Kabat and McLeod, Connecticut Medicine (1959),185 reported the successful use of PHT in five of six athetosis patients. Treatment with PHT resulted in prompt and striking improvement in neuromuscular performance.

185. Kabat, H. and McLeod, M., Neuromuscular dysfunction and treatment in athetosis, Conn. Med., 23: 710-714, 1959.

Stevens, Archives of Neurology (1966), 351 reported that PHT treatment was usually promptly effective in the relief of symptoms of paroxysmal choreoathetosis.

351. Stevens, H., Paroxysmal choreo-athetosis: a form of reflex epilepsy, Arch. Neurol., 14: 415-420, 1966.

Hudgins and Corbin, Brain (1966),549 treated a mother, son and daughter suffering from familial paroxysmal choreoathetosis with PHT and mephobarbital. The relief was prompt and lasting with continued treatment.

549. Hudgins, R. L. and Corbin, K. B., An uncommon seizure disorder: familial paroxysmal choreoathetosis, Brain, 89: 199-204, 1966.

Kertesz, Neurology (1967),191 reported ten patients with paroxysmal kinesigenic choreoathetosis as an entity within the paroxysmal choreoathetosis syndrome. The attacks consisted of athetoid movements or tonic posturing of limbs, trunk and face. Duration was usually fifteen to thirty seconds. Paroxysms were precipitated by sudden movements, associated with surprise or haste. The author reported that the majority of patients responded well to PHT.

191. Kertesz, A., Paroxysmal kinesigenic choreoathetosis: an entity within the paroxysmal choreoathetosis syndrome, Neurology, 17: 680-690, 1967.

Jung, Chen and Brody, Neurology (1973),1200 reported ten cases of paroxysmal choreoathetosis in two families. The authors state that, to their knowledge, this is the first report of its occurrence among the Chinese. Episodes may occur several times daily with varying degrees of bizarre posturing, which can reach such intensity that the patient is hurled to the floor. The authors state that the therapeutic effect of PHT is so prompt and so dramatic that there is little doubt as to the effectiveness of the treatment. They state that, except for one early report, PHT has been the drug of choice for this disorder.

1200. Jung, S. S., Chen, K. M., Brody, J. A., Paroxysmal choreoathetosis: report of Chinese cases, Neurology, 23: 749-55, 1973.

Zenteno Vacheron, Carrasco Zanini and Ramos Ramirez, Epilepsy Abstracts (1977),2137 described the successful use of PHT in treating two patients with paroxysmal dystonic choreoathetosis.

2137. Zenteno Vacheron, J. S., Carrasco Zanini, J. and Ramos Ramirez, R., Paroxysmal dystonic choreoathetosis. A form of reflex epilepsy (two sporadic cases), Epilepsy Abstracts, 10(3): 84, 1977.

Waller, American Journal of Psychiatry (1977),2111 described a case of paroxysmal kinesigenic choreoathetosis in a twenty-two-year old female whose condition was markedly improved by PHT.

2111. Waller, D. A., Paroxysmal kinesigenic choreoathetosis or hysteria? Am. J. Psychiat., 134(12): 1439-40, 1977.

Goodenough, Fariello, Annis and Chun, Archives of Neurology (1978),1866 reported the complete cessation of symptoms of from two to eight years duration in three cases of familial kinesigenic dyskinesia treated with PHT. The authors state that the response to PHT was prompt.

1866. Goodenough, D. J., Fariello, R. G., Annis, B. L. and Chun, R. W. M., Familial and acquired paroxysmal dyskinesias, Arch. Neurol., 35: 827-31, 1978.

Fukuyama, Ochiai, Hayakawa and Miyagawa, Neuropadiatrie (1979),1841 reported the complete elimination of choreoathetoid attacks in an eight-year-old boy with PHT. Marked improvement in sleep was also observed.

1841. Fukuyama, Y., Ochiai, Y., Hayakawa, T. and Miyagawa, F., Overnight sleep EEG and cerebrospinal fluid monoamines in seizures induced by movement, Neuropadiatrie, 10(2): 138-49, 1979.

Homan, Vasko and Blaw, Neurology (1980),1899 report on the use of PHT in the treatment of five patients with paroxysmal kinesigenic choreoathetosis. The patients, two children and three adults, experienced episodes of choreoathetoid posturing without alteration of consciousness. PHT controlled these symptoms in all cases. Discontinuation of PHT resulted in a return of symptoms.

1899. Homan, R. W., Vasko, M. R. and Blaw, M., Phenytoin plasma concentrations in paroxysmal kinesigenic choreoathetogis, Neurology, 30: 673-76, 1980.

Franssen, Fortgens, Wattendorff and Van Woerkom, Archives of Neurology (1983),2508 report a study of an eighteen-year-old male patient with paroxysmal kinesigenic choreoathetosis of a year’s duration. Attacks were precipitated by anticipated movements, confirmed by enhancement of the slow negative wave component of the contingent negative variation. With PHT, the attacks of paroxysmal choreoathetosis disappeared and the slow negative wave amplitude became normal.

2508. Franssen, H., Fortgens, C., Wattendorff, A. R., Van Woerkom, C. A., Paroxysmal kinesigenic choreoathetosis and abnormal contingent negative variation, Arch. Neurol., 40: 381-5, 1983.

Plant, Journal of Neurology, Neurosurgery and Psychiatry (1983),2875 presented three cases of unilateral and one case of bilateral paroxysmal kinesigenic choreoathetosis. In all four patients the attacks were completely abolished by PHT. There were no recurrences during an eighteen-month follow-up.

2875. Plant, G., Focal paroxysmal kinesigenic choreoathetosis, J. Neural. Neurosurg. Psychiatry, 46: 345-8, 1983.

Zacchetti, Sozzi and Zampollo, Italian Journal of Neurological Sciences (1983),3098 report the treatment of a patient with paroxysmal kinesigenic choreoathetosis. Treatment with PHT, 200 mg/day, led to complete disappearance of the attacks, confirmed in follow-up. It was of interest that symptoms were controlled with PHT blood levels of 3 µg/ml.

3098. Zacchetti, O., Sozzi, G., Zarnpollo, A., Paroxysmal kinesigenic choreoathetosis. Case report, ltal. J. Neurot. Sci., 3: 345-7, 1983.

Wang and Chang, Therapeutic Drug Monitoring (1985),3058 reported the effectiveness of PHT at varying therapeutic blood levels in the control of paroxysmal choreoathetosis in eight patients. Successful treatment in all eight patients was defined as complete control of attacks within six months, at which time blood levels were measured. The doses used ranged from 50 to 200 mg per day. Effective blood levels ranged from 1.1 to 10.9 µg /ml. The authors emphasize that, in the treatment of disorders other than epilepsy, lower doses of PHT are often effective. See also Refs. 718, 1305, 1431, 1610, 2339, 2366, 2721, 2809.

3058. Wang., B. J., Chang, Y. C., Therapeutic blood levels of phenytoin in treatment of paroxysmal choreaoathetosis, Ther. Drug. Monit., 7(l): 81-2, 1985.
718. Williams, J. and Stevens, H., Familial paroxysmal choreaathetosis, Pediatrics, 31: 656-659, 1963.
1305. Loong, S. C. and Ong, Y. Y., Paroxysmal kinesigenic choreoathetosis, J. Neurol. Neurosurg. Psychiat., 36: 921-924, 1973.
1431. Pryles, C. V., Livingston, S. and Ford, F. R., Familial paroxysmal choreoathetosis of Mount and Reback, Pediatrics, 9: 44-47, 1952.
1610. Tassinari, C. A. and Fine, R. D., Paroxysmal choreoathetosis, Proc. Aust. Assoc. Neurot., 6: 71-75, 1969.
2339. Bird, T. D. Carlson, C. B., Horning, M., Ten year follow-up of paroxysmal choreoathetosis: a sporadic case becomes familial, Epilepsia, 19: 129-32, 1978.
2366. Burger, L. J., Lopez, R. I., Elliott, F. A., Tonic seizures induced by movement, Neurology, 22: 656-9, 1972.
2721. Lishman, W. A., Symonds, C. P., Whitty, C. W., Willison, R. G., Seizures induced by movement, Brain, 85: 93-108, 1962.
2809. Mount, L. A., Reback, S., Familial paroxysmal choreoathetosis, Arch. Neurol. Psychiatry, 44: 841-7, 1940.

Wang, Zhonghua Shen Jing Jing Shen Ke Za Zhi (1989), 3190 report twenty cases of paroxysmal kinesigenic choreoathetosis. The patients had frequent, brief (lasting no longer than 3 minutes), tonic, dystonic or choreoathetoid attacks. The attacks were triggered not only by sudden initiation of movements following a period of inactivity, but also by sudden increase in speed, amplitude, force strength and a sudden addition of new actions during steady movements in progress. The paroxysms reached their climax during puberty with a frequency of 30-100 times per day. The author does not provide details about his treatment of these patients but merely states that the attacks responded to phenytoin and L-dopa.

3190. Wang, J., Paroxysmal kinesigenic choreoathetosis, Zhonghua Shen Jing Jing Shen Ke Za Zhi, 22(1):44-5, 63, 1989.

Garg and Shukla, Indian Journal of Psychiatry (1993), 3191 report a 24 year-old male with a three-year history of idiopathic sporadic paroxysmal kinesiogenic choreoathetosis. His disorder was charecterized by bilateral rapid, stereotypic walking-like movements lasting 20-30 seconds and occurring several times a day. Most of the episodes were associated with physical activity, such as rising abruptly from a sitting or lying position. The patient did not lose consciousness and was able to prevent an attack, if he was mentally prepared for it. The authors found that the movements disappeared completely three days after treatment with phenytoin sodium (300 mg/day).

3191. Garg, R.K. and Shukla, R., Paroxysmal kinesiogenic chorioathetosis: A frequently misdiagnosed movement disorder, Indian J. Psychiatry, 35(2):137-38, 1993.

Hayashi, Hanyu, Yahikozawa and Yanagisawa, Electromyography and Clinical Neurophysiology (1997), 3192 studied three patients, two males and one female, with paroxysmal kinesigenic choreoathetosis (PKC) in order to determine the pattern of muscle activity of limbs during attacks and to measure postictal regional cerebral blood flow (rCBF) in the basal ganglia as an indicator of neuronal activity before and after PHT treatment. In all three patients, PHT treatment dramatically improved the symptoms and reduced the difference in rCBF between the right and left basal ganglia. The rCBF in the patients who experienced unilateral PKC attacks in the right extremities was higher in the left basal ganglia than in the right basal ganglia before treatment (11% in patient 1 and 6% in patient 2). Treatment with PHT improved symptoms and reduced the difference in rCBF between the right and left basal ganglia to 5% in patient 1, to 1% in patient 2. Patient 3 experienced bilateral attacks that were more severe on the right than the left side of the body. The rCBF was 4% higher in the left basal ganglia than in the right basal ganglia before treatment. This difference was reduced to 0.1% after PHT treatment.

3192. Hayashi, R., Hanyu, N., Yahikozawa, H., Yanagisawa, N., Ictal muscle discharge pattern and spect in paroxysmal kinesigenic choreoathetosis, Electromyogr. Clin. Neurophysiol., 37: 89-94, 1997.

See also Ref.

3193. Fukuda, M., Hashimoto, O., Nagakubo, S., and Hata, A., A family with an atonic variant of paroxysmal kinesigenic choreoathetosis and hypercalcitoninemia, Mov. Disord., 14(2):342-344, 1999.

3194. Nair, K.R., Paroxysmal hypnogenic dystonia responsive to phenytoin, Mov.Disord., 5(2): 180-1, 1990.