Abstract
Five cases of tuberous sclerosis with rotational movement or backward or forward falling attacks following psychomotor seiures were studied.
We noted in all cases subependymal nodules on the thalamostriate sulcus. Three girls with unilateral nodules rotated to the side contralateral to the nodules; two boys with bilateral nodules fell backwards or forwards. Pharmaco-behavioral observation and polysomnography (PSG) were conducted to elucidated the pathophysiology of these phenomena. PSG findings in one case revealed a pattern of dopaminergic hyperactivity. Furthermore, small doses of L-dopa exacerbated the rotational attacks and induced right-left asymmetry in the incidence of twitch movement (TM) of the limbs on PSG, whereas pimozide alleviated both rotational attacks and asymmetric TM.
These results suggest that the observed rotational or falling attacks in tuberous sclerosis are mostly caused by dopaminergic hyperactivity, the direction of rotation depending on the nodule location.
