Nocturnal frontal lobe epilepsy in mucopolysaccharidosis.
Nocturnal frontal lobe epilepsy (NFLE) is an epileptic syndrome that is primarily characterized by seizures with motor signs occurring almost exclusively during sleep. We describe 2 children with mucopolysaccharidosis (MPS) who were referred for significant sleep disturbance. Long term video-EEG monitoring (LT-VEEGM) demonstrated sleep-related hypermotor seizures consistent with NFLE. No case of sleep-related hypermotor seizures has ever been reported to date in MPS. However, differential diagnosis with parasomnias has been previously discussed. The high frequency of frontal lobe seizures causes sleep fragmentation, which may result in sleep disturbances observed in at least a small percentage of MPS patients. We suggest monitoring individuals with MPS using periodic LT-VEEGM, particularly when sleep disorder is present. Moreover, our cases confirm that NFLE in lysosomal storage diseases may occur, and this finding extends the etiologic spectrum of NFLE.
Epilepsy and Clinical Neurophysiology Unit, IRCCS "E. Medea", Conegliano, TV, Italy. Electronic address: paolo.bonanni@cn.lnf.it.
Paolo
P
Bonanni
Epilepsy and Clinical Neurophysiology Unit, IRCCS "E. Medea", Conegliano, TV, Italy.
Anna
A
Volzone
Epilepsy and Clinical Neurophysiology Unit, IRCCS "E. Medea", Conegliano, TV, Italy.
Giovanna
G
Randazzo
Epilepsy and Clinical Neurophysiology Unit, IRCCS "E. Medea", Conegliano, TV, Italy.
Lisa
L
Antoniazzi
Department of Paediatrics, University of Padova, Italy.
Angelica
A
Rampazzo
Department of Paediatrics, University of Padova, Italy.
Maurizio
M
Scarpa
"Claudio Munari" Epilepsy Surgery Centre, Niguarda Ca'Granda Hospital, Milan, Italy.
Lino
L
Nobili
D001921:Brain / Q000503:physiopathology; D002648:Child; D003937:Diagnosis, Differential; D004569:Electroencephalography; D017034:Epilepsy, Frontal Lobe / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D006801:Humans; D009083:Mucopolysaccharidoses / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D017286:Polysomnography; D012893:Sleep Wake Disorders / Q000175:diagnosis; D014741:Video Recording
Epilepsy; Mucopolysaccharidosis; Sleep disturbances