Table 4 Case example of PWS mUPD and psychosis

JJ, a 21-year-old male with PWS due to mUPD, lives at home with his parents and attends a vocational day program. He is healthy following a weight loss of 103 lb at age 17 that occurred during and after a stay in an in-patient hospital specializing in PWS. At the time of his follow-up research visit, JJ weighed 136 lb, was 5 ft tall, and taking antipsychotic medications. His parents were diligent with his diet and locking food. JJ was quite proud of his weight loss and tended to open conversations with, “I lost 103 lb, are you proud of me?”

JJ’s parents reported increased agitation and aggressive behavior aimed at both them and his program staff (e.g., hitting, pushing, verbal threats). He was emotionally labile, laughing, for example, over something funny “in my head” and then within seconds being verbally abusive. At age 14 JJ experienced auditory and visual hallucinations, including an episode when his parents found him sitting naked on a couch outside their bedroom door, seeing and talking to make believe cartoon characters. He was subsequently placed on anti-psychotic medications but continued to have looseness of thoughts and magical thinking.

During his visit to the lab, JJ hit the staff and then demanded that they repeat the phrase “No, I did not hit you”, and he became enraged when they did not exactly comply. JJ’s speech was repetitious, nasal, pressured and perseverative, and sprinkled with demands that the staff repeat phrases verbatim that were unrelated to context, e.g., “Yes, that really looks like blue green.” JJ believed that others could know what he was thinking even if he did not say it aloud and he became angry when the staff would not acknowledge his powers and believed that they were making fun of him. He was not oriented to person or place and insisted on eating lunch at his favorite restaurant some 600 miles away.

JJ’s cognitive level (FSIQ = 41) had declined dramatically since his first psychotic episode at age 14 when he was functioning in the borderline range (FSIQ = 77). His parents were quite concerned that his escalating and bizarre behavior would not allow him to continue in the vocational day program and they feared that they would need to quit working in order to take care of him. The research team referred JJ to a PWS residential program for evaluation and possible placement as his escalating behaviors and thought disturbances required a more intensive treatment approach.