title: Chronic high-frequency deep-brain stimulation in progressive myoclonic epilepsy in adulthood--report of five cases.
abstract: OBJECTIVE
  To assess the efficacy and tolerability of chronic high-frequency deep brain stimulation (DBS) in adult patients with progressive myoclonic epilepsy (PME) syndromes.
  
  
  METHODS
  Five adult patients (four male, 28-39 years) with PME underwent chronic high-frequency DBS according to a study protocol that had been approved by the local ethics committee. Electrodes were implanted in the substantia nigra pars reticulata (SNr)/subthalamic nucleus (STN) region in the first patient and additionally in the ventral intermediate nucleus (VIM) bilaterally in the following four cases. Follow-up took place in intervals of 3 months and DBS effects were compared with baseline frequency of passive and activation-induced myoclonic jerks and daily life performance 8 weeks prior to implantation.
  
  
  RESULTS
  Follow-up periods ranged from 12-42 months (median 24 months). The best clinical effects were seen with SNr/STN DBS in all patients. VIM stimulation failed to achieve acute therapeutic effects and revealed low side-effect thresholds and even triggering of myoclonia. In all patients the reduction of myoclonic seizures was observed and ranged between 30% and 100% as quantified by a standardized video protocol. All patients reported clinically relevant improvements of various capabilities such as free standing and walking or improved fine motor skills. In one patient with an excellent initial response generalized tonic-clonic seizures increased after 3 months of stimulation following extensive trauma-related surgery. The best effect was seen in the least impaired patient.
  
  
  CONCLUSIONS
  DBS of the SNr/STN may be an effective treatment option for patients with PME. Less impaired patients may benefit more markedly.
authors.affiliation: Division of Functional Neurosurgery, Neurosurgical Clinic, Heinrich-Heine-University, Duesseldorf, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Wille
authors.forename: Bernhard J
authors.initials: BJ
authors.lastname: Steinhoff
authors.forename: Dirk-Matthias
authors.initials: DM
authors.lastname: Altenmüller
authors.forename: Anke Maren
authors.initials: AM
authors.lastname: Staack
authors.forename: Sofia
authors.initials: S
authors.lastname: Bilic
authors.forename: Guido
authors.initials: G
authors.lastname: Nikkhah
authors.forename: Jan
authors.initials: J
authors.lastname: Vesper
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000008:administration & dosage; D003131:Combined Modality Therapy; D046690:Deep Brain Stimulation / Q000379:methods*; D004292:Dominance, Cerebral / Q000502:physiology; D004569:Electroencephalography / Q000379:methods*; D004831:Epilepsies, Myoclonic / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008134:Long-Term Care; D008297:Male; D012815:Signal Processing, Computer-Assisted; D013238:Stereotaxic Techniques; D013378:Substantia Nigra / Q000503:physiopathology; D020531:Subthalamic Nucleus / Q000503:physiopathology; D016896:Treatment Outcome; D020651:Ventral Thalamic Nuclei / Q000503:physiopathology
chemical_list: D000927:Anticonvulsants