Nocturnal frontal lobe epilepsy in mucopolysaccharidosis

Paolo Bonanni, Anna Volzone, Giovanna Randazzo, Lisa Antoniazzi, Angelica Rampazzo, Maurizio Scarpa, Lino Nobili

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)826-829
Number of pages4
JournalBrain and Development
Issue number9
Publication statusPublished - Oct 1 2014


  • Epilepsy
  • Mucopolysaccharidosis
  • Sleep disturbances

ASJC Scopus subject areas

  • Clinical Neurology
  • Developmental Neuroscience
  • Pediatrics, Perinatology, and Child Health
  • Medicine(all)


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