title: Autosomal Dominant Cortical Tremor, Myoclonus, and Epilepsy Syndrome mimicking Juvenile Myoclonic Epilepsy.
abstract: BACKGROUND
  Autosomal dominant cortical tremor, myoclonus, and epilepsy (ADCME) syndrome is a genetically heterogeneous and under-recognized disease characterized by tremulous movements mimicking essential tremor, myoclonus, and rare generalized tonic-clonic seizures. Here we described the clinical and electrophysiological features of three siblings with ADCME syndrome mimicking juvenile myoclonic epilepsy (JME).
  
  
  METHODS
  Three siblings (two females and one male) diagnosed with ADCME were analyzed by electroencephalogram (EEG), somatosensory evoked potentials, and accelerometric recordings. The results were compared with 14 JME patients without tremor and 14 with essential tremor (ET).
  
  
  RESULTS
  The shared features of the siblings were cortical tremor, myoclonia, epilepsy, migraine, and psychiatric symptoms. In all siblings, tremor had started before myoclonic epilepsy associated with 4-6 Hz generalized spike and wave discharges. The N20-P25 and P25-N35 amplitudes were substantially higher in the three siblings with ADCME. Although tremor frequencies were similar to those of the ET group, the siblings had mild interrupting low-amplitude myoclonus, suggestive of cortical tremor, in the accelerometric analysis.
  
  
  CONCLUSIONS
  We presented a detailed clinical evaluation with electrophysiological confirmation of ADCME syndrome in a Turkish family. This rare clinical picture might be misdiagnosed as JME and should be kept in mind to ensure correct diagnosis and to provide a homogenous group for genetic studies.
authors.affiliation: Departments of Neurology and Clinical Neurophysiology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Aydin Özemir
authors.affiliation: Departments of Neurology and Clinical Neurophysiology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
authors.forename: Emel
authors.initials: E
authors.lastname: Oğuz Akarsu
authors.affiliation: Departments of Neurology and Clinical Neurophysiology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
authors.forename: Zeliha
authors.initials: Z
authors.lastname: Matur
authors.affiliation: Departments of Neurology and Clinical Neurophysiology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
authors.forename: Ali Emre
authors.initials: AE
authors.lastname: Öge
authors.affiliation: Departments of Neurology and Clinical Neurophysiology, İstanbul University İstanbul School of Medicine, İstanbul, Turkey.
authors.forename: Betül
authors.initials: B
authors.lastname: Baykan
keywords: Cortical tremor; epilepsy; myoclonus