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Alterations of the contingent negative variation in a patient with paroxysmal kinesigenic choreoathetosis
139
SCIENTIFIC MEETING OFTHE'AMSTERDAMSCHENEUROLO(;ENVEREENIGING'HELDINAMSTERDAM
ONMAY
6.
1982. ALTERATIONS OF THE CONTINGENT NEGATIVE XYSMAL KINESIGENIC CHOREOATHETOSIS H.FRANSPN.C.FORTGENS.A.R.
WATTENDORF,T.(‘.
VARIATION
A.M.VAN
WOERKOM
IN A PATIENT
WITH
PARO-
(Den Haag)
The case of an 18 year-old boy with paroxysmal kinesigenic choreoathetosis (PKC) is presented. This disorder of unknown aetiology is characterized by episodes of chorea. athetosis. dystonia and/or ballismus, usually evoked by sudden movements. Generally no neurological abnormalities are found between attacks. In a minority of the cases. PKC is associated with hypoparathyreoidism. thyreotoxicosis. epilepsy. hypoxic encephalopathy and traumatic brain injury. In most cases, however. neuroradiological examinations. spinal fluid examination and laboratory investigations reveal no abnormalities. The EEG is usually normal. also during attacks. In a small number of cases a non-specific abnormality I+ found and in a minority epileptic changes occur. Most patients respond favourably to treatment with anticonvulsants. Because many patients including our patient with PCK experience more attacks when anticipating a movement. we investigated the contingent negative variation CNV) in our patient. The CNV is a slow brain potential following a warning stimulus which prepares the subject to expect an imperative stimulus. The imperative stimulus requires a motor response from the subject. In our patient two components of the CNV showed a shift in negative direction: the slow positive wave and in particular the slow negative wave. The third CNV component (Terminal CNV), which seems to be associated with motor preparation. did not show changes in amplitude. After treatment with diphenylhydantoin the attacks subsided. Parallel with the clinical improvement both CNV components mentioned shifted clearly in a positive direction. A relationship between these CNV alterations and PKC is suggested. The nature of this relationship is discussed.
SCIENTIFIC MEETING OFTHE'AMSTERDAMSCHENEUROLO(;ENVEREENIGING'HELDINAMSTERDAM
ONMAY
6.
1982. ALTERATIONS OF THE CONTINGENT NEGATIVE XYSMAL KINESIGENIC CHOREOATHETOSIS H.FRANSPN.C.FORTGENS.A.R.
WATTENDORF,T.(‘.
VARIATION
A.M.VAN
WOERKOM
IN A PATIENT
WITH
PARO-
(Den Haag)
The case of an 18 year-old boy with paroxysmal kinesigenic choreoathetosis (PKC) is presented. This disorder of unknown aetiology is characterized by episodes of chorea. athetosis. dystonia and/or ballismus, usually evoked by sudden movements. Generally no neurological abnormalities are found between attacks. In a minority of the cases. PKC is associated with hypoparathyreoidism. thyreotoxicosis. epilepsy. hypoxic encephalopathy and traumatic brain injury. In most cases, however. neuroradiological examinations. spinal fluid examination and laboratory investigations reveal no abnormalities. The EEG is usually normal. also during attacks. In a small number of cases a non-specific abnormality I+ found and in a minority epileptic changes occur. Most patients respond favourably to treatment with anticonvulsants. Because many patients including our patient with PCK experience more attacks when anticipating a movement. we investigated the contingent negative variation CNV) in our patient. The CNV is a slow brain potential following a warning stimulus which prepares the subject to expect an imperative stimulus. The imperative stimulus requires a motor response from the subject. In our patient two components of the CNV showed a shift in negative direction: the slow positive wave and in particular the slow negative wave. The third CNV component (Terminal CNV), which seems to be associated with motor preparation. did not show changes in amplitude. After treatment with diphenylhydantoin the attacks subsided. Parallel with the clinical improvement both CNV components mentioned shifted clearly in a positive direction. A relationship between these CNV alterations and PKC is suggested. The nature of this relationship is discussed.