Congential hemifacial spasmis a rare condition that is characterized by the occurrence of paroxysmal hemifacial contractions in neonates. We review the clinical, neurophysiological, neuroimaging, and histopathological findings, as well as the differential diagnosis, therapeutic approach, and outcome of all the described cases. Moreover, we report two new cases including the ictal video-electroencephalography recordings. Hemifacial spasm starts early in life, and is characterized by unilateral, involuntary, irregular tonic or clonic contractions ofmuscles innervated by the seventh cranial nerve. Hemifacial spasmis associated with eyelid blinking, and sometimes with breathing irregularities, hyperventilation, and ⁄ or other neurological manifestations (dystonicmovements, nystagmus). Interictal and ictal video-electroencephalography did not reveal epileptiform abnormalities. In all cases, brain magnetic resonance imaging showed amass involving the cerebellar peduncle, the cerebellar hemisphere, or the floor of the fourth ventricle. The semiology of the paroxysmal attacks is probably due to the activation of cranial nerve nuclei through intralesional hypersynchronous discharges, as shown by the intraoperative recordings and functional brain imaging described in the literature.We point out the importance of identifying such seizures in order tomake an early diagnosis of the underlying cerebral lesion.
Neonatal hemifacial spasm and fourth ventricle mass
FAGGIOLI, Raffaella;FIUMANA, Elisa;
2012
Abstract
Congential hemifacial spasmis a rare condition that is characterized by the occurrence of paroxysmal hemifacial contractions in neonates. We review the clinical, neurophysiological, neuroimaging, and histopathological findings, as well as the differential diagnosis, therapeutic approach, and outcome of all the described cases. Moreover, we report two new cases including the ictal video-electroencephalography recordings. Hemifacial spasm starts early in life, and is characterized by unilateral, involuntary, irregular tonic or clonic contractions ofmuscles innervated by the seventh cranial nerve. Hemifacial spasmis associated with eyelid blinking, and sometimes with breathing irregularities, hyperventilation, and ⁄ or other neurological manifestations (dystonicmovements, nystagmus). Interictal and ictal video-electroencephalography did not reveal epileptiform abnormalities. In all cases, brain magnetic resonance imaging showed amass involving the cerebellar peduncle, the cerebellar hemisphere, or the floor of the fourth ventricle. The semiology of the paroxysmal attacks is probably due to the activation of cranial nerve nuclei through intralesional hypersynchronous discharges, as shown by the intraoperative recordings and functional brain imaging described in the literature.We point out the importance of identifying such seizures in order tomake an early diagnosis of the underlying cerebral lesion.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
