New antidepressive and antipsychotic drugs in juvenile neuronal ceroid lipofuscinoses--a pilot study

Eur J Paediatr Neurol. 2001;5 Suppl A:163-6. doi: 10.1053/ejpn.2000.0455.


Patients with juvenile neuronal ceroid lipofuscinosis (JNCL) often have severe psychiatric symptoms. These are common in their mid-teens and include such symptoms as anxiety and affective and psychotic disorders. The older antidepressants and antipsychotics do not seem to be effective and often cause many adverse effects. Therefore, we wanted to try the new psychotropic drugs in Finnish patients with JNCL. We also wanted to determine the profile of these drugs in this patient group. Fourteen Finnish patients with JNCL receiving psychotropic drug treatment with citalopram, risperidone, olanzapine or quetiapine, were included. The mean age at initiation of the new psychotropic drugs was 13.8 years. Indications for treatment were psychotic symptoms, affective symptoms, anxiety and an inadequate response to other psychotropic drugs, or even adverse reactions. Information on psychiatric symptoms and current treatment was gathered from interviews and from the medical records. Indications and the clinical outcome of the treatment were determined by a consensus of the assessments by parents and physicians. The psychotropic drugs most commonly used in Finnish patients with JNCL are citalopram and risperidone. The clinical outcome was good or satisfactory in 70%. The adverse effects most commonly reported were fatigue, weight gain and aggravation of extrapyramidal symptoms. Little research has been done in this area and there are no good guidelines for treatment of psychiatric symptoms in patients with JNCL. Therefore, every patient should be treated with the safest and most commonly used drugs in the lowest possible doses.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antidepressive Agents, Second-Generation / administration & dosage*
  • Antidepressive Agents, Second-Generation / adverse effects
  • Antipsychotic Agents / administration & dosage*
  • Antipsychotic Agents / adverse effects
  • Benzodiazepines
  • Child
  • Citalopram / administration & dosage*
  • Citalopram / adverse effects
  • Dibenzothiazepines / administration & dosage
  • Dibenzothiazepines / adverse effects
  • Female
  • Humans
  • Male
  • Neuronal Ceroid-Lipofuscinoses / drug therapy*
  • Olanzapine
  • Pilot Projects
  • Pirenzepine / administration & dosage
  • Pirenzepine / adverse effects
  • Pirenzepine / analogs & derivatives
  • Quetiapine Fumarate
  • Risperidone / administration & dosage*
  • Risperidone / adverse effects


  • Antidepressive Agents, Second-Generation
  • Antipsychotic Agents
  • Dibenzothiazepines
  • Citalopram
  • Benzodiazepines
  • Quetiapine Fumarate
  • Pirenzepine
  • Risperidone
  • Olanzapine