Epilepsia, 32(4):492-494, 1991 Raven Press, Ltd.. New York 0 International League Against Epilepsy

Paroxysmal Kinesigenic Choreoathetosis with Abnormal Electroencephalogram During Attacks Koichi Hirata, Soichi Katayama, Tomoji Saito, Kinya Ichihashi, Tadao Mukai, Mari Katayama, and “Takashi Otaka Departments of Neurology and *Psychiatry, Dokkyo University School of Medicine, Mibu, Tochigi, Japan

Summary: An 18-year-old man with paroxysmal kinesigenic choreoathetosis (PKC) showed rhythmic electroencephalographic (EEG) discharges of 5-Hz spikes over the entire scalp during episodes. The EEG findings in this

case suggest that PKC may have an epileptogenic basis. Key Words: Paroxysmal kinesigenic choreoathetosisElectroencephalogram-Epilepsy-Seizures.

Paroxysmal kinesigenic choreoathetosis (PKC) is more common than paroxysmal dystonic choreoathetosis. While >300 cases have been reported, electroencephalographic (EEG)abnormalities have been reported only three times. We report another case.

the maximum frequency of the attacks was -80 times per day. Neurological examination revealed no abnormalities. Brain computed tomography scan and magnetic resonance imaging findings were normal. There were no blood chemistry abnormalities and no elevation of hepatic enzymes. The EEG at rest revealed no specific abnormalities. The EEG during attacks showed a rhythmic 5-Hz spike discharge over the entire scalp (Fig. 2). In a 1-h record eight attacks were observed. The duration of each attack was 4-8 s. Phenytoin and clonazepam have been very effective in suppressing the attacks, however clonazepam induced sleepiness and a consequent aversion to usage.

CASE REPORT An I&year-old man was admitted for diagnosis and control of abnormal movement induced by sudden motion. The patient had a history of chronic hepatitis. Prenatal, perinatal, and natal development were normal. There was no family history of epileptic phenomena or abnormal movement. Attacks began at age 13, usually with quick movements, for example, arising in the classroom to go before the teacher. Attacks were sometimes evoked when stepping out of an elevator. Attacks were always stereotyped. The left arm flexed at the elbow and the head assumed a dystonic posture relative to the back while the right arm extended. The fingers of both hands were in a clawing position (Fig. 1). He was often forewarned by an unusual sensation, “numbness or something like a shock,” in his arms and hands. Although there were no attacks at rest or during sleep, he experienced more frequent and severe attacks when nervous or self-conscious. The duration of the attacks ranged from 10 s to 1 min;

DISCUSSION The classification of PKC remains a matter of controversy. PKC constitutes a unique entity with features of both a convulsive disorder and a movement disorder (Kertesz, 1970). Lishman et al. (1962) and Stevens (1965) discussed the mechanism of the paroxysms and concluded that PKC should be considered a variety of “reflex epilepsy,” the chief argument being the response to antiepileptic drugs and the paroxysmal nature of the attacks. However, these highly stereotyped attacks do not resemble any of the commonly described forms of epilepsy. The tendency for tonic and athetoid rather than clonic movements has prompted authors to speculate that basal ganglia dysfunction may be the cause. The essentially normal interictal EEG in the

Received April 1990; revision accepted July 1990. Address correspondence and reprint requests to Dr. K. Hirata at Department of Neurology, Dokkyo University School of Medicine, Mibu, Tochigi 321-02, Japan.

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PKC WITH ABNORMAL EEG DURING ATTACKS

FIG. 1. A typical attack of paroxysmal kinesigenic choreoathetosis in an 18-year-old man. The left arm flexed at the elbow and the head assumed a dystonic posture relative to the back while the right arm was extended. The fingers of both hands were in a clawing position.

majority of cases, the lack of a family or past history of epilepsy, and maintenance of consciousness would tend to set this entity apart from previously recognized convulsive disorders (Kertesz, 1970). Most authors have regarded PKC as a form of reflex epilepsy involving the thalamus and basal ganglia (Lance, 1977). Mild interictal EEG abnormalities, such as bilateral slow waves, bitemporal sharp waves, or paroxysmal spike and wave discharges have been previously reported (Lishman et al., 1962). There have been three reports of abnormal EEG findings during PKC attacks. Perez-Borja et al. (1967) described one patient with a 12-15 Hz rhythmic discharge over the vertex and hemisphere during a seizure, but Lance (1977) was unable to exclude the possibility of muscle artifact. Jung et al. (1973) reported one patient with bilateral rhythmic 2-4 Hz highvoltage slow waves mixed with hypersynchronous rhythm in the alpha-wave range in the frontal area, but these EEG abnormalities are not considered

pathognomic of epileptic disorders. Kato and Araki (1969) described an autosomal recessive case who displayed diffuse spike and wave patterns in the EEG during attacks, but did not publish the EEG. Kato et al. (1970) described six cases of PKC without EEG abnormalities during attacks, but reported that some cases displayed paroxysmal spike and wave EEG discharges after activation by intravenous bemegride during which the patients described unusual sensations resembling a seizure. Pryles et al. (1952) reported a family history of epilepsy in some cases. While these reports do not constitute direct evidence that PKC is an epileptic disorder, they at least suggest that PKC is related to epilepsy. The 5-Hz EEG discharge of our patient during an attack differed from the usual pattern in epileptic seizures. The prolonged spike duration and its appearance over the entire scalp suggested that the spike originated in a deep brain region. We have no evidence suggesting that PKC is related to dysfunction of the thalamus or basal ganglia. We believe that the EEG findings in our case tend to corroborate the postulation that PKC may have an epileptogenic basis.

REFERENCES . .

EMG

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FIG. 2. The electroencephalogram during an attack of paroxysmal kinesigenic choreoathetosis displayed rhythmic 5Hz spike discharge over the entire scalp (superficial electromyogram: right biceps brachii). In this record, the discharge lasted 6 s.

Jung SS, Chen KM, Brody JA. Paroxysmal choreoathetosis: report of Chinese cases. Neurology 1973;23:749-55. Kato M, Araki S. Paroxysmal kinesigenic choreoathetosis: report of a case relieved by carbamazepine. Arch Neurol 1969;20:508-13. Kato M, Kaga M, Araki S, Kuroiwa Y. Kinesigenic seizure: Epilepsia. Vol. 32, N o . 4, 1991

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report of six cases. Clinical Neurology (Tokyo) 1970;10:299304. Kertesz A. Paroxysmal kinesigenic choreoathetosis: an entity within the paroxysmal choreoathetosis syndrome: description of 10 cases, including 1 autopsied. Neurology 1970; 17:68&90. Lance JW. Familial paroxysmal dystonic choreoathetosis and its differentiation from related syndromes. Ann Neurol 1977; 2:285-93. Lishman WA, Simmonds CP, Witty GWM, Willison RG. Seizures induced by movement. Bruin 1962;85:93-108. Perez-Borja C, Tassinari CA, Swanson AG. Paroxysmal choreoathetosis and seizures induced by movement (reflex epilepsy). Epilepsia 1967;8:260-70. Pryles CV, Livingston S, Ford FR. Familial paroxysmal choreoathetosis of Mount and Reback. Pediatrics 1952;9: 44-7. Stevens H. Paroxysmal choreo-athetosis. A form of reflex epilepsy. Arch Neurol 1965;14:415-20.

&SUME Chez un jeune homme de 18 ans prCsentant une chortoathttose paroxystique kinksigtnique (CPK), I’tlectroencCphalogramme a mis en evidence des dCcharges rythmiques de pointes

Epilepsia, Vol. 32, NO. 4 , 1991

a 5 Hz couvrant toute l’etendue du scalp pendant les Cpisodes. Des constatations EEG recueillies dans ce cas suggtrent que la CPK pourrait &re d’origine tpileptique. (P. Genton, Marseille)

RESUMEN Un varon de 18 anos de edad con coreoatetosis quinesigknica paroxistica (PKC), mostrb descargas electroencefalogrtificas clinicas de puntas de 5 Hz. en todo el cuero cabelludo durante 10s episodios. Los hallazgos del EEG en este caso sugieren que la PKC puede tener bases epileptogCnicas. (A. Portera-SAnchez, Madrid)

ZUSAMMENFASSUNG Ein 18 jahriger Mann mit paroxysmaler kinesiogener Choreoathetose (PKC) zeigte rhythmische 5 Hz Spike Entladung wahrend einer Episode iiber dem gesamten Kopf. Dieser EEGBefund lapt vermuten, dap PKC moglichenveise eine epileptogene Grundlage haben. (C. Benninger, Heidelberg)

Paroxysmal kinesigenic choreoathetosis with abnormal electroencephalogram during attacks.

An 18-year-old man with paroxysmal kinesigenic choreoathetosis (PKC) showed rhythmic electroencephalographic (EEG) discharges of 5-Hz spikes over the ...
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