Hemifacial Seizure of Cerebellar Ganglioglioma Origin: Seizure Control by Tumor Resection

Abstract

Summary:The cerebellum is known to have an inhibitory effect on seizures. Nevertheless, cerebellar dysplastic lesions can be epileptogenic. A 4-month-old infant had paroxysmal facial contractions; tachypnea and nystagmoid eyeball and tremulous movements were occasionally combined. These evolved to stereotypic clinical patterns and frequencies, which increased despite administration of antiepileptic drugs (AEDs). Magnetic resonance imaging (MRI) demonstrated a mass arising from the superior cerebellar peduncle, although video-scalp EEG monitoring revealed no abnormal findings. Positron emission tomography with [ 18 F]fluorodeoxyglucose revealed focal hypermetabolism in the same area identified by MRI. A depth electrode implanted in the mass revealed focal spike-and-wave discharges. The lesion was partly removed; pathologic diagnosis was ganglioglioma. Because of incomplete seizure control and residual tumor visible on MRI, a second operation was performed. After complete excision of the tumor, the patient became seizure free without AEDs. This case confirms the presence of seizure originating from the cerebellum and emphasizes the need for the complete removal of an epileptogenic lesion.

Tracking the source of cerebellar epilepsy: Hemifacial seizures associated with cerebellar cortical dysplasia

“…Furthermore, complete surgical resection or disconnection of the lesion result in clinical improvement with about 80% seizure freedom (Delande et al, 2001;Pontes-Neto et al, 2006). Conversely, patients with partial lesion removal did not benefit from seizure reduction after surgery (Chae et al, 2001;Park et al, 2009). Despite the fact that our patient underwent partial surgical resection, by sparing the cerebellar peduncles which were also affected, she remained seizurefree at 8 months follow-up, although a longer observation is required to better assess the outcome.…”

“…The seizure onset and symptomatogenic zones as well as the pathophysiology of such seizures related to cerebellar lesions have been largely debated in the literature, suggesting either exclusively a cerebellar role (Chae et al, 2001;Harvey et al, 1996), or a cerebellar onset with secondary spread to the brainstem (Delande et al, 2001). A recent study, using concomitant iEEG and electromyographic recordings, implied that hemifacial contractions originate in the ipsilateral cerebellar dysplastic area and propagate via the facial nucleus into the ventrolateral region of the inferior pons (Yagyu et al, 2011).…”

Chronic isolated hemifacial spasm as a manifestation of epilepsia partialis continua

“…(3-6) The former resulted from simple partial seizures of left temporal lobe origin, propagating to the left cingulate region, and resolving after surgical treatment. (3) The latter was described in children with structural lesions in the pontomedullary junction,(4) superior cerebellar peduncle, (5,6) and cerebellar hemisphere. (6) Lacking other ictal deficits and imaging abnormalities, the epileptic nature of the HFS case reported here was initially elusive given their clinical disappearance during voluntary tasks and sleep, unlike what is reported to occur in focal seizures, where motor phenomena are typically not suppressed or substantially modified by volitional tasks.…”

“…These have been recognized in a patient with simple partial seizures of temporal lobe origin (3) and in three children with associated neurological deficits and structural lesions in the ponto-medullary junction or superior cerebellar peduncle. (4)(5)(6) The objective of this report is to describe an adult case of chronic HFS without any associated neurological abnormalities, clinically similar to the segmental facial myoclonic movements of idiopathic HFS, but demonstrating an abnormal electrodiagnostic evaluation suggestive of EPC and to outline the anatomical and functional underpinnings of the irritative zone using combined EEG/fMRI.…”

Seizures in Children with Brain Tumours — Epidemiology, Significance, Management, and Outcomes

“…In addition, there is a subset of children, up to 50% [32], whose low-grade brain cancer presents as seizures [26,[32][33][34][35][36][37][38][39][40][41][42]. Though the vast majority of epileptogenic tumours are supratentorial, some are not, especially among children in whom infratentorial tumours generally comprise the majority [43,44], and in less typical locations like the thalamus and hypothalamus [38,[45][46][47]. Among thalamic tumours, for example, up to one third of paediatric patients present with seizures [38].…”