A case of Dravet syndrome with cortical myoclonus indicated by jerk-locked back-averaging of electroencephalogram data

Yoshinori Kobayashi, Yoshiyuki Hanaoka, Tomoyuki Akiayma, Iori Ohmori, Mamoru Ouchida, Toshiyuki Yamamoto, Makio Oka, Harumi Yoshinaga, Katsuhiro Kobayashi

Research output: Contribution to journalArticle

Abstract

We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk-locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46. ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age. She suffered from recurrent afebrile or febrile generalized tonic-clonic seizures that often developed into status epilepticus. Interictal EEG and brain magnetic resonance imaging (MRI) showed no significant findings. The amplitudes of the somatosensory-evoked potentials were not extremely large. She has a chromosomal microdeletion involving . SCN1A and adjacent genes.

Original languageEnglish
JournalBrain and Development
DOIs
Publication statusAccepted/In press - 2016

Fingerprint

Myoclonic Epilepsy
Myoclonus
Electroencephalography
Seizures
Muscles
Somatosensory Evoked Potentials
Status Epilepticus
Forearm
Cerebral Cortex
Fever
Magnetic Resonance Imaging
Brain
Genes

Keywords

  • Cortical myoclonus
  • Dravet syndrome
  • EEG
  • Jerk-locked averaging

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

Cite this

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title = "A case of Dravet syndrome with cortical myoclonus indicated by jerk-locked back-averaging of electroencephalogram data",
abstract = "We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk-locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46. ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age. She suffered from recurrent afebrile or febrile generalized tonic-clonic seizures that often developed into status epilepticus. Interictal EEG and brain magnetic resonance imaging (MRI) showed no significant findings. The amplitudes of the somatosensory-evoked potentials were not extremely large. She has a chromosomal microdeletion involving . SCN1A and adjacent genes.",
keywords = "Cortical myoclonus, Dravet syndrome, EEG, Jerk-locked averaging",
author = "Yoshinori Kobayashi and Yoshiyuki Hanaoka and Tomoyuki Akiayma and Iori Ohmori and Mamoru Ouchida and Toshiyuki Yamamoto and Makio Oka and Harumi Yoshinaga and Katsuhiro Kobayashi",
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AU - Kobayashi, Yoshinori

AU - Hanaoka, Yoshiyuki

AU - Akiayma, Tomoyuki

AU - Ohmori, Iori

AU - Ouchida, Mamoru

AU - Yamamoto, Toshiyuki

AU - Oka, Makio

AU - Yoshinaga, Harumi

AU - Kobayashi, Katsuhiro

PY - 2016

Y1 - 2016

N2 - We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk-locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46. ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age. She suffered from recurrent afebrile or febrile generalized tonic-clonic seizures that often developed into status epilepticus. Interictal EEG and brain magnetic resonance imaging (MRI) showed no significant findings. The amplitudes of the somatosensory-evoked potentials were not extremely large. She has a chromosomal microdeletion involving . SCN1A and adjacent genes.

AB - We report a female patient with Dravet syndrome (DS) with erratic segmental myoclonus, the origin of which was first identified in the cerebral cortex by the detection of myoclonus-associated cortical discharges. The discharges were disclosed through jerk-locked back-averaging of electroencephalogram (EEG) data using the muscle activity of myoclonus as triggers. The detected spikes on the contralateral parieto-central region preceded myoclonic muscle activity in the forearms by 28-46. ms. The patient was six months old at the time of examination, and was developing normally before seizure onset at two months of age. She suffered from recurrent afebrile or febrile generalized tonic-clonic seizures that often developed into status epilepticus. Interictal EEG and brain magnetic resonance imaging (MRI) showed no significant findings. The amplitudes of the somatosensory-evoked potentials were not extremely large. She has a chromosomal microdeletion involving . SCN1A and adjacent genes.

KW - Cortical myoclonus

KW - Dravet syndrome

KW - EEG

KW - Jerk-locked averaging

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