Movement Disorders
Vol. 8, NO. 2, 1993, pp. 213-216
0 1993 Movement Disorder Society

Brief Report

Basal Ganglia Infarction as a Possible Cause of
Cervical Dystonia
Eric S. Molho and Stewart A. Factor
Department of Neurology, Albany Medical College, Albany, New York, U.S.A.

Summary: Cervical dystonia (CD) is usually an idiopathic disorder that results in abnor-

mal movements and painful postures of the neck. Although symptomatic CD caused by
focal CNS lesions has been described in the literature, it is an exceedingly rare phenomenon. We report two women who had an abrupt onset of CD at the ages of 39 and 68
years. Each patient had rotation of the head to the right and was found to have a lacunar
infarction in the left putamen on magnetic resonance imaging scan. The abrupt onset of
symptoms and appropriate location and laterality of the cerebral lesions suggest an
etiologic link between the infarctions and the patients’ CD. These cases are the first
reports of CD possibly caused by basal ganglia infarction. Key Words: Cervical dystonia-Secondary dystonia-Infarction.

isolated CD secondary to a focal CNS lesion have been
previously reported (8,ll-13). We present two additional
cases both associated with unilateral putamenal infarction.

Cervical dystonia (CD) is the most common of the focal
dystonias and is usually idiopathic (14). This disorder is
characterized by abnormal involuntary movements and
typical postures of the neck and shoulders that are often
painful. The mean age of onset is early in the 5th decade
and there is a slight female preponderance in the patient
population (2,5,6). The onset is usually insidious, with
symptoms occurring intermittently at first and in association with specific actions. Most patients deteriorate during the initial 5 years, and then the symptoms tend to
stabilize (2,3,5). The condition is ultimately characterized
by dystonic postures that are present at rest, worsen with
action, and improve with sleep. Spontaneous remissions
occur in 1&30% of patients but are rarely complete or
permanent (3,5,7). Rotation of the neck (torticollis) is the
most common posture seen, with lateral flexion (laterocollis), flexion (anterocollis), and extension (retrocollis) also occurring in various combinations (2,5-7). Although symptomatic dystonia resulting from focal CNS
lesions has been well described in the literature, in most
of these cases the patients have had involvement of one
side (hemidystonia) or one limb (8-10). Only five cases of

CASE 1
A 68-year-old woman had a history of hypertension and
peptic ulcer disease. On her 67th birthday she experienced the sudden onset of sharp pain in the left side of her
neck that was accompanied by a pulling sensation in the
same area and mild turning of her head to the right. She
denied other neurologic symptoms. Initially, the sharp
“neuralgic” pain dominated her clinical picture and she
was treated with carbamazepine. She experienced a moderate improvement of her pain, but over the next several
months the pulling sensation and twisting of her neck
became more prominent.
She was referred to the Albany Medical College movement disorder clinic 1 year later. She complained of turning of her head to the right with tilting of her head to the
left. There was a moderate amount of discomfort that was
continuous during waking hours and painful spasms that
lasted several minutes and occurred -10 times daily. Her
symptoms were relieved to a mild extent by wrapping her
neck with a scarf, wearing a soft cervical collar, supporting the left side of her neck with her hand, or lying down.
Walking or standing was associated with a significant
worsening of her discomfort.
She had no history of birth injury, head or neck trauma,

Address correspondence and reprint requests to Dr. S. A.
Factor, Department of Neurology (A70), Albany Medical College, New Scotland Avenue, Albany, NY 12208, U.S.A.
This work was presented in part at the Symposium on Hyperkinetic Movement Disorders, cosponsored by the Movement
Disorder Society and the Parkinson Study Group, September 29,
1991, Seattle, Washington.

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E. S. MOLHO AND S . A . FACTOR

exposure to neuroleptic or related medications, liver disease, or a family history of a movement disorder. Her
medications were carbamazepine 200 mg t .i.d., clonidine,
and alprazolam.
On examination, she had hypertrophy and tenderness
of the left sternocleidomastoid muscle. Her left trapezius
muscle was also tender. Mild right torticollis and left laterocollis were present. Her left shoulder was mildly elevated. A low-amplitude, jerky, negative head tremor was
intermittently present as well. Head rotaiion to the left
was limited to 6&70". Otherwise, the range of motion of
her neck was full. Lying supine had no appreciable effect,
but walking was associated with an increase in both lateral flexion to the left and left shoulder elevation. The
remainder of her neurologic examination was unremarkable.
Serum ceruloplasmin and copper levels were normal.
Plain radiographs, computed tomography (CT), and magnetic resonance (MR) imaging of the cervical spine all
showed diffuse degenerative arthritis. A cranial MR scan
was obtained that showed a lesion in the left putamen,
consistent with a lacunar infarction (Fig. 1A and B).
She has been successfully treated on three occasions
with botulinum toxin A injections.

A

CASE 2
A 41-year-old woman had a long history of anxiety and
depression, which were treated with various anxiolytic
and antidepressant medications. At the age of 38 years
she experienced the abrupt onset of neck stiffness (she
recalled the specific month this occurred), followed by
constant jerking and twisting of her head to the right. A
careful review of the records revealed that she had taken
alprazolam, doxepin, phenelzine, lorazepam, diazepam,
imipramine, and fluoxetine, but no dopamine-antagonist
medications before the onset of her neck difficulties. Despite multiple chiropractic manipulations and treatment
with diazepam, her condition worsened. She complained
of pain in her shoulders and the back of her neck. Sitting
or standing worsened the pain and twisting. Lying down
and putting both hands on the back of her head improved
her discomfort. The movements disappeared during
sleep.
There was no history of head or neck injury, liver disease, or a family history of movement disorder. She was
born -2 months prematurely, but there was no history of
neonatal asphyxia. She reached all motor and cognitive
milestones within normal age limits. She was a cigarette
smoker but did not use birth control pills. She had no
other risks for cerebrovascular disease.
On examination, she had hypertrophy of her left sternocleidomastoid and right trapezius muscles. Her ability
to rotate her head to the left was limited to 15". Otherwise, the range of motion of her neck was full. When
seated, at rest, there was rotation to the right of 30",
lateral flexion to the right of 30°, and mild anterocollis.
Superimposed spasms periodically increased the rotation
as well as pulling her head anteriorly and downward. Her
right shoulder was elevated and anteriorly displaced.
Placing the back of her head against a wall or putting both
hands behind her neck significantly improved her dysto-

Movemenr Disorders, Vol. 8, No. 2 , 1993

FIG. 1. Magnetic resonance scan, case 1. A: T1-weighted image
shows lacunar infarction in the left putamen (arrow). B: T2weighted image of same (arrow).

nia. The remainder of her examination was unremarkable.
Normal studies included an electroencephalogram, MR
imaging of the cervical spine, serum ceruloplasmin and
copper levels, complete blood count and smear, full
chemistry profile, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, antiphospholipid antibody titers, and dilution studies for lupus anticoagulant.
A cranial MR scan revealed a left putamenal lesion consistent with a lacunar infarction (Fig. 2A and B). Treatment with trihexyphenidyl and ethopropazine was unsuccessful. She experienced a partial remission from June
1990 to June 1991. She was not seen in our clinic during
this time, and details of her condition during the remis-

BASAL GANGLIA INFARCTION AND CERVICAL D YSTONIA
A

B

FIG. 2. Magnetic resonance scan, case 2. A T1-weighted image
in the coronal plane with movement artifact and Iacunar infarction in the left putamen (arrow). B: T2-weighted image of same
(arrow).

sion are not available. She has been successfully treated
on two occasions with botulinum toxin A injections.

DISCUSSION
Dystonia secondary to a focal CNS lesion has been well
described in the literature. Most of these reports have
involved cases of hemidystonia or focal limb dystonia.
Much of what is known about the pathological-anatomic
basis of dystonia, in general, is based on findings in these
reports. In 1975, Dooling and Adams (9) performed neuropathological examinations in five patients with posthemiplegic athetosis and found that lesions of the striatum were central to this disorder. The authors also proposed that relative sparing of the thalamus and cortical
spinal tracts was necessary for the development of dystonia. In 1985, Marsden et al. (8) reported on 28 patients

215

with symptomatic dystonia. Twenty-six of these patients
had hemidystonia and the great majority of those patients
had lesions involving the contralateral striatum (particularly putamen), thalamus, or both. It was proposed that
the dystonia resulted from an anatomic or functional disconnection of the thalamus from the striatum, resulting in
a disruption of subsequent thalamic modulation of premotor and motor cortex. In 1985, Pettigrew and Jankovic
(10) reported on 22 patients with hemidystonia with a
similar anatomic distribution of lesions, and reasserted
the importance of lesions involving the striatum and relative sparing of the corticospinal tracts. These anatomical
concepts are now generally accepted, with the putamen
being the most common location for lesions. A number of
other case reports have supported these findings (14-20).
One article, however, has called into question the requirement for corticospinal tract sparing (20).
Other typical forms of focal dystonia have been reported with focal CNS lesions, but these reports have
been rare. Isolated blepharospasm has been described in
a patient with an angioma in the rostral brainstem (21).
Several cases of blepharospasm associated with other
forms of dystonia or as part of a more generalized extrapyramidal syndrome have been reported as well (2224). The lesions associated with these cases were not localized to any one area, but were most common in the
rostral brainstem and thalamus.
Cervical dystonia has been observed in five patients
with focal CNS lesions. In 1985, Marsden et al. (8) described two patients with typical CD and an arteriovenous malformation involving the caudate nucleus contralateral to the direction of neck rotation. In 1980, Maki et
al. (1 1 ) published a case of a child who had transient CD
3 days after sustaining a traumatic infarction in the caudate nucleus and putamen contralateral to the direction of
rotation. In 1989, Isaac and Cohen (12) reported a patient
with CD occumng 5 years after sustaining a traumatic
hemorrhage in the contralateral striatum. Most recently,
Biary et al. (13) reported a patient with CD occumng after
sustaining a traumatic lesion in the left centrum semiovale. The direction of rotation was not described.
We report the first cases of CD possibly due to nontraumatic lacunar infarctions in the putamen contralateral
to the direction of neck rotation. Although strict causality
cannot be proven in these cases, there are a few compelling reasons suggesting that the CD was secondary to
these lesions. First, the location of the lesion was in the
putamen in both cases. In addition, the lesions were contralateral to the direction of neck rotation, consistent with
the previously described cases of secondary hemidystonia and cervical dystonia. Second, the clinical presentation of each of our patients was atypical when compared
with that of large populations of patients with idiopathic
CD (2,5-7). In both cases, the abrupt onset suggested a
vascular insult. A sudden onset of symptoms is rarely
seen in idiopathic CD. Only 11% of 220 patients reported
by Rondot et al. (6) had such a presentation. Results of
cranial MR imaging or CT in these patients was not given.
In addition, the age of onset in our first patient was -20
years older than the mean age of onset of idiopathic CD
(2,5-7). Third, definite risk factors for cerebrovascular
disease were present in our first patient, who had a long

Movement Disorders, Vol. 8, No. 2, 1993

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E. S. MOLHO AND S. A . FACTOR

history of hypertension. Finally, the neuroradiologic appearance of the lesions in these two patients is not consistent with artifact or nonspecific white matter disease.
The decreased signal seen on T1 images, and the shape
and size of the lesions in question are all consistent with
lacunar infarction.
In summary, it is generally accepted that putamenal
dysfunction is important in the pathogenesis of dystonia.
We believe that the two cases presented here suggest that
CD can be caused by lacunar infarction of the putamen.
Additionally, any CD patient with atypical clinical features such as an abrupt onset of symptoms, especially
those with risk factors for stroke, should have a cranial
MR scan. CT scanning, although helpful, may not be sensitive enough to demonstrate all of these lesions.
Acknowledgment: We thank Faith Wood for typing the manuscript. This article was supported by the Albany Medical College
Parkinson’s Research Fund.

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