A female infant with cerebellar ganglioglioma who developed hemifacial seizures from the first day of life is reported from the Miami Children’s Hospital, FL. When investigated at 6 months of age there were daily episodes of left hemifacial contraction, resistant to medication, head and eye deviation to the right, nystagmoid jerks to the right, autonomic dysfunction, while consciousness was retained. MRI at 2 months showed a mass in the left cerebellar hemisphere. The scalp EEG recordings were normal, while interictal and ictal intracranial EEGs revealed focal spikes, confirming seizures arising in the region of the left cerebellar mass. Partial resection of a ganglioglioma at 3 months was accompanied by remission of seizures. Six previous reports of infants with hemifacial spasms and cerebellar mass lesions are cited in the literature and reviewed, 3 having gangliogliomas. [1]

COMMENT. The authors describe a syndrome of infantile focal facial seizures associated with cerebellar ganglioglioma. Seizures associated with cerebellar tumors in infants and children have been described previously. The term “ictus infratentorialis” was coined by Penfield and Jasper for attacks of opisthotonus, syncope, vertigo, and focal clonic movements occurring in patients with infratentorial tumors. In a study at the Mayo Clinic of 291 children with intracranial tumors, seizures occurred in 17% of the total group, in 25% of those with supratentorial and in 12% of infratentorial tumors. None had gangliogliomas. [2]