Hyperactive delirium in an 11-year-old boy with juvenile neuronal ceroid lipofuscinosis and a SARS-CoV-2 infection

ABSTRACT

An 11-year-old boy with juvenile neuronal ceroid lipofuscinosis (JNCL) is admitted because of acute agitation and hallucinations. Upon admission, the patient takes lorazepam, which does not induce the expected rest. A PCR-test had a positive result for SARS-CoV-2. Juvenile neuronal ceroid lipofuscinosis (JNCL) is a rare neurodegenerative disease in children and adolescents. Hallucinations are a known symptom in the course of the disease. In the case discussed in this article, however, the pronounced hallucinations fit within a broader clinical picture of a hyperactive delirium. A delirium is by definition provoked by a physical cause. In the presented case, JNCL was an existing risk factor for a delirium, the SARS-CoV-2 infection and lorazepam were presumably the triggering factors. Recent literature shows that an asymptomatic or mildly symptomatic SARS-CoV-2 infection can also trigger a delirium. Treatment consists of treating the physical cause (if possible), supportive measures for the patient and context, as well as medication. The antipsychotics risperidone and haloperidol are recommended. Within the context of JNCL, cautious initiation of a second-generation antipsychotic, such as risperidone, along with great alertness to possible side effects, such as extrapyramidal symptoms and neuroleptic malignant syndrome, are advised. For the young patient in the discussed case risperidone was started, supplemented with olanzapine as rescue medication. The medication had a good effect and no side effects were observed.