Paroxysmal choreoathetosis precipitated by movement,
sound and photic stimulation in a case of arterio-venous
malformation in the parietal lobe
Shuzo Shintani* * *, Z e n j i Shiozawa*, Shinichi Tsunoda*, and T a t s u o Shiigai* * *

Introduction
Summary

The movement-induced brief attack of dystonia
and choreoathetosis was initially described by
Gowers 1, which was named paroxysmal kinesigenic choreoathetosis (PKC) by Kertesz2. We
present here a case of paroxysmal choreoathetosis precipitated by movement, sound and photic
stimulation associated with an arterio-venous
malformation (AVM). This case seems to be
interesting in considering the mechanism of this
disorder.
Case history

A 17-year-old Japanese high school girl first
noticed paroxysmal kinesigenic involuntary
movements when she was 15 year old. One day
in December 1983, when she heard the ringing
sound of a telephone and began to walk to answer it, she suddenly experienced involuntary
movements in her right extremities. The movements included dystonic and choreoathetotic
components. Her right arm was flexed at the
elbow and carpal joints with dystonic posture
and choreoathetotic movements were observed
in her right fingers. Her right leg was extended
at the knee and ankle joints with dystonia.
These episodic movements lasted, without disturbance of consciousness or continence, for

A patient is presented with paroxysmal choreoathetosis precipitated by movement, sound
and photic stimulation associated with an arterio-venous malformation (AVM) who responded to carbamazepine treatment.
Hemodynamic circulatory disorder of the sensory-motor cortices having AVM may alter
striatal function and produce paroxysmal choreoathetosis. This finding supports the concept
that paroxysmal choreoathetosis results from
an abnormality at the connections between
basal ganglia and cerebral cortices.
Key words: Paroxysmal choreoathetosis,
sound and photic stimulation, arterio-venous
malformation (AVM)

about 15 seconds and then she was thrown off
balance and fell to the floor. Almost daily for
two years, she has been affected by this attack at
least once or twice a day, sometimes 20-30 times
when she begins to walk.
Concerned with sound-induced paroxysmal
choreoathetosis, one day when she was sitting to
play the "solovan" (Japanese antique calculating machine made of wood) with her classmates
in school, about 5 seconds after she heard many
clicking sounds of the machine, choreo-athetot-

* Third Department oflnternal Medicine (Neurology), Yamanashi Medical University, 409-38 Yamanashi, Japan;
* * Department of Neurology, and * * *Department of Internal Medicine, Toride Kyodo General Hospital, 5901-1, Terada
Toride, 302 Japan
Address for correspondence and reprint requests: Dr. S. Shintani, Department of Neurology, Toride Kyodo General Hospital,
5901-1, Terada Toride, 302 Ibaraki, Japan
Accepted 21-8-90
Clin Neurol Neurosurg 1991. Vol. 93-3

237

Figure 1: Late arterial phase of the left internal carotid angiography. Subependymalveins (arrow head) in the area of the
branches of the ascendingparietal artery are remarkably shown in this stage.
ic involuntary movements of her right fingers
and dystonia in flextion of her right arm appeared. This episodic attack abnormality continued during the phonic stimulation and lasted
for about 10 minutes even after the clicking
sounds disappeared. She failed to continue playing "solovan". This attack has been provoked
once or twice a week, once or twice a day maximally for the last two years.
Family history was unremarkable. She had no
past hisoty of epilepsy or other neuromuscular
disorders.
On admission, Feb. 11, 1985, she was physically normal, except for a moderate degree of
obesity. Neurological examination revealed no
abnormalities except for a mild motor weakness
in her right extremities.
In the hospital, paroxysmal choreoathetosis
in her right extremities were provoked many
times a day when she began to walk, lasting for
10-20 seconds. The other form of paroxysmal
choreoathetosis could be induced by continuous
photic stimulations of flashlights (3-20 c/s) to her
right eye using an E E G apparatus and also by
238

phonic stimulations of clicking sounds (60-70
c/s) to her right ear using an auditory apparatus,
but not provoked by alcohol, coffee, aminophylline, caffeine, startle stimulations, or fatigue. Anti-convulsants, such as 200mg carbamazepine daily, were effective to prevent these
phenomena.
Routine laboratory studies including a CBC,
multiphasic chemical analysis, urinalysis, roentgenograms of the skull and chest, and an E C G
demonstrated no abnormalities. Plain and enhanced CTs and cerebro-spinal fluid were normal. E E G showed bifrontal slow waves (4-5Hz)
with theta activities (5-7Hz) in bilateral centroparietal areas.
Selective internal carotid angiographies demonstrated that subependymal veins appeared in
the area of several branches of the ascending
parietal artery in the late arterial phase [Figure
1]. This abnormal arteriovenous malformation
was observed in the parietal lobe including sensory-motor cortices in the left hemisphere. Cerebral arterial vessels seemed to be rather tortuously running.

Discussion

In our case, the paroxysmal choreoathetosis was
provoked by photic and phonic stimulation as
well as sudden movement. The attack of 10-20
minutes duration seems to be the intermediate
type of paroxysmal kinesigenic choreoathetosis
and paroxysmal dystonic choreoathetosis described by Lance in 19773.
There has been 0nly one report to my knowledge of paroxysmal choreoathetosis induced by
dermal sensory stimulation. Plant et al described a 34-year-old housewife with paroxysmal choreoathetosis provoked by dermal Sensory stimulation, in this case the application of a
vibrator to the ankle 4. Although photic stimulation has not been previously reported to induce a paroxysmal choreoathetosis, focal E E G
paroxysms contralateral to the side of the movements has been reported 5.
The acquired paroxysmal choreoathetosis has
been described in cerebral p a l s y 6'7, in multiple
sclerosis 8,9, after head injury 1~ and in metabolic
diseases including thyrotoxicosis 11, idiopathic
hypoparathyroidism 12'13 and hypoglycemia TM.
There have been no reports of paroxysmal choreoathetosis associated with arterio-venous
malformation (AVM).
In revewing the literature, there have been
only two reports of neuroradiological abnormalities in PKC. Watson initially reported a 22year-old man with PKC who had severe enlargement of the fourth ventricle and moderate enlargement of the circummesencephalic cisterns
in computed tomographic findings 15. The other
case of PKC with abnormal CT findings was
described by Gilroy 16. CT with iodine enhancement showed an abnormality in the right frontotemporal area with minimal attenuation but no
enhancement appeared. In our case, cerebral
angiography revealed an AVM in the left parietal lobe including sensory and motor cortices.
The pathophysiology of paroxysmal choreoathetosis has been poorly understood. It is not
possible to define the pathological conditions,
such as the gliosis of the basal ganglia, according
to the two autopsied cases of P K C 2't7. Reviewing
literature, there were two opinions regarding
the pathogenesis of PKC; one was an epileptic 1'18'19 and the other was an extra-pyramidal
disorder 7'~2'~7. Lobo-Antunes et al reported ex-

trapyramidal
dysfunction
with
cerebral
AVMs 2~ In our case, hemodynamic circulatory
disorder of the sensory-motor cortices with
AVM may alter striatal function and produce
paroxysmal choreoathetosis, when she begin s to
walk and receives continuous visual and auditory stimulation.
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