Management of autism in the adult intensive care unit.

Autism comprises a growing segment of the population and can be a management challenge in the intensive care unit (ICU). We present the case of a 22-year-old male with severe autism and intellectual disorder who developed respiratory failure and required a prolonged ICU course. This patient exhibited severe distress, aggression, and self-injurious behavior. Management challenges included sedation, weaning from sedation, and liberation from mechanical ventilation. Success was achieved with a multispecialty team and by tailoring the environment and interactions to the patient's known preferences. The use of dexmedetomidine to wean high-dose benzodiazepines and opiates also permitted successful liberation from mechanical ventilation.

Improved access to mental health evaluation for patients with developmental disabilities using telepsychiatry.

The Cedars-Sinai Telepsychiatry Clinic uses a collaborative-care model to treat patients with developmental disabilities. We examined its practice in four areas: patient characteristics, clinical care, symptom severity and diagnostic outcomes to describe the care provided and the population seen in the telepsychiatry clinic. In a chart review, 45 out of 126 cases were selected and evaluated at three times: initial evaluation, year one and year three. Most of the patients (84%) had an intellectual disability, 55% had a pervasive developmental disorder and 71% spoke approximately 50 words or less. Prior to the initial assessment, none of the patients were diagnosed with anxiety or mood disorders, while almost one-third of patients received one of these diagnoses in the telepsychiatry clinic. Patients were seen six times on average in the first year and three times in the second and third years. The telepsychiatrist recommended a change in the patient's medication for 82% of patients at initial assessment, 41% at year one and 46% at year three. The review suggests that telepsychiatry evaluations can be valuable for patients with developmental disabilities, providing diagnostic clarity and specific recommendations that can be implemented by the primary care physician.

Telepsychiatry for children with developmental disabilities: applications for patient care and medical education.

Telemedicine is able to provide specialized care to rural patients with limited access. The developmentally disabled population is highly suited to this modality and can be seen in general and specialized clinics. The collaborative care model, which is useful for this population, provides ongoing collaboration with both the local primary care provider and the local treatment team. Telepsychiatry can be used for education of trainees working together with an attending physician. These clinics can become exceptional opportunities to provide exposure to the specialty of child psychiatry for young doctors in training. As new programs develop, telepsychiatry for treatment and telepsychiatry for training should go hand in hand.

Training psychiatric residents and fellows in a telepsychiatry clinic: a supervision model.

OBJECTIVES: The authors assess the effectiveness of a specialized telepsychiatry training and supervision training model. METHODS: Fifteen residents and eight child fellows rotated through Cedars Sinai Medical Center Telepsychiatry Developmental Disability Clinic and completed questionnaires of knowledge and self-assessed skills at commencement and completion of the rotation. The supervision was on site, side-by-side, and directive. RESULTS: Both the residents and the fellows demonstrated improvement. Increase in knowledge was equal in the study cohorts, while residents' self-assessed skills were significantly greater than the fellows'. CONCLUSION: A telepsychiatry clinic appears to be an appropriate setting in which to provide direct supervision. Exposure to such opportunities early in training may yield a greater impact.

Clinical and behavioral features of patients with Borjeson-Forssman-Lehmann syndrome with mutations in PHF6.

OBJECTIVE: To describe clinical and behavioral features of 10 men from 2 families with Borjeson-Forssman-Lehmann syndrome (BFLS) and missense mutations in the PHF6 zinc-finger transcription factor gene. STUDY DESIGN: BFLS behavioral features were compared with other age-matched men with other syndromes and similar intellectual functioning through the use of standardized questionnaires: the Child Behavior Checklist, the Vineland Adaptive Behavior Scales, and the Reiss Personality Profile. Participants included 10 with BFLS, 10 with Prader-Willi syndrome, and 23 with Klinefelter syndrome variants (13 with 48,XXYY, 4 with 48,XXXY, and 6 with 49,XXXXY). RESULTS: Contrary to initial reports, our men with BFLS had no microcephaly, seizures, or short stature. They manifested deep-set eyes with large ears, coarse facial features, small external genitalia, gynecomastia, and obesity. Family A had mild to moderate mental retardation, whereas family B was more severely affected. On Vineland Adaptive Behavior Scales, men with BFLS had higher daily living and social skills than communicative skills. Men with BFLS also had lower internalizing and externalizing symptoms and appeared more social and helpful than men with Prader-Willi syndrome or Klinefelter syndrome variant. CONCLUSIONS: Men with BFLS from 2 families with mutations in the PHF6 gene manifested distinctive clinical features and a low risk for maladaptive behaviors.