Abstract
In order to clarify the neural mechanisms that contribute to the age dependency of epileptic seizures in ADEE, we examined sleep structures in four patients before and after administration of haloperidol (HAL) or L-DOPA. Case 1 suffered from tuberous sclerosis and developed West syndrome at 5 months. At 2 years tonic seizures developed and HAL administration at 14 years caused reduction of seizures for one month. Case 2 suffered from neonatal seizures. At 8 years he developed Lennox syndrome and also showed the sleep-wake rhythm disturbance followed by an increase of epileptic seizures. At 14 years, HAL was administered in addition to anticonvulsants, and seizures decreased for 2 months. Case 3 was born with asphyxia and developed West syndrome at 10 months. At 8 years tonic spasms and tonic seizures started. The seizures disappeared for one month by the administration of HAL at 14 years. Case 4 suffered from tuberous sclerosis. She developed West syndrome and Lennox syndrome at 10 months and 5 years, respectively. The seizures were improved by administration of L-DOPA at 10 years of age.
Polysomnographical examination was performed before and after administration of HAL or L-DOPA. Body movements (BM) were classified into two types; gross movement (GM) involving the body trunk and lasting for more than two seconds, and twitch movement (TM) localized in one muscle on surface EMG recordings and lasting less than 0.5 second.
The frequency of TM of mentalis muscle during sleep in cases 1 and 3 was high, and it decreased to the control level by HAL administration. In contrast to this, the frequency of GM and TM was low in Case 4, and TM increased after L-DOPA. Our previous study revealed that in ADEE the frequency of BM was low during early childhood and paradoxically increased thereafter. The present study confirmed that a dopamine antagonist suppressed the paradoxical increase of TM in ADEE and decreased seizures. In contrast, the administration of HAL before this paradoxical increase rather enhanced the seizure activity while the administration of L-DOPA showed beneficial effects.
