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The 4-month-old baby girl who has had persistent left eye blinking with ipsilateral head rotation since birth undergoes epilepsy examination (video).
MRI showed a mass in the left upper foot of the cerebellum (Fig.
A), and PET/CT showed lesions and occipital lobe hypermetabolism
.
Scalp EEG is normal
.
During surgical resection, electrodes are placed directly in the lesion and seizure activity derived from it is recorded (panel C).
Histopathology confirms the diagnosis of gangliomatous hamartoma (Figure D).
The child did not have seizures
after surgery.
Cerebellar lesions rarely cause epilepsy manifested as hemifacial spasm; Possible mechanisms are dentorubrothalamic tract disruption and compression of the facial nerve nucleus
.
(Figure: Coronal T2WI[A] showing dilated lesions [white arrows] in the upper foot of the left cerebellum; Surgical image [B] showing tumor [white arrow] extending into the fourth ventricle between cerebellar hemispheres; Intraoperative EEG [C] with superficial [a], deep [b], and facial muscle [c] electrodes confirms seizure activity within the lesion; Micrograph [D, hematoxylin and eosin staining, ×400] shows hypercellular hyperarrangement, disorderly arrangement, with dysplastic neurons [long black arrows] and astrocytes [long double-headed arrows]) within the fibrous background matrix)