Psychiatric symptoms of children and adolescents with juvenile neuronal ceroid lipofuscinosis.

BACKGROUND: Juvenile neuronal ceroid lipofuscinosis (JNCL) is one of the most common neurodegenerative disorders in childhood and adolescence. The clinical picture includes diverse and complex psychiatric symptoms that are difficult to treat. Only symptomatic treatment is available. To improve symptomatic therapy, it is important to recognize the symptoms. The purpose of this study was to identify predominant psychiatric symptoms in patients with JNCL. METHODS: The study included 27 patients with JNCL with and without psychotropic treatment. The mean age was 15.2 (range 9-21) years. Characteristic psychiatric symptoms in this patient group were clarified by using the following standardized questionnaires filled in by parents, teachers and the patients themselves: Child Behavior Checklist (CBCL), Teacher Report Form (TRF) and Children's Depression Inventory (CDI). The symptoms were recorded for the entire study group and compared between patients with and without psychotropic treatment and between genders. RESULTS: The patients had a large number of psychiatric symptoms according to the CBCL and TRF. The most commonly reported symptoms were social, thought, attention problems, somatic complaints and aggressive behaviour. Patients receiving psychotropic medication had more psychiatric symptoms according to the CBCL and TRF. Moreover, female patients had more problems than male patients according to the CBCL. The total psychiatric symptom score was at clinical or borderline range for psychiatric disturbance in 74% of patients. The number of depressive symptoms reported by the patients themselves was low. CONCLUSIONS: JNCL patients suffer from a multitude of psychiatric symptoms. To improve drug choice and dosage, a thorough evaluation of these symptoms by standardized methods is needed before initiating treatment. Progress and possible adverse effects of treatment should be monitored on a regular basis.

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

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.