with Alzheimer’s disease has been
using donepezil for her ailing memory. Risperidone was added
recently for progressively agitated behaviors. Several months
later, she appears slow and has had several falls.
Examination reveals a shuffling gait, stooped posture, and slowed
movements. Otherwise, her examination is unchanged.
Appropriate management includes
A. Starting levodopa/carbidopa for possible Parkinson’s disease
B. Starting an antidepressant for suspected depression
C. Discontinuing risperidone
D. Discontinuing donepezil
