Journal of Neurology, Neurosurgery, and Psychiatry 1983;46:404-409

Dystonia caused
by putamino-capsulo-caudate vascular lesions
B DEMIERRE AND P RONDOT
From the Service of Neurology, H6pital Sainte Anne, Paris, France

Three cases of unilateral dystonia of vascular origin are presented and compared with
those in the literature. Damage in the region of the lenticulostriate arteries accounted for
ischaemic lesions of the putamino-capsulo-caudate region, the external pallidum probably also
being involved. This syndrome appears if the ischaemic accident took place during childhood.
The functions of each of these structures and the possible role of the anterior limb of the internal
capsule are discussed.
SUMMARY

Dystonia represents a particular group of diseases
involving the motor system. Denny-Brown' defined
it as "a fixed or relatively fixed attitude in association with other extrapyramidal disorders of movement". Dystonia musculorum deformans is its major
form, but the absence of specific pathology in this
condition makes any physiopathological interpretation particularly difficult.2 However, besides this
idiopathic, occasionally hereditary3 form of the disease, dystonic syndromes can occur in metabolic,4
anoxic,5 tumoural6 and infectious disorders.7 Dystonia of vascular origin is particularly interesting,
because the responsible lesions may be more limited
than those of metabolic or anoxic origin, thus permitting speculation on the pathological-anatomic
basis of this unique motor disease. Vascular dystonia
usually is unilateral, and frequently, but not necessarily, follows a hemiplegia.' 8-15 We report three
cases in which the CT scan enabled us to diagnose
the obliteration of a lenticulo-striate artery, causing
lesions in the putamen, to the head of the caudate
nucleus and to the anterior limb of the internal capsule.
Case Reports
Case I A 3-year-old child without any medical, surgical
or family history, had a mild head injury without loss of
consciousness in 1967; 15 minutes later, the parents
Address for reprint requests: Prof P Rondot, Service of Neurology,
Hopital Sainte Anne, 1 rue Cabanis, 75674 Paris Cedex 14,
France.

Received 6 September 1982 and in revised form 21 November
1982.
Accepted 2 December 1982.

noticed that the child was crying and that she had micturated. Two days later, a right hemiparesis with dysarthria
occurred, without any disorder of comprehension, in a
matter of several minutes. The right hemiparesis improved
over four weeks but, at the same time, dystonia appeared
in right upper and lower limbs, with athetoid movements of
the right hand. Anticholinergic treatment (trihexyphenidyl) aggravated the symptoms. In 1979, the patient
was admitted to the neurological ward of the Centre Hospitalier Saint-Anne, where clinical examination revealed
athetoid movements of the right upper limb and a dystonic
posture involving mainly the upper but also the right lower
limb. The dystonia became worse with voluntary movements. The right plantar response was extensor. The rest of
the neurological examination was normal.
CT scan showed a hypodense lesion involving the head
of the left caudate nucleus, the putamen and the anterior
limb of the internal capsule (fig 1), without any visible
arterio-venous malformation. A left carotid arteriogram
showed dilatation of the left sylvian group of vessels, with
early venous drainage and an accentuation of the small
vessels of the insular region. Surface electromyogram of
the flexor and extensor muscles of the right upper limb
showed that, during passive movements, there was a shortening reflex as well as an exaggerated myotatic reflex in
the muscles examined. Attitude fixing, stress and movement executed by other muscle groups induced bursts of
long-duration activity.
Treatment for six months with baclofen and tetrazepam
was without effect. Piribedil, under electromyographical
control, induced a hypotonia of the right half of the body,
with cessation of abnormal movements and of the muscle
shortening reflex for 15 minutes. The patient was then
treated with piribedil, baclofen and clonazepam, with a
slight improvement of symptoms.
The patient was seen again in April 1982 and the following clinical observations were made: On walking: the right
elbow was in extension, and the wrist and thumb were in
hyperflexion; the hand had the attitude of a "bowl player",
the fingers having a tendency to extension and abduction.

404

Dystonia caused by putamino-capsulo-caudate vascular lesions

405

Fig 1 Case I CT scan without contrast: hypodensity of the left lenticulo-capsulo-caudate region.
At rest: there was a generalised hypotonia of the upper
limb interrupted by marked spasms with flexion-extension
movements of the elbow and wrist. There was an exaggeration of the shortening reflex of the triceps muscle. The
position of the right lower limb was the opposite to that of
the right upper limb with a tendency to flexion of the knee
and extension of the foot, the latter setting itself in varus
equinus. The choreo-athetoid movements were exaggerated with sensory stimulation, or with emotions. There was
improvement of symptoms on treatment with piribedil,
clonazepam and dantrolene.

performing mental arithmetic. During passive movement
of the wrist, a tonic myotatic flexor response and a
shortening reaction of the extensor muscles was recorded.
CT scan showed a hypodensity of the right putaminocapsulo-caudate region without any visible arteriovenous
malformation (fig 2). The patient refused an arteriographic

examination.
We noted a mild improvement of the dystonia with an
almost complete cessation of abnormal movements on
treatment with benzodiazepine or dantrolene.

Case 3 A 17-year-old right-handed patient, without any
Case 2 A seven-year-old, left-handed schoolgirl, without medical surgical or family history developed a sudden left
hemiplegia, The patient was stuporous, with a left hemiany medical, surgical or family history, had a sudden 15
minutes loss of consciousness. On waking, a left hemiplegia plegia and left facial hypoaesthesia. Bilateral vertebral
without speech disorder was noted. Three weeks later, artery murmurs were heard. The level of consciousness
there was a partial recovery of the hemiplegia, but dystonia improved over four days, with a complete regression of the
of the left upper-limb developed with hyperflexion of the sensory disturbances and a partial regression of the motor
elbow and wrist. The foot was in varus equinus with deficit. During treatment there was one episode of a
hyperflexion of the toes. The family noted a postural tre- auricular fibrillation followed by ventricular premature
beats. On discharge, four weeks later, there was a partial
mor in the left upper-limb and, two years later, athetoid
left hemiplegia but dystonic disorders had appeared. A
type movements of the left hand.
We examined this patient in 1980 and 1982: On walk- right carotid arteriogram was normal.
Cf scan showed a hypodensity with a central hyperdening: There was a tendency to hyperextension and external
rotation of the left lower-limb with hyperflexion of the toes sity of the right lenticulo-capsulo-caudate region.
Clinical examination in 1982 showed there was on walkand a limp. There was hyperextension-anteduction of the
elbow with a tendency to separate from the body, and ing: extension of the left elbow as well as of the wrist with
hyperflexion of the wrist and hyperextension-abduction of flexion of the metacarpo-phalangeal joints. The left arm
the fingers and athetoid movements. At rest: we observed had a tendency to retropulsion-abduction with elevation of
alternately hypertonia with shortening of the triceps and the shoulders. The hand was hollow with adduction of the
biceps muscles on passive movements of the elbow and thumb, hyperextension-abduction of the first three fingers
ankle, followed by hypotonia. Voluntary movements were and athetoid movements of each of the latter. The left
executed very slowly with creeping motions of the hand lower limb was held in external rotation with hyperflexion
and wrist, pronounced "main creuse" with adduction of of the toes, without abnormal movements. At rest: The left
the thumb. The foot was in varus equinus with hyperflexion half of the body was rather hypotonic with a sudden conof the toes. Abnormal movements increased with sensory traction and shortening of the triceps on passive extension
stimulation and emotion. The left plantar response was of the elbow. The same phenomenon was noted at the left
extensor and there was a facial assymetry. The rest of the -wrist and ankle. There was a left hemiparesis with an
extensor plantar response. The rest of the neurological
neurological examination was normal.
The surface electromyogram of the deltoid, and extensor examination was normal.
Cardiological investigations showed a mitral valve proand flexor muscles of the left elbow showed tonic activity
of these muscles during attitude fixing, exaggerated by lapse, which probably was the cause of the intermittent

406

Demierre, Rondot

Fig 2 Case 2 CT scan without contrast: hypodensity of the right putamino-capsulo-caudate region.
arrhythmias. Surface electromyography of the left biceps,
triceps, extensor and flexor muscles of the wrist revealed
continued and very prolonged spontaneous activity of
these muscles. This activity was increased by stress and
voluntary movements. Furthermore, there was rhythmic
activity of these muscles at 10 Hz. Passive mobilisation of
the left elbow revealed the existence of a shortening reflex
in the biceps and triceps muscles and a tonic myotatic
reflex in the triceps. These dystonic alterations were
improved by piribedil.
A second CT scan two years after the initial crisis
showed a hypodensity of the right lenticulo-capsulo caudate region (fig 3).

Discussion
The putamino-capsulo-caudate lesions observed in
the three preceding cases were due to ischaemia in
the territory of the lenticulo-striate artery near its
origin from the middle cerebral artery, before
penetrating the anterior perforated substance.
According to Salamon et al'6 and to Percheron,'7

approximately 20 arterioles detach themselves from
the middle cerebral artery and divide into two
groups: the internal pedicle-giving the lenticular
arteries-and the external pedicle-giving the
lenticulo-striate arteries. These irrigate the putamen, the external pallidum, the anterior limb of the
internal capsule, to terminate in the body and posterior part of the head of the caudate nucleus.'7-'9
After experimental occlusion, the extent of the
ischaemic zone depends on lepto-meningeal anastomosis or on anastomosis between internal or
external lenticulo-striate arteries.'9
The lesions causing contralateral limb dystonia
were confined to a relatively limited region. In the
19 cases of unilateral dystonia, assembled in tables 1
(anatomical confirmation of site of lesion) and 2
(neuroradiologically suggested site of lesion), the
caudate nucleus and putamen were involved 13
times. In these cases, the lesions extended to the
anterior limb of the internal capsule, with exception
of cases no 1 and no 2, but the latter lacked exact

Fig 3 Case 3 CT scan without contrast: hypodensity of the right putamino-capsulo-caudate region.

Dystonia caused by putamino-capsulo-caudate vascular lesions

407

Table 1 Unilateral dystonia with anatomical verification of site of lesion
Interval of
onset of
dystonia

Authors

Sex

Age at onset
of initial
disease
(yr)

Most probable
aetiology

Initial
neurological
deficit

1-Austregesilo,
Borges-Forte24

F

68

stroke

6

epileptic fit

hypotonia
immediate
demential state
Mutism?
9 yr
R. post-ictal

8

angioma

dysarthria
sudden loss of

2-Denny-Brown'
3-Oppenheimer9

F

4-Dooling, Adams'° M

5-Dooling, Adams'° M

2

6

syphilis

hemiplegia

consciousness
l. hemiparesis

(arteritis)

sudden r
hemiparesis

fever

r hemiplegia
and aphasia

(24h onset)

followed by
epileptic fit

6-Dooling, Adams'0 M

5

fever with
convulsions

post-ictal
aphasia and
r.

7-Dooling, Adams'0 M

8-Dooling, Adams'0

F

2

3

scarlet fever
syphilis

hemiplegia

hypotonia

Type of extra- Site of lesion
pyramidal signs

torsion spasms head CN
r
with choreiform Put
movements
Pall
of
CN
dystonia
trunk rUL, rLL Put
Thal
?
1UL dystonia CN
with "repetitive Put
Ext Cap
spasms"
Ant L, Int Capr
Pall
Amygd N
Thal
10 yr
rUL, rLL
head CN
dystonia with Put
Ant L, Int Cap
athetoid
movements
Pall
Amygd N
Thal
14 y
rUL, rLL
head CN
Put
dystonia with
Ant and Post L
"'Involuntary
of Int Cap
spasms"
Ext Pall
Thal
several months dystonia of
head CN
trunk rUL and Put
rLL with
Ant and Post L 1
athetoid
of Int Cap
movements
Ext Pail
Thal
?
rUL, rLL
head CN
dystonia
Put
Ant and Post L
of Int Cap
Thal
CN
16 yr
1UL, 1LL
dystonia with Put, Thal
athetoid
Ant and Post L>
of Int Cap
movements
J

1

i}

convulsive crisis post-ictal
1. hemiplegia

r = right, 1 = left, UL = upper limb, LL = lower limb, L = limb, CN = caudate nucleus, Put = putamen, Pail = pallidum,
Thal = thalamus, Ant = anterior, Int cap = internal capsule, Post = posterior, Ext = external, Amygd = amygdaloid, M = male,
F = female

information. In the majority of observations (14 out
of 19) the lesions occurred before 10 years of age,
often quite early in childhood. This is common in
athetosis. A hemiplegia usually precedes the occurrence of the dystonia (14 cases), and was present in
our three cases. Dystonia developed either immediately following the hemiplegia (cases no 9, 14, 17,
18, 19) or much later (cases no 2, 4, 5, 8, 11, 12,

13).
The ischaemia occurring in young individuals can
be attributed either to head injury (7 cases) particularly children under 10 years of age, to vascular
malformations (cases no 3, 17) or to embolism (case
no 19). An initial temperature rise led certain
authors to suspect an arteritic cause (cases no 4, 7).
The mechanism responsible for elective involvement of lenticulo-striate arteries following head

injury, has been suggested by Maki et al20 as being a
torsion of the pedicle. Oppenheimer9 believed that
vascular malformations could cause ischaemia
through spasm, even in the absence of a proven
haemorrhage. The predominance of anoxic lesions
in this zone is accounted for by the particular sensitivity of these structures to anoxia.20.
The three preceding cases and other similar ones
reported by the literature, make it possible to suggest that lesions of caudate nucleus, putamen and
anterior limb of internal capsule may induce dystonia. This most often is the case if the lesion occurs
during childhood. Indeed of 11 cases of capsular
infarct in hypertensive patients reported by Fisher,22
even though seven cases where located in the putamen and anterior limb of the internal capsule, not
one developed dystonia, whether or not the caudate

Demierre, Rondot

408

Table 2 Unilateral dystonia with site of lesion based on CT scan image except case no 9 (pneumoencephalography) and
case no 11 (no neuroradiological examination).
Authors

Sex

Age at onset
of initial
disease

Most probable
aetiology

Initial
neurological
deficit

Interval of
onset of
dystonia

Type of extra- Site of lesion
pyramidal signs

1. hemiplegia
(several hours
later)
none

4 weeks

torsion dystonia ?
"with spasms"

4 yr

torsion dystonia r head CN
IUL with
choeiform
movements
rUL and rLL
?
dystonia with
athetoid
movements
IUL and ILL
r. middle cerebral
dystonia
artery territory
IUL and ILL
no visible lesion
dystonia
1. athetoid
CN
Put.
movements
Ant L
spasmodic
Jr
of Int. Cap.
torticollis
IUL and ILL
Put
I
dystonia with Post L of
J
Int Cap
athetoid

(yr)
9-O'Callaghan8

F

10

head injury

10-Messimy et al."

M

21

head injury

11-Burke et al. 2

F

2

head injury

r. hemiparesis
aphasia

6 yr

12-Burke et al. 12

F

65

stroke

1 yr

13-Burke et al. 12

M

54

stroke

sudden 1.
hemiparesis
1. hemiparesis

14-Maki et al.20

F

5

head injury
followed by
epileptic fit

1. hemiparesis

6h
3 days

15-Brett et al. '4

M

5

head injury

none

1 week

1 6-Andrews et al.'5

M

8

head injury

none

24 h

17-Personal case

F

3

head injury
Angioma?

r. hemiparesis 1 mon
dysarthria
(48 hours later)

(Case 1)
18-Personal case

F

6

(Case 2)
19-Personal case
(Case 3)

M

17

discomfort with sudden
loss of
1. hemiplegia
consciousness

1 mon

sudden
1. hemiplegia

1 mon

embolic stroke

nucleus was involved. Likewise, no dystonic syndrome occurred in 11 patients over 50 years of age
reported by Damasio et at,23 with ischaemic lesions
in the same territory. It is extremely rare for dystonia to occur when such a lesion develops in
adults.'2 24

We thank the following for their invaluable contributions: Professor D Fredy, neuroradiogist, Dr N
Bathien electromyographist, Dr K Strommer for the
translation of this text.

References
'Denny-Brown D. The basal ganglia. London: Oxford
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1 yr

movements

IUL torsion
head CN
dystonia, 1. oro- lentiform n
r
facial dystonia, Ant. L of
J
IUL hemiballic Int Cap
and ILL
myoclonic
movements

rUL and rLL
dystonia with
choreo-athetoid
movements

head CN
Put
Ant L of
Int Cap
Ext Pall
postural tremor head CN
Put
IUL and ILL
dystonia with
Ant L of
choreo-athetoid Int Cap
Ext Pall
movements
IUL and ILL
head CN
dystonia with
Put
Ant L of
athetoid
Int Cap
movements
Ext Pall

|
J

I

r

J
r

J

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