MOVEMENT DISORDERS
Other movement
disorders
Monty A Silverdale
Abstract
This article examines movement disorders other than Parkinson’s dis-
ease, including ataxias and hyperkinetic movement disorders. Ataxias
are caused by dysfunction of the cerebellum or its connections and
cause irregular disordered movement. Hyperkinetic movement disor-
ders involve involuntary movements that often interfere with normal
movements. There are several different hyperkinetic movement disor-
ders, which can usually be distinguished by clinical assessment: in
chorea, the involuntary movements are random in nature; in dystonia,
the movements are patterned; in tremor, the movements are rhythmic;
in myoclonus, the movements are very brief, jerky and irregular; and in
tics, the movements are suppressible and associated with an underly-
ing urge sensation This article gives an approach to diagnosing pa-
tients with ataxic and hyperkinetic movement disorders, and discuss
some of the more common and important associations. It also dis-
cusses treatment including using deep brain stimulation, which can
be extremely effective in correctly selected patients.
Keywords Ataxia; chorea; dystonia; myoclonus; tardive dyskinesia;
tics; Tourette’s syndrome; tremor
Introduction
Ataxic disorders are caused by dysfunction of the cerebellum or
its connections and cause irregular disordered movement. Hy-
perkinetic movement disorders cause involuntary movements.
Simply observing the patient allows most hyperkinetic move-
ment disorders to be diagnosed. Much of this observation can be
performed while taking the history. Table 1 illustrates some of
the tricks used during examination to help reach a diagnosis of a
hyperkinetic movement disorder.
Ataxia
Definition
The cerebellum fine-tunes and coordinates movement so patients
with ataxia complain of clumsiness on attempted movement. They
can also have poor balance when walking, and slurred speech.
Testing for ataxia
Eye movements: pursuit eye movements (following a finger) are
often very jerky in ataxic patients. Saccadic eye movements
(looking from one target to another) often undershoot or over-
shoot the target. Nystagmus may be seen.
Monty A Silverdale PhD FRCP is a Consultant Neurologist and
Movement Disorder Specialist at the Greater Manchester
Neuroscience Centre, Manchester, UK. Competing interests: none
declared.
MEDICINE 44:9
Key points
C Ataxias are caused by dysfunction of the cerebellum or its
connections and cause irregular disordered movement
C The differential diagnosis of ataxia is large but the speed of
onset helps in making a diagnosis
C Hyperkinetic movement disorders are associated with excess
involuntary movements that often interfere with normal
movements. The type of hyperkinetic movement disorder is
usually apparent after careful observation of the patient
C In chorea, the involuntary movements are random in nature
C In dystonia, the movements are patterned and sustained
C In tremor, the movements are rhythmic
C In myoclonus, the movements are very brief and irregular
C In tics, the movements are suppressible and associated with
an underlying urge sensation
Speech: patients with ataxic disorders often have dysarthria.
Cerebellar dysarthria can be difficult to distinguish from other
forms of dysarthria. Getting the patient to say ‘Ta Ta Ta’ repet-
itively can help. In cerebellar dysarthria, speech can be irregular
(analogous to ‘atrial fibrillation’ of speech).
Upper limbs: when testing for upper limb ataxia, the finger
enose test is used. The examiner asks the patient to stretch out
their arm in order to touch the examiner’s finger, then flex their
arm to touch their own nose with their fingertip. The process is
then repeated. The examiner is looking for poorly coordinated
movements that can overshoot or undershoot the target, some-
times developing a tremor as the target is approached (intention
tremor). It is important that the examiner gets the patient to
straighten their arm fully as subtle ataxia may only develop as
the arm extends to approach the target. A disorder of rapidly
alternating movement (dysdiadochokinesia) is tested by asking
the patient to alternately pronate and supinate the tapping hand.
Lower limbs: ataxia in the lower limbs is tested using the heel
eshin test, in which the heel is passed up and down the shin.
Gait: an ataxic gait is usually broad-based and unsteady. Patients
usually struggle with tandem walking (where the heel of the front
foot touches the toes of the back foot with each step).
Causes of ataxia
There are many causes of ataxia (Table 2). It is helpful to divide
cerebellar disorders into those presenting acutely (seconds, mi-
nutes or hours), those presenting subacutely (days or weeks) and
those presenting over a chronic time course (months or years).
Toxic causes such as alcohol are usually obvious from the
547 Ó 2016 Elsevier Ltd. All rights reserved.
 MOVEMENT DISORDERS
Examining a patient with a hyperkinetic movement
disorder
C Observe the patient at rest with their hands resting on their lap.
This can be done while taking the history
C Distract the patient by asking them to close their eyes and
recount the months of the year backwards. Most movement dis-
orders worsen with distraction
C Ask the patient to hold their arms outstretched. The involuntary
movements of dystonia, chorea and essential tremor usually
worsen on sustained posture
C Ask the patient to perform actions including writing and pouring
water between cups. Most hyperkinetic movement disorders,
including chorea, dystonia and essential tremor, become more
obvious with action
C Ask the patient to try and suppress the movements for 10 sec-
onds. Tic disorder can usually be partially or completely sup-
pressed but will become much worse afterwards
C Test the patient for motor impersistence by asking them to stick
their tongue out for a count of 10 seconds. Patients with chorea
are not usually able to do this, and the tongue will keep popping
in and out of the mouth
C Ask the patient to walk. Many movement disorders including
chorea and dystonia worsen on walking
C Perform a full general and neurological examination looking for
other clues to the diagnosis
C If a psychogenic tremor is suspected, check for entrainment by
getting the patient to tap a rhythm with one hand and see
whether the frequency of tremor in the other hand changes to the
tapping frequency
Table 1
history. Structural problems usually show on cranial magnetic
resonance imaging. The age of onset often helps to reach a
diagnosis in chronic cerebellar disorders. Many genetic condi-
tions present at a younger age, whereas other conditions such as
multiple system atrophy present at an older age. Cranial imaging
can help in chronic cerebellar disorders: some conditions cause
cerebellar atrophy (e.g. ataxia telangiectasia) whereas others
usually do not (e.g. Friedreich’s ataxia). Cerebellar disorders are
discussed in more detail elsewhere.1
Chorea
Definition
Chorea denotes involuntary movements that are completely
random in nature. In most cases, the whole body is affected. A
patient with chorea can initially appear simply fidgety, and it is
sometimes difficult to distinguish an anxious, restless person
from someone with chorea. A fidgety patient is usually able to
suppress the movements when concentrating on something such
as writing, whereas chorea usually worsens on action, and
writing can be difficult. Testing for motor impersistence often
helps (Table 1).
Causes
Medical causes: these include non-ketotic hyperglycaemia, hy-
perthyroidism, systemic lupus erythematosus, anticardiolipin
MEDICINE 44:9
Important causes of ataxia
Immediate onset
Cerebellar stroke
Cerebellar bleed
Onset over days
Wernicke’s encephalopathy
Multiple sclerosis relapse
Viral cerebellitis (mainly in children)
Onset over weeks or months
Paraneoplastic
CreutzfeldteJakob disease
Autoimmune (e.g. anti-Glutamic Acid Decarboxylase (GAD)
antibodies)
Posterior fossa tumour (months or years depending on speed of
tumour growth)
Onset over years
Alcoholic cerebellar degeneration (can also present more quickly)
Friedreich’s ataxia (autosomal recessive)
Spinocerebellar ataxia (autosomal dominant, many genes)
Mitochondrial disease (can also present acutely or subacutely)
Multiple system atrophy (progresses over months or 1e2 years)
Idiopathic late-onset ataxia
Ataxia with vitamin E deficiency
Non-cerebellar causes
Sensory ataxia (impaired proprioception, positive Romberg’s test)
Bilateral vestibulopathy, (unsteady gait, positive Romberg’s test,
impaired dynamic visual acuity test)
Table 2
syndrome and polycythaemia, Sydenham’s chorea (post-strep-
tococcal) and chorea during pregnancy (chorea gravidarum).
Medications, including levodopa, the oral contraceptive pill,
anticholinergic drugs, anticonvulsants and antidepressants, can
cause chorea.
Huntington’s disease: this is an autosomal dominant condition
caused by an expanded trinucleotide repeat in the huntingtin
gene on chromosome 4. It causes psychiatric problems (depres-
sion, personality change), cognitive problems and a movement
disorder (usually chorea but sometimes dystonia or
parkinsonism).
Other causes: these include benign hereditary chorea, senile
chorea and various Huntington’s mimics.2
Dystonia
Definition
In dystonia, sustained muscle contractions lead to twisting
movements and abnormal postures. Whereas chorea causes
random movements, dystonia causes patterned movements e
meaning that the same movement is performed over and over
again. For example, if the head turns to the left, it repeatedly
turns to the left. Dystonia is focal when only one body part is
affected, for example the neck (cervical dystonia), the eyes
(blepharospasm) or the limbs. It can also be generalized,
meaning that the whole body is affected.
548 Ó 2016 Elsevier Ltd. All rights reserved.
 MOVEMENT DISORDERS
Types
Task-specific dystonia: this occurs only with certain activity,
such as writing (writer’s cramp). In this case, the patient finds
their fingers or arm adopt an unusual posture when they try to
write, making writing difficult. This condition is particularly
common in people who do a lot of writing. Other high-intensity
activities can also be associated with task-specific dystonia,
including playing a musical instrument (e.g. piano, violin) and
certain sports (e.g. the ‘yips’ in golf).
Primary torsion dystonia: it is usually genetic in nature. Patients
are neurologically normal apart from the dystonia (and some-
times tremor). Some, although not all, of the genes involved have
been characterized. When the condition starts in childhood, it
usually presents as lower limb dystonia, which gradually pro-
gresses into generalized dystonia. Adults developing dystonia
usually present with focal dystonia, in particular cervical dysto-
nia and blepharospasm.
Cerebral palsy: although classically caused by neonatal jaun-
dice, any cause of cerebral palsy can be associated with
dystonia.
Dopa-responsive dystonia: this form is an autosomal dominant
condition often presenting with childhood dystonia that can
mimic dystonic cerebral palsy. Patients respond dramatically to
levodopa and can go from needing a wheelchair to having
normal mobility. Although it is rare, it is important not to miss
this, and all patients thought to have dystonic cerebral palsy
should have a trial of levodopa (e.g. co-careldopa 125 mg three
times daily for 2 weeks).
Wilson’s disease: this is caused by an autosomal recessive defect
in copper metabolism. It can present with hepatic disease or
neurological problems, including personality change, dystonia
and tremor. Most patients have a ring of copper around the
cornea that can be seen on slit-lamp examination (Kayser
eFleischer ring) as well as a low serum ceruloplasmin concen-
tration and high urinary copper excretion. Early diagnosis is
important to commence treatment and prevent permanent
disability.
Other causes: rarer causes of dystonia include neuro-
acanthocytosis and neurodegeneration with brain iron
accumulation.3
Tremor
Definition
Tremor is a rhythmic oscillation about a body part. It can affect
any part of the body, most commonly the upper limbs. The na-
ture of the tremor gives an important clue to the diagnosis, in
particular whether it is a rest tremor or a postural and action
tremor. Predominantly resting tremors are usually due to Par-
kinson’s disease. Parkinsonian tremor can sometimes occur with
a sustained posture, but there is usually a delay between the
arms being outstretched and the tremor developing (this tremor
is termed a postural re-emergent tremor). An action tremor rarely
occurs in Parkinson’s disease.
MEDICINE 44:9
Causes
Medical causes: we all have a high-frequency physiological
tremor that becomes more apparent with stress. Hyperthyroidism
and drugs (including b-adrenoceptor agonists, lithium and so-
dium valproate) can cause or worsen tremor, and careful atten-
tion should be paid to the drug history.
Essential tremor: this causes a postural and action tremor of
slightly lower frequency than physiological tremor. Unlike a
Parkinsonian re-emergent tremor, essential tremor occurs
immediately on sustained posture. The tremor worsens on
action (which is unusual for parkinsonian tremor). A typical
patient with a parkinsonian tremor might complain that their
tremor was worst when watching television, whereas patients
with essential tremor typically complain about difficulty
holding a cup of tea. Essential tremor is usually familial,
although the causative gene is unknown. Although often
termed ‘benign’, essential tremor can in severe cases be very
disabling.
Other causes: there are many other causes including dystonic
tremor, Holmes’ tremor (rubral or midbrain tremor) and cere-
bellar tremor.4
Myoclonus
Definition
Myoclonus refers to brief jerks of the muscles. It can affect the
whole body at once (generalized myoclonus) or a small area such
as the fingers (focal myoclonus). Myoclonus can be positive (in
which the jerks are brief contractions of the muscle) or negative
(whereby the jerks are caused by brief loss of sustained muscle
contraction).
Causes
A large number of conditions can cause myoclonus, and the
diagnosis is usually made by recognizing the features of these
conditions other than myoclonus itself.
Medical causes: several medical conditions can cause myoc-
lonus. The metabolic flap of the outstretched arms seen in res-
piratory, hepatic and renal failure is a form of negative
myoclonus (asterixis). Drugs including opioids can also cause
myoclonus.
Neurological causes: myoclonus can originate at any level of the
central nervous system, including the cortex, subcortical regions
and spinal cord. Neurological conditions causing myoclonus
include several epileptic disorders, Alzheimer’s disease, cortical
Lewy body disease, CreutzfeldteJacob disease and post-hypoxic
myoclonus.5
Tics
Definition
A tic is a movement performed in response to an internal urge (in
the same manner that an itching sensation causes a desire to
scratch). Tics can usually be suppressed for a brief period if the
patient is asked to do so, but worsen when patients stop trying to
suppress them. Tics can affect any part of the body, although
549 Ó 2016 Elsevier Ltd. All rights reserved.
 MOVEMENT DISORDERS

Some drugs used to treat hyperkinetic movement disorders

Drug Starting dose Typical maintenance dose Comments

Tetrabenazine 12.5 mg daily 25e75 mg/day e divided into three doses Can help chorea, dystonia, tics and tardive
dyskinesia. Adverse effects include
parkinsonism and depression
Haloperidol 500 micrograms daily 1e15 mg/day e divided into two or three Can help chorea, dystonia and tics. Can cause
doses sedation and parkinsonism. Prolonged use
can cause tardive dyskinesia
Baclofen 5 mg daily 20e60 mg/day e divided into three doses Mainly used for dystonia. Can cause sedation
Trihexyphenidyl 1 mg daily 3e9 mg/day e divided into three doses May improve dystonia. Can make chorea
worse. Adverse effects including dry eyes, dry
mouth, urinary retention, sedation and
cognitive problems
Propranolol 80 mg daily using a 80e160 mg/day e once daily using a long- Useful treatment for essential tremor.
long-acting preparation acting preparation Contraindicated in asthmatic patients
Primidone 50 mg daily 250e500 mg/day e once daily or divided into Useful treatment for essential tremor. May
two doses cause sedation
Clonidine 50 micrograms daily 200e400 microgram/day e divided into two Useful treatment for tics (unlicensed indication
doses in the UK) and Tourette’s syndrome. Adverse
effects include sedation and hypotension
Sodium valproatea 300 mg daily 600e2000 mg/day e divided into two doses Useful treatment for myoclonus. Adverse
effects include tremor. Teratogenic
a
Use for this indication is unlicensed in the UK and not described in the BNF.

Table 3

eye-blinking, eyebrow-raising and shoulder movements are Deep brain stimulation

common. These motor tics can be confused with other move-
ment disorders unless the patient is asked about internal urge
and an attempt is made to see whether they can suppress the Deep brain stimulator lead
movements. As well as these motor tics, vocal tics such as throat-
Electrodes
clearing are common. Swearing (coprolalia) can occur but is less Subthalamic nucleus
common.

Causes
Connective wires
Tics are very common, affecting >10% of the population in
childhood and adolescence. They usually improve after adoles-
cence, but can occasionally worsen in adulthood. Tourette’s
syndrome is a severe form of tic disorder, often accompanied by Pacemaker
obsessiveecompulsive disorder and attention-deficit hyperactiv-
ity disorder. Tic disorder can also occur in other neurological
conditions including Huntington’s disease.

Tardive dyskinesia
Prolonged treatment with dopamine receptor blocking drugs
can lead to involuntary movements termed tardive dyskinesia.
The most commonly implicated drugs are neuroleptics,
including haloperidol and chlorpromazine. The dopamine-
blocking anti-nausea drugs prochlorperazine and metoclopra-
mide can also cause tardive dyskinesia. The most common
manifestations of tardive dyskinesia are repetitive orolingual
movements that reduce when the patient speaks or eats.
Virtually any movement disorder can be tardive in nature,
including dystonia, tremor and an internal restlessness termed
akathisia.
Figure 1 Deep brain stimulation is effective treatment for some
movement disorders, particularly Parkinson’s disease, essential
tremor and dystonia. A wire is placed in a small area of the basal
ganglia. The specific target varies between different conditions and
includes the subthalamic nucleus, the globus pallidus, the thalamus
and the zona incerta. The wire is attached to a pacemaker box in the
chest wall that passes a high-frequency current into the basal ganglia
target. The settings can be manipulated using a computer, often
leading to dramatic improvements in correctly selected patients.

MEDICINE 44:9 550 Ó 2016 Elsevier Ltd. All rights reserved.

 MOVEMENT DISORDERS

Psychogenic movement disorders disorders are often associated with adverse effects that can be
worse than the movements themselves. Many patients with hy-
Many of the above-mentioned movement disorders can have
perkinetic movement disorders prefer to avoid drug treatment.
psychogenic causes, which make up at least 5% of referrals to a
Table 3 illustrates some of the drugs used to treat these disorders.
movement disorder clinic. Distinguishing a psychogenic move-
In more severe cases, deep brain stimulation is used and can be
ment disorder from an organic movement disorder can be diffi-
extremely effective in the correctly chosen patient (Figure 1).A
cult. It is important to look for incongruity (the movement
disorder is not typical for a known movement disorder) and
inconsistency (the movement disorder changes over time). Un- KEY REFERENCES
like organic movement disorders, most psychogenic movement 1 Teive HA, Ashizawa T. Primary and secondary ataxias. Curr Opin
disorders improve with distraction. Entrainment is common in Neurol 2015; 28: 413e22.
psychogenic tremor (see Table 1). 2 Walker RH. Differential diagnosis of chorea. Curr Neurol Neurosci
Rep 2011; 11: 385e95.
Treatment of other movement disorders 3 Phukan J, Albanese A, Gasser T, Warner T. Primary dystonia and
dystonia-plus syndromes: clinical characteristics, diagnosis, and
Some forms of ataxia can be treated by addressing the underlying
pathogenesis. Lancet Neurol 2011; 10: 1074e85.
cause, for example giving thiamine for Wernicke’s encephalop-
4 Alty JE, Kempster PA. A practical guide to the differential diagnosis
athy or immunotherapy for autoimmune ataxia. Other than this,
of tremor. Postgrad Med J 2011; 87: 623e9.
drug treatment of ataxias is disappointing. If there is a secondary
5 Kojovic M, Cordivari C, Bhatia K. Myoclonic disorders: a practical
cause of a hyperkinetic movement disorder, treatment should
approach for diagnosis and treatment. Ther Adv Neurol Disord
initially focus on this. Botulinum toxin injections are very
2011; 4: 47e62.
effective in the treatment of focal dystonia and also for some
focal tics. The drugs used to treat hyperkinetic movement

MEDICINE 44:9 551 Ó 2016 Elsevier Ltd. All rights reserved.