Unusual Behaviors in a 7-year-old Boy

Lawrence Chang; Diane D. Shao; Florian S. Eichler; Siddharth Srivastava

doi:10.1542/pir.2019-0244

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Download PDF

Lawrence Chang, MD*,^†
Diane D. Shao, MD, PhD^‡
Florian S. Eichler, MD^§
Siddharth Srivastava, MD^‡

^*Department of Pediatrics, Boston Children’s Hospital;
^†Department of Pediatrics, Boston Medical Center, Boston, MA
^‡Department of Neurology, Boston Children’s Hospital, Boston, MA; and
^§Department of Neurology, Massachusetts General Hospital, Boston, MA

Address correspondence to Lawrence Chang, MD, Department of Pediatrics, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail: lawrence.chang{at}childrens.harvard.edu

AUTHOR DISCLOSURE
Dr Eichler has sponsorships of clinical trials from bluebird bio and Minoryx Therapeutics and a sponsored research agreement with Applied Genetic Technologies Corporation. Dr Eichler has spoken at an educational session sponsored by bluebird bio, has performed consulting for Ionis Pharmaceuticals, Sanofi Genzyme, and SwanBio Therapeutics, and holds equity in SwanBio Therapeutics. Drs Chang, Shao, and Srivastava have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Presentation

A 7-year-old boy with no significant abnormal medical history presents to the emergency department of a tertiary care children’s hospital with subacute progressive increase in unusual behaviors. For the past several weeks, his parents have noted instances during conversations when he pauses for a few seconds after being spoken to before asking, “What?” Usually after the words are repeated to him, he responds appropriately, although he has also sometimes had strange responses to questions; once when asked, “Can you get that shoe?” he appeared confused then replied, “There are no cows in the house.” The instances occurred only a few times daily at first but have progressively increased in frequency to multiple times per conversation. When they have been pointed out to him, he becomes defensive and blames them on his parents not speaking loudly enough or the room being too loud. Otherwise, he has behaved and interacted normally. His parents have not noticed any unusual movements, and he has not had any other symptoms including fever, vision changes, and gait abnormalities. He was performing well at school but has been on summer break during the course of his symptom onset. Due to concern for a hearing problem, he underwent an audiogram, which was normal, after which he was referred by his pediatrician to the children’s hospital …