Successful treatment of psychosis in a patient with Kufor-Rakeb syndrome with low dose aripiprazole: a case report

Neurocase. 2019 Jun-Aug;25(3-4):133-137. doi: 10.1080/13554794.2019.1625928. Epub 2019 Jun 24.


We present a case of a 32-year-old male with Kufor-Rakeb syndrome (KRS), a form of juvenile parkinsonism due to mutations of the ATP13A2 gene at PARK9 locus. The patient was seen for daily behavioral outbursts and psychotic symptoms. At first assessment, CGI scale was estimated at 5; "Markedly ill". Aripiprazole was started at 2 mg and then increased to 3 mg. Two years later, psychotic symptoms were judged to be "much improved" (CGI-C = 2). This significant improvement without drug-induced motor side effects suggests that aripiprazole at low doses (2-5 mg) is effective and tolerated in patients with KRS.

Keywords: Aripiprazole; Kufor-Rakeb syndrome; juvenile parkinsonism; neuronal ceroid lipofuscinosis; psychosis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antipsychotic Agents / therapeutic use*
  • Aripiprazole / therapeutic use*
  • Humans
  • Male
  • Parkinsonian Disorders / complications*
  • Psychotic Disorders / drug therapy*
  • Psychotic Disorders / etiology*
  • Treatment Outcome


  • Antipsychotic Agents
  • Aripiprazole

Supplementary concepts

  • Kufor-Rakeb syndrome