Paroxysmal Hemifacial Contractions in an Infant: Think of a Fourth Ventricle Mass

Background: The mother of this female infant noticed since the first week of life episodes of eyelid blinking and eye closure lasting a few seconds. The symptomatology was more pronounced on the right side. The left eye usually remained partly open and variable eye deviation was observed. In addition the right corner of the mouth was pulled downwards and an unusual respiration pattern was present during these clusters of episodes lasting about 10–20 seconds. The infant developed normally but interrupted her habitual activity like eating or playing during these episodes, which could not be stopped by the parents and happened during awake and sleep more than 100 times per day.

Material and Methods: Case report with video documentation.

Results: EEG examinations during the episodes were normal. At the age of 7 months MRI revealed a brain tumour located at the right cerebellar peduncle/fourth ventricle. Treatment with oral anticonvulsants was without effect on the frequeny of the seizures.

Conclusion: Since 1976 about twenty cases were published with this highly specific history, clinical findings and imaging. Antiepileptic drugs are not helpful, although an epileptic background is discussed. Patients usually became „seizure free“ after surgery. Histological findings were mainly hamartoma, ganglioglioma and gangliocytoma. Epileptic surgery procedure with complete lesionectomy should be forced in these patients. The timing of the surgery is controversial because of the potential complications especially at a young age.