Tonic eyelid movements in a patient with hypothalamic hamartoma

Aims: Is the symmetric tonic close up of the eyes in a suckling with hypothalamic hamartoma epileptic or not?

Methods: In a 5 month old otherwise healthy suckling there were described repeated episodes with crying sometimes accompanied by opisthotonoid head deviation since birth. During the following months he showed more and more a tonic movement of the whole body and fisting of both hands. Later on he showed in growing number and intensity a forced closing of both eyes with distinct photophobia.

Results: Already the MRI showed a hypothalamic hamartoma at the bottom of the III. ventricle. Because of the clinical findings with abrupt start and end of the symptomatology with symmetric close up of the eyes with painful facial expression we concluded an epileptic genesis. We performed a video eeg for several hours. Parallely to the clinical symptoms we could not find epileptiform discharges or a seizure pattern in the surface eeg.

Because we suspected a deeper epileptogenic focus in the region of the lesion we started a therapy with LEVETIRACETAME (225mg/d equal to 29mg/kgBW). Even after one day we saw a reduction of frequency and intensity of the clinical symptoms. Rarely only the patient showed short symmetric tonic movements of his eyebrows. The symptomatology slightly increased again. Therefore we increased LEVETIRACETAME up to 550mg/d equal to 61mg/kgBW). That again influenced the seizure situation positive until discharge.

Conclusion: Hamartomas are benigne malformations with local growing of tissue during postnatal development. Rarely they occur in the brain. Frequently there is a correlation with precocious puberty (production of GnRH) or typically gelastic seizures. Hamartomas as reason for epilepsies even with other seizures types are well known but uncommon in very young children.

We took the symptomatology with forced closing of both eyes as series of tonic seizures caused by the hypothalamic hamartoma. Even if the surface eeg didn't show any changes parallelly to the clinical symptoms we think that the positive effect of LEVETIRACETAME on seizure frequency proofed the epileptic genesis.