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Rotatory seizures in a patient with tuberous sclerosis Volume 1, numéro 4, Décembre 1999

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  • Rotatory seizures in a patient with tuberous sclerosis

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Seizures in which the patients rotate several times in a circle around their own axis are called rotatory seizures and are very rare. The direction of the rotatory phenomenon remains constant with 2 to 5 circles and is usually contralateral to the EEG focus. Frequently, the seizure is followed by a generalized tonic-clonic attack. This form of epilepsy is mostly associated with focal EEG (frontal or temporal) and cerebral abnormalities.

We have recently observed an 11-year-old, white girl with tuberous sclerosis and rotatory seizures originating from the right temporal lobe. Tuberous sclerosis is a dominantly transmitted disorder with a variable expression usually presenting with the triad of mental retardation, epilepsy, and sebaceous adenomas. MRI showed a characteristic subcortical hamartoma in the right temporal region.

The purpose of this communication is to describe the association of rotatory seizures with tuberous sclerosis.

Case report

An 11-year-old girl was admitted to our hospital for treatment of intractable convulsive seizures. There was no personal or family history of any neurological disorders. The patient was born by caesarean section after an uneventful pregnancy.

Following delivery, the course was uneventful. After the age of six months she presented with febrile convulsions. At the age of three years she started to have partial seizures, with speech arrest, a fixed gaze and mouth automatisms. These attacks lasted from 20 seconds to one minute. In spite of therapy with several antiepileptic drugs (phenobarbital, carbamazepine, valproic acid and lamotrigine) the frequency of the seizures could not be reduced. The interictal EEG demonstrated a spike focus in the right temporal region. Neurological examination showed no focal signs, but typical skin lesions. She demonstrated facial angiofibromas (adenoma sebaceum), that occupied symmetrical areas over the cheeks, nasolabial folds and chin. In addition she had multiple, achromic naevi and a "shagreen patch" on the forehead. Routine hematological and biochemical investigations were normal. During hospitalization she presented an attack during which she was standing and started turning to the left (see videotape). EEG showed the onset of focal epileptiform activity. Above the right fronto-temporal region (electrodes F8/T4/T6), in the form of a rhythmic theta-alpha activity. The girl appeared to be confused, slowly raised her left arm over her head and turned her head to the left. The focal epileptiform activity increased progressively in amplitude as the girl presented hand automatisms persisting for about 2 minutes. Thereafter she left the bed, rose to her feet, still confused. Five minutes after the beginning of the seizure; she opened her mouth, turned her head with a jerk to the left and started to rotate around her own body axis to the left. She turned around six times without falling. She returned to bed and her behavior again appeared relatively normal. The right frontal spike activity decreased progressively over 30 seconds; the diffuse high amplitude theta-delta activity was progressively replaced (within about 10 minutes) by a normal 9 Hz alpha activity.

A T2-weighted MRI showed multiple areas of increased signal in the temporo-parietal areas corresponding to tubers and bilateral periventricular nodules (figure 1). Therapy with topiramate was begun and her seizures disappeared. She has remained well on follow-up for one year and attends a special school.

Discussion

Our patient with tuberous sclerosis fits the description of rotatory seizures in that she rotated around her own axis. Gastaut et al. (1986) presented the concept of two types of giratory epilepsy [1]. The common form, which occurs mainly in childhood or adolescence, features bilateral rythmic, symmetrical, or asymmetrical 3-Hz EEG slow-wave discharges, associated with generalized seizures and no demonstrable pathology. This type is possibly a benign variant of primary generalized epilepsy [1-3]. Rotatory seizures are, by contrast, a very rare manifestation of simple partial seizures and secondarily generalized tonic-clonic seizures, as seen in our patient. Between 1968 and 1998, 24 cases of children with rotatory seizures have been reported in 9 reports [4-12]. The clinical, electroencephalographic and neuroradiological findings observed in these 24 patients as well as in our case are summarized in table I.

As in our patient, in most of the cases the direction of rotation was contralateral to the epileptogenic focus and only three patients showed rotation towards the side of the epileptogenic activity [4, 5, 9]. Twelve patients rotated around their own axis to the left side and 9 patients to the right. The direction was unknown in three patients. The direction of the rotatory phenomenon remained constant in all seizures. Most patients rotated 3 to 5 times in a "ballet-like fashion". This complex circling behavior is usually observed at the beginning of the attack when the patient is standing and it may be the sole expression of the seizure. In such cases, the patients remain conscious. More frequently, secondary tonic-clonic generalization follows with loss of consciousness.

The rotatory seizure is not the only expression of the epilepsy as it is frequently preceded or followed by other epileptic manifestations.

All previously reported cases have been documented with an interictal EEG and the majority showed an epileptogenic focus localized temporally (7/24); frontally (9/24); frontotemporally (3/24) and fronto-parientally (3/24). To our knowledge, our patient is the only one in which an ictal EEG with focal sharp and slow waves over the right fronto-temporal region could be documented. The body rotation was the terminal part of a complex partial seizure which probably started in the right temporal region and diffused to the right frontal region.

Although circling occurs with primary generalized epilepsy, asymmetry of convulsive seizure activity with predominance in one cerebral hemisphere seems necessary for its production. The rotational activity requires more than one unilateral temporal lobe epileptic discharge. Vercueil presented a patient with rotatory seizures in whom intracerebral EEG recording showed activation of the basal ganglia [13].

Electrical stimulation of the caudate nucleus in cats produces head turning and body and circling towards the opposite side [14]. Irritation of the vestibular cortex has been shown to cause circling movements in animal experiments [15].

The role of the striatum and of the temporal lobe in the genesis of these rare epileptic phenomena is controversial and not clear.

CONCLUSION

Acknowledgement:

The authors would like to thank Jane McDougall for her assistance in preparing this manuscript.