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Paroxysmal choreoathetosis precipitated by movement, sound and photic stimulation in a case of arterio-venous malformation in the parietal lobe
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. References 1
2
3
4
5
6 7
8 9 10 11
12 13
14
is
16
17 18
19 20
GOWERSWR. Epilepsy and Other Chronic Convulsive Diseases. (Reprint of 1885 edition.) New York, Dover, 1964, pp. 75-6. KERTESZA. Paroxysmal kinesigenic choreoathetosis: An entity within the paroxysmal choreoathetosis syndrome: Description of ten cases, including one autopsied. Neurology 1967; 17:680-690. LANCEJW. Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977; 2:285-293. PLANT GT, WILLIAMS AC, EARL CJ, MARSDEN CD. Familial paroxysmal dystonia induced by exercise. J Neurol Neurosurg Psychiatry 1984; 47:275-9. JACOME DE, RISKO M. P h o t i c i n d u c e d - d r i v e n
PLEDs
in
paroxysmal dystonic choreoathetosis. Clin Electroenceph 1984; 15:151-4. LANCEJW. Sporadic and familial varieties of tonic seizures. J Neurol Neurosurg Psychiatry 1963; 26:51-9. ROSENJA. Paroxysmal choreoathetosis associated with perinatal hypoxic encephalopathy. Arch Neurol 1964; 11:385-7. JOYNTILl, GREEN D. Tonic seizures as a manifestation of multiple sclerosis. Arch Neurol 1962; 6:293-9. MATrEws WB. Tonic seizures in disseminated sclerosis. Brain 1958; 81:193-206. ROBEN JJ. Paroxysmal choreoathetosis following head injury. Ann Neurol 1977; 2:447-8. FISHBECK KH, LAYZER RB. Paroxysmal choreoathetosis associated with thyrotoxicosis. Ann Neurol 1979; 6:453-4. ARDEN F. Idiopathic hypoparathyroidism. Med J Aust 1953; 2:217-9. TOBALE MS, FROME B, KAPPHAHA K. Kinesigenic choreoathetosis and idiopathic hypoparathyroidism. N Engl J Med 1972; 286:762-3. NEWMAN RP, KINKEL WR. Paroxysmal choreoathetosis due to hypoglycemia. Arch Neurol 1984; 41:341-2. WATSONRT, sCOTT W~. Paroxysmal kinesigenic choreoathetosis and brain stem atrophy. Arch Neurol 1979; 36:522. OILROVJ. Abnormal computed tomograms in paroxysmal kinesigenic choreoathetosis. Arch Neurol 1982; 39:779-780. STEVENSn. Paroxysmal choreoathetosis: A form of reflex epilepsy: Arch Neurol 1966; 14:415-420. FUKUYAMA Y, OKEDA R. Hereditary kinaesthetic r e f l e x epilepsy: report of five pedigrees with seizures induced by movement and review of literature. Proc Aust Assoc Neurol 1968; 5:583-7. LISHMAN WA, SYMONDS CP, WHITTY CWM, e t al. Seizures induced by movement. Brain 1962; 85:93-108. LOBO-ANTUNES J, YAHR MD, HILAL SK. Extrapyramidal dysfunction with cerebral arteriovenous malformation. J Neurol Neurosurg Psychiatry 1974; 259-268.
239
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. References 1
2
3
4
5
6 7
8 9 10 11
12 13
14
is
16
17 18
19 20
GOWERSWR. Epilepsy and Other Chronic Convulsive Diseases. (Reprint of 1885 edition.) New York, Dover, 1964, pp. 75-6. KERTESZA. Paroxysmal kinesigenic choreoathetosis: An entity within the paroxysmal choreoathetosis syndrome: Description of ten cases, including one autopsied. Neurology 1967; 17:680-690. LANCEJW. Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977; 2:285-293. PLANT GT, WILLIAMS AC, EARL CJ, MARSDEN CD. Familial paroxysmal dystonia induced by exercise. J Neurol Neurosurg Psychiatry 1984; 47:275-9. JACOME DE, RISKO M. P h o t i c i n d u c e d - d r i v e n
PLEDs
in
paroxysmal dystonic choreoathetosis. Clin Electroenceph 1984; 15:151-4. LANCEJW. Sporadic and familial varieties of tonic seizures. J Neurol Neurosurg Psychiatry 1963; 26:51-9. ROSENJA. Paroxysmal choreoathetosis associated with perinatal hypoxic encephalopathy. Arch Neurol 1964; 11:385-7. JOYNTILl, GREEN D. Tonic seizures as a manifestation of multiple sclerosis. Arch Neurol 1962; 6:293-9. MATrEws WB. Tonic seizures in disseminated sclerosis. Brain 1958; 81:193-206. ROBEN JJ. Paroxysmal choreoathetosis following head injury. Ann Neurol 1977; 2:447-8. FISHBECK KH, LAYZER RB. Paroxysmal choreoathetosis associated with thyrotoxicosis. Ann Neurol 1979; 6:453-4. ARDEN F. Idiopathic hypoparathyroidism. Med J Aust 1953; 2:217-9. TOBALE MS, FROME B, KAPPHAHA K. Kinesigenic choreoathetosis and idiopathic hypoparathyroidism. N Engl J Med 1972; 286:762-3. NEWMAN RP, KINKEL WR. Paroxysmal choreoathetosis due to hypoglycemia. Arch Neurol 1984; 41:341-2. WATSONRT, sCOTT W~. Paroxysmal kinesigenic choreoathetosis and brain stem atrophy. Arch Neurol 1979; 36:522. OILROVJ. Abnormal computed tomograms in paroxysmal kinesigenic choreoathetosis. Arch Neurol 1982; 39:779-780. STEVENSn. Paroxysmal choreoathetosis: A form of reflex epilepsy: Arch Neurol 1966; 14:415-420. FUKUYAMA Y, OKEDA R. Hereditary kinaesthetic r e f l e x epilepsy: report of five pedigrees with seizures induced by movement and review of literature. Proc Aust Assoc Neurol 1968; 5:583-7. LISHMAN WA, SYMONDS CP, WHITTY CWM, e t al. Seizures induced by movement. Brain 1962; 85:93-108. LOBO-ANTUNES J, YAHR MD, HILAL SK. Extrapyramidal dysfunction with cerebral arteriovenous malformation. J Neurol Neurosurg Psychiatry 1974; 259-268.
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