Cariprazine in the treatment of a long lasting psychosis in a female patient with Morbus Niemann-Pick Type B

Introduction M. Niemann Pick type A and B is caused by an autosomal recessive inherited gene defect located on chromosome 11 and is a lysosomal disease. Due to the reduced activity of acid sphingomyelinase damage of liver, spleen and lungs occurs, especially in patients with type A the CNS is commonly affected. These affection of the CNS is uncommon in patients with type B. Nevertheless some descriptions of treatment resistant psychosis in patients with M. Niemann Pick type B can be found.

Methods We report on a 30 year old female patient with M. Niemann Pick type B who is treated in our specialized outpatient clinic for mental health in rare diseases since early 2018. She suffered from psychotic symptoms such as optic and acoustic hallucinations, delusion and periodic temper tantrums. Besides the mild mental retardation she was diagnosed with epilepsy and aortic valve insufficiency III.

Results and Conclusion Treatment was initialized using quetiapine as monotherapy, then in combination with aripiprazole, augmented with pipamperone. None of these did show any improvement, but dysphagia occurred as a side effect which had occurred in earlier years when she was treated with risperidone. After changing medication to cariprazine we observed a noticeable decrease of psychotic symptoms. Even regular visits at a day care center were possible.