title: Long-term follow-up of cortical hyperexcitability in Japanese Unverricht-Lundborg disease.
abstract: OBJECTIVE
  To delineate chronological changes of cortical hyperexcitability by long-term follow-up of the amplitudes of somatosensory evoked potentials (SEPs) in patients with Japanese Unverricht-Lundborg disease (ULD).
  
  
  METHODS
  SEPs to median nerve stimulation were repeatedly examined in 7 genetically diagnosed ULD patients with the mean interval of 11.9 years. The degree of temporal changes in the amplitude of 3 early cortical components, N20, P25 and N35, to the age was analyzed and compared with that of healthy subjects.
  
  
  RESULTS
  Their clinical course was almost stable during the follow-up period, namely cessation of generalized tonic-clonic seizures and little or no progression of myoclonus. SEP amplitudes of P25 and N35 were enlarged in all patients and were gradually decreased with aging in ULD on average. The degree of temporal changes of P25 and N35 in ULD was similar or even lower than that of healthy subjects.
  
  
  CONCLUSIONS
  Enlarged but relatively stable SEP amplitudes had a consistency with so-called self-limited clinical course in Japanese ULD. SEP amplitude could be one of the surrogate markers of the degree of cortical hyperexcitability in ULD during the long-term follow-up period.
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Katsuya
authors.initials: K
authors.lastname: Kobayashi
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan; Department of Respiratory Care and Sleep Control Medicine, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Takefumi
authors.initials: T
authors.lastname: Hitomi
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan; Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Riki
authors.initials: R
authors.lastname: Matsumoto
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kondo
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Kawamata
authors.affiliation: Human Brain Research Center, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Masao
authors.initials: M
authors.lastname: Matsuhashi
authors.affiliation: Department of Neurology, Tenri Hospital, Japan.
authors.forename: Shuji
authors.initials: S
authors.lastname: Hashimoto
authors.affiliation: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Ikeda
authors.affiliation: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.
authors.forename: Yasumichi
authors.initials: Y
authors.lastname: Koide
authors.affiliation: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Japan.
authors.forename: Yushi
authors.initials: Y
authors.lastname: Inoue
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Takahashi
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Japan; Department of Epilepsy, Movement Disorders and Physiology, Kyoto University Graduate School of Medicine, Japan. Electronic address: akio@kuhp.kyoto-u.ac.jp.
authors.forename: Akio
authors.initials: A
authors.lastname: Ikeda
mesh_terms: D000293:Adolescent; D000328:Adult; D044466:Asian People; D004558:Electric Stimulation; D004569:Electroencephalography; D005073:Evoked Potentials, Somatosensory / Q000502:physiology*; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008475:Median Nerve / Q000502:physiology; D013003:Somatosensory Cortex / Q000503:physiopathology*; D018709:Statistics, Nonparametric; D020194:Unverricht-Lundborg Syndrome / Q000235:genetics / Q000473:pathology*; D055815:Young Adult
keywords: Giant SEP; Somatosensory evoked potential; Unverricht–Lundborg disease