Movement Disorders
Vol. 17, No. 4, 2002, pp. 821–825
© 2002 Movement Disorder Society

Brief Report

Tourettism and Dystonia After Subcortical Stroke
Carolyn H. Kwak, MS, PA-C, and Joseph Jankovic, MD*
Baylor College of Medicine, Parkinson’s Disease Center and Movement Disorders Clinic, Houston, Texas, USA

Abstract: The term “tourettism” has been used to describe
Tourette syndrome (TS)-like symptoms secondary to some specific cause. Tics associated with attention deficit hyperactivity
disorder (ADHD), obsessive–compulsive disorder (OCD), or
both, are commonly present in TS, but this constellation of
symptoms has been rarely attributed to stroke. We describe two
boys who suffered a subcortical stroke and subsequently developed hemidystonia, tics, and behavioral comorbidities. Both
had right hemispheric stroke involving the basal ganglia at 8
years of age, and in both the latency from the stroke to the onset

of left hemidystonia was 2 weeks. In addition to ADHD and
OCD, both exhibited cranial-cervical motor tics but no phonic
tics. The temporal relationship between the stroke and subsequent TS-like symptoms, as well as the absence of phonic tics
and family history of TS symptoms in our patients, argues in
favor of a cause and effect relationship, and the observed association provides evidence for an anatomic substrate for TS
and related symptoms. © 2002 Movement Disorder Society
Key words: tourettism; stroke; dystonia; obsessive compulsive disorder; attention deficit hyperactivity disorder

Tourette syndrome (TS) is a neurological disorder of
childhood onset characterized by tics that vary in distribution, severity, and frequency which are commonly
associated with neurobehavioral conditions such as
attention deficit hyperactivity disorder (ADHD) and
obsessive–compulsive disorder (OCD).1 The term tourettism has been used to describe TS-like symptoms secondary to some specific cause.2 Besides TS, tics have
been reported secondary to numerous causes such as infection, head trauma, medication use, and in association
with other neurodegenerative disorders.3–13 Tourettism
secondary to cerebral infarction have been described previously in only three patients.14–16 We describe here two
boys who suffered a subcortical stroke, both at 8 years of
age, with subsequent onset of hemidystonia, tics and behavioral comorbidities.

PATIENT 1
This 12-year-old, right-handed boy, a product of a full
term, uncomplicated pregnancy and delivery with normal
developmental milestones, suffered a right subcortical
hemorrhagic stroke secondary to the formation of a
blood clot of unknown etiology in the region of the right
caudate and putamen, 4 years earlier while assisting his
father unload wood boards. At age 8, he had a sudden
onset of severe headache with subsequent weakness and
numbness of the left side of the body. Within 2 weeks
after the stroke, he regained his motor strength and the
left hemi- paresthesia completely resolved. He, however,
developed involuntary flexion of his left forearm
muscles and left-hand deformity. On examination he had
left hemidystonia manifested by flexion of the left metacarpophalangeal (MCP), proximal interphalangeal (PIP),
and distal interphalangeal (DIP) joints of the second and
third digits with hyperextension of the left MCP, PIP,
and DIP joints of the fourth and fifth digits, involuntary
left foot inversion with toe flexion of digits 2 to 5 and
extension of the left big toe (see Video, Segment 1). He
also held his arm behind his back, with supination and
abduction of the left arm and elevation of left shoulder
while walking. His symptoms have stabilized within the
past 2 years. Levodopa, trihexyphenidyl, and baclofen

A videotape accompanies this article.
*Correspondence to: Joseph Jankovic, MD, Department of Neurology, 6550 Fannin, Suite 1801, Houston, TX 77030. E-mail:
josephj@bcm.tmc.edu
Received 15 December 2000; Revised 4 May 2001, 4 September
2001; Accepted 2 October 2001
Published online 5 April 2002 in Wiley InterScience (www.
interscience.wiley.com). DOI 10.1002/mds.10207

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C.H. KWAK AND J. JANKOVIC

failed to provide any improvement of his dystonia. A
trial of clonazepam partly improved his symptoms, but
the drug was discontinued due to side effects of severe
personality changes and mood fluctuations. Botulinum
toxin A (BTX) injections in the left forearm and left foot,
administered every 3 months for the past 3 years, markedly improved his left foot dystonia.
Six months after the stroke, the patient developed
compulsive tics of repetitive blowing of air onto his
palm, compulsive scratching and a persistent urge to
sniff (particularly food and people). He was started on
fluoxetine with notable improvement of his compulsive
symptoms. When the medication was discontinued he
had exacerbation of all symptoms and developed depression. In addition, the patient’s parents noted the onset of
behavioral changes including restlessness, hyperactivity,
difficulty focusing, and low distraction tolerance. He was
diagnosed with ADHD based on the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV),17 and his
concentration and academic performance markedly improved with dextroamphetamine-racemic amphetamine
20 mg without exacerbation of compulsive tics. There is
no family history of dystonia, tics, ADHD, or OCD and
his parents claim that none of these symptoms were present before the stroke. At age 11 years, he exhibited additional tics including bursts of rapid, clonic, head nodding followed by slower, dystonic, cervical extension
and flexion, and more sustained stretching and thrusting
movement of the neck forward, which his parents noted

6 months before his initial visit. The patient did not have
a history of phonic tics nor did he exhibit vocalizations
during the initial visit. Clonidine 0.25 mg per day provided no meaningful improvement of his tics or ADHD.
His parents note the motor tics waxed and waned in
severity and frequency, but the compulsive type behaviors such as the urge to smell and pick on scabs have
persisted over the past 4 years. On our initial physical
examination he had severe excoriations and scabs on
both legs (see Video, Segment 1). The most recent MRI
showed infarct in the right putamen and caudate (Fig. 1).
He continues to be an active child who enjoys riding
his bike and playing basketball, although he is unable to
dribble with his left hand. He is unable to play baseball
because he has difficulty releasing the bat due to his left
hand dystonia.
PATIENT 2
This is a 22-year-old, right-handed man who was first
seen in our clinic at 8 years of age, shortly after he
suffered a right ischemic stroke in the distribution of the
right middle cerebral artery secondary to migraine headache, with basal ganglia involvement. This was manifested by a migrainous headache, unsteady, propulsive
gait and left side weakness. At the time of hospital discharge, he regained his motor strength and ability to
walk, but had occasional clonic seizures. Approximately
2 weeks after the stroke, he developed left-sided upper
extremity dystonia. During his initial physical examina-

TABLE 1. Acquired tics/obsessive–compulsive disorder secondary to brain lesion
Study
23

Rodrigo et al., 1997
Simpson et al., 199524
Swoboda et al., 199522
Ward et al., 198816
Demirkol et al., 199928
25

Hugo et al, 1999

Lesion site

Etiology

Bilateral globus pallidus
Right inferior parietal
Right posterior frontal, right internal
carotid occlusion
Fronto-arietal lobe, left frontal, lacunar
infarcts in right superior cerebellar
peduncle and left basal ganglia
Bilateral, symmetrical globus pallidus
“tiger’s eye” lesion
Temporal and frontal lobes

Carbon monoxide poisoning, infarction
Infarction
Infarction

OCD
OCD
OCD

Infarction, tumor

Premonitory urge

Unknown

Tics, ADHD, OCD

Hypoperfusion and compensatory
hyperperfusion
Tumor (lipoma, hygroma, angioma,
hamartoma, tuberous sclerosis),
arachnoid cyst, post-encephalitic
hydrocephalus, head trauma,
infarction
Malignancy

OCD

Berthier et al., 199626

Frontal and temporal lobes, cingulate,
caudate

Peterson et al., 196629

Ventral striatum, corpus collosum,
thalamus, midbrain

Max et al., 199527
Ward et al., 198816

Frontal and temporal lobe lesions
Fronto-parietal lobe, left frontal, lacunar
infarcts in right superior cerebellar
peduncle and left basal ganglia

OCD, obsessive–compulsive disorder.

Movement Disorders, Vol. 17, No. 4, 2002

Head trauma
Infarction

Symptom

OCD

Tics, OCD, with temporal
progression of tics and OCD
with tumor progression
OCD
Transient feelings of compulsion

TOURETTISM AND DYSTONIA AFTER SUBCORTICAL STROKE

FIG. 1. Flair/90 MRI of the brain of Patient 1 showing infarction of
right putamen and caudate.

tion in our clinic, he exhibited involuntary flexion of the
left hand, abduction of the left arm, drawing of the left
arm behind his back with a clinched left fist. He was able
to passively open the fingers of the left hand, but when
released the fingers remained flexed (see Video, Segment 2).
Four weeks after the stroke, he developed excessive
bilateral eye blinking and transient blepharospasm with
occasional upward oculogyric deviation. He exhibited
increased frequency of blinking, transient upward oculogyric deviation, facial grimacing with contraction of
the paranasal muscles and jaw opening, but no phonic or
other motor tics were present at the time of initial examination in our clinic. Although he was a “straight-A”
student before the stroke, his academic performance
steadily declined (grades D and F), mainly attributed to
difficulty sustaining attention. The MRI showed infarction in the distribution of the right middle cerebral artery
with involvement of the basal ganglia, specifically the
head of the caudate nucleus. Migraine prophylaxis with
verapamil was started at the time of his stroke.
The patient was delivered after a full term, uncomplicated pregnancy to a healthy mother and had appropriate
developmental milestones. There is a multigenerational
family history of seizures but no tics, ADHD, OCD, or
dystonia. His father died of a ruptured aneurysm at age
24. No prior history of dystonia, tics, or ADHD was
noted in the patient.
DISCUSSION
Our two patients exhibit the rare combination of dystonia, tics, ADHD, and OCD. Both had right hemisphere

823

strokes involving the basal ganglia at 8 years of age and
in both the latency from the stroke to the onset of left
hemidystonia was 2 weeks. In addition to ADHD and
OCD, both exhibited cranial–cervical motor tics, but no
phonic tics. The temporal relationship between the stroke
and subsequent TS-like symptoms, as well as the absence
of phonic tics and family history of TS symptoms in our
patients, argues in favor of a cause and effect relationship
and against a simple coincidental occurrence of a stroke
and TS.
The coexistence of tics and dystonia has been well
documented18,19 but the association of tourettism (consisting of tics, ADHD, and OCD) with post-stroke hemidystonia has not been described previously. Three reports have described individual cases of TS-like symptoms secondary to vascular lesions.14–16 In one report, a
43-year-old man developed motor tics after a four-artery
angiography.14 Although neuroimaging was not available when the case was first reported, a vascular lesion in
the basal ganglia was suspected to be responsible for
subsequent tics including tongue clicking and protrusion,
eye closure, sniffing, frowning, and numerous other motor tics as well as palilalia. Neurobehavioral changes
were not reported in this patient. Masso and Obeso15
described a 66-year-old man with post-anoxic hemiballism and concomitant onset of coprolalia, but without
motor or phonic tics. Both the hemiballism and coprolalia improved with tetrabenazine. More recently, Ward16
reported a 62-year-old woman with acute onset of dysphasia and a subsequent suppressible urge to shake her
right arm. A CT scan showed lacunar infarcts in the right
superior cerebellar peduncle and left basal ganglia.
The occurrence of tics, ADHD and OCD, in our two
patients after a subcortical stroke provides additional
support for the notion that these TS symptoms have a
biological and neuroanatomical substrate. Well documented cases of ADHD after stroke are lacking, but
hemi-inattention, or neglect, is a common phenomenon
after a stroke.20 Although idiopathic OCD is usually not
associated with any identifiable, anatomic lesion21
several cases of secondary OCD have been documented.16,20,22–29 Acquired obsessive–compulsive symptoms have been reported secondary to infarction of inferior parietal lobe,24 posterior frontal lobe,22 both globus
pallidi,23 both caudate nuclei,20 superior cerebellar peduncle, and left basal ganglia.16 Several documented
cases of acquired OCD associated with cerebral malignancy, trauma or perfusion abnormalities have been attributed to lesions involving the frontal, parietal, temporal lobes and cingulate areas.16,25–27 There is now a substantial body of evidence that implicates basal ganglia
dysfunction in the pathogenesis of OCD.30–33 In a study

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C.H. KWAK AND J. JANKOVIC

of patients with bilateral basal ganglia lesions secondary
to trauma, anoxic or toxic encephalopathy stereotyped
and obsessive–compulsive behaviors were seen in many
of these patients after the reported injuries.30 We and
others have observed patients with Parkinson’s disease
and atypical parkinsonism who have developed features
of OCD, such as obsessions with bowels, compulsive
gambling, and other obsessive–compulsive behaviors as
their disease progresses.32
Acquired TS symptoms of tics and neurobehavioral
comorbidities have been reported secondary to a variety
of other lesions, usually involving the basal ganglia.30,34–39
Furthermore, various imaging and biochemical studies
provide support for frontal-subcortical involvement in
mediating human behavior.40 In TS, the cortico-striatalthalamic-cortical circuit plays an important role in the
pathogenesis of TS and related disorders.39 A dysfunction in the dorsolateral prefrontal circuit, which links
Brodmann’s area 9 and 10 with the dorsolateral head of
the caudate has been implicated in an impairment of
executive functions and possibly in ADHD. The lateral
orbitofrontal circuit originates in the inferior lateral prefrontal cortex (area 10) and projects to the ventral medial
caudate. An abnormality to this circuit is associated with
personality changes, mania, disinhibition, and irritability.
Lastly, the anterior cingulate circuit, which arises in the
cingulate gyrus (area 24), receives input from the amygdala, hippocampus, medial orbitofrontal cortex, entorhinal and perirhinal cortex, and projects to the ventral striatum has been linked to a variety of behavioral problems
including OCD.41
In conclusion, this unique presentation of left hemidystonia and tourettism in our two young patients after a
right subcortical hemorrhagic stroke involving the basal
ganglia support the neuroanatomical substrate for tics,
associated neurobehavioral comorbidities and dystonia.
LEGENDS TO THE VIDEOTAPE
Segment 1. Patient 1 demonstrates left hemidystonia
and mild blinking, head jerking and shoulder shrugging,
but most of his motor tics are suppressed. He also describes his urges and other features of OCD and shows
severe markings on his legs from self scratching.
Segment 2. Patient 2 shows blinking, oculogyric deviations, facial grimacing, jaw opening and other tics in
addition to left hemidystonia.
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Movement Disorders, Vol. 17, No. 4, 2002