Brain stem epilepsy, a rare disease? Case studies of two children

Seeing through the literature of the last years, you will rarely find reports about brain stem epilepsy. In 1998 Wieser, Hajek, Constantinopol, Siegel, Yonekewa and Wichmann published an article in „Klinische Neurophysiologie“ about 9 patients with a symptomatic epilepsy within a lesion of the brain stem.

During the last 2 years we have treated two patients with the diagnosis of a brain stem epilepsy (a boy, born 2001, a girl, born 2002). The main pathology was recurring vomiting associated with a clouding of consciousness appearing in clusters. Additionally both patients showed a central apnoea. Last year, due to a down-beat-nystagmus, the learning-disabled boy continued to hold his head in a more and more ducked position. This symptomatology usually occurs with lesions of cerebellum, pons or medulla oblongata. The girl, a former premature infant, triplet of the 30^th week of gestational age suffering from tetra spastic, microcephaly and retarded development additionally shows hypothermia during her seizures. The EEG did not show epileptic discharges during the seizures. Both children had ictal rhythmic Theta-Delta-waves with high amplitudes.

Both children didn't become seizure free with 6 (boy) respectively 9 (girl) anticonvulsants as mono- or combination therapy in maximum dose. With regard to the boy we have now achieved a clear reduction of seizures with topiramate. The girl suffers from a progressive athrophy of the brain stem. Her seizures cannot be influenced.

Brain steam epilepsies are underdiagnosed for sure. You should think of a brain stem epilepsy if there are clinical suspicion of epileptic seizures, pharmacoresistance and rhythmic Theta-Delta-waves with high amplitudes.