title: Carbamazepine and oxcarbazepine in adult patients with Dravet syndrome: Friend or foe?
abstract: OBJECTIVE
  In newly diagnosed patients with Dravet syndrome sodium channel blockers are usually avoided. However, in many adult patients the diagnosis was made long after the initiation of therapy. The purpose of our study was to acquire information concerning the potential risks and benefits of (ox)carba(ma)zepine withdrawal in adult patients with genetically confirmed Dravet syndrome.
  
  
  METHODS
  We identified 16 adults with Dravet syndrome, living in a tertiary care facility for people with epilepsy and an intellectual disability. We reviewed clinical history, genetic findings, the type and duration of sodium channels blockers that were used, seizure types and frequency, and the effect of a change in these medications.
  
  
  RESULTS
  The study population consisted of 9 men and 7 women. Median age was 35 years (range 20-61 years). An attempt to withdraw carbamazepine (CBZ) was made in 9 patients. In 3 of these patients an increase in tonic-clonic seizures was observed. An attempt to withdraw oxcarbazepine (OXC) was made in 3 patients, leading to a complete stop in 2 patients. 3 of the 4 deaths in the withdrawal-group were related to epilepsy.
  
  
  CONCLUSIONS
  In adult patients with Dravet syndrome withdrawal of CBZ or OXC is not without risks. We suggest that (ox)carba(ma)zepine withdrawal should be considered in these patients but only if there is a good reason to do so and only if they are closely monitored.
authors.affiliation: Department of Residential Care, Kempenhaeghe, The Netherlands. Electronic address: SchouwenaarsF@kempenhaeghe.nl.
authors.forename: F M
authors.initials: FM
authors.lastname: Snoeijen-Schouwenaars
authors.affiliation: Department of Residential Care, Kempenhaeghe, The Netherlands.
authors.forename: M J B M
authors.initials: MJ
authors.lastname: Veendrick
authors.affiliation: Department of Neurology, Academic Center for Epileptology Kempenhaeghe/Maastricht University Medical Center, The Netherlands.
authors.forename: P
authors.initials: P
authors.lastname: van Mierlo
authors.affiliation: Department of Neurology, Academic Center for Epileptology Kempenhaeghe/Maastricht University Medical Center, The Netherlands.
authors.forename: G
authors.initials: G
authors.lastname: van Erp
authors.affiliation: Department of Neurology, Academic Center for Epileptology Kempenhaeghe/Maastricht University Medical Center, The Netherlands.
authors.forename: A J A
authors.initials: AJ
authors.lastname: de Louw
authors.affiliation: Department of Neurology, Academic Center for Epileptology Kempenhaeghe/Maastricht University Medical Center, The Netherlands.
authors.forename: B U
authors.initials: BU
authors.lastname: Kleine
authors.affiliation: Department of Neurology, Academic Center for Epileptology Kempenhaeghe/Maastricht University Medical Center, The Netherlands.
authors.forename: H J
authors.initials: HJ
authors.lastname: Schelhaas
authors.affiliation: Department of Residential Care, Kempenhaeghe, The Netherlands.
authors.forename: I Y
authors.initials: IY
authors.lastname: Tan
mesh_terms: D000328:Adult; D000927:Anticonvulsants / Q000009:adverse effects / Q000627:therapeutic use*; D002220:Carbamazepine / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D004831:Epilepsies, Myoclonic / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality; D005260:Female; D006801:Humans; D007297:Inpatients; D008297:Male; D008875:Middle Aged; D062550:NAV1.1 Voltage-Gated Sodium Channel / Q000235:genetics; D000078330:Oxcarbazepine; D012189:Retrospective Studies; D012640:Seizures / Q000188:drug therapy / Q000235:genetics / Q000401:mortality; D013375:Substance Withdrawal Syndrome; D062606:Tertiary Care Centers; D061567:Voltage-Gated Sodium Channel Blockers / Q000009:adverse effects / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D000927:Anticonvulsants; D062550:NAV1.1 Voltage-Gated Sodium Channel; C568242:SCN1A protein, human; D061567:Voltage-Gated Sodium Channel Blockers; D002220:Carbamazepine; D000078330:Oxcarbazepine
keywords: Adults; Carbamazepine; Dravet syndrome; Epilepsy; Oxcarbazepine; Withdrawal