Hemifacial seizures and cerebellar ganglioglioma

In 1996 Harvey et al. reported an infant with a cerebellar ganglioglioma and episodes of hemifacial contraction that were shown conclusively to be epileptic seizures of cerebellar origin. Previous reports in the literature of six infants with cerebellar tumours and ‘hemifacial spasm’ suggested they might have a similar syndrome of cerebellar epilepsy. Since the report by Harvey et al., there have been three further cases reported (Arzimanoglou, 1996; A.S. Harvey et al., unpublished data), with further ictal SPECT evidence in two cases to support an epileptic basis to the attacks of ‘hemifacial spasm’. The clinical seizure characteristics, the location and signal characteristics of the lesions on magnetic resonance imaging (MRI), and the histopathological features of the tumours in these ten patients are so strikingly similar as to conceivably constitute an epilepsy syndrome of infancy characterized by seizures of cerebellar origin.

The initial case remains the most well-studied case in the literature, with unequivocal evidence of an epileptic basis and cerebellar origin of seizures (Harvey et al., 1996). Her seizures began on day 1 of life with twitching of the left orbicularis oculi muscle. She was evaluated at the Miami Children's Hospital at age 6 months when attacks of ‘hemifacial spasm’ were occurring multiple times each day. Immediately prior to each episode she became quiet and fixed her gaze straight ahead.