The Neuropsychological Profile of Postural Orthostatic Tachycardia Syndrome: Sibling Case Study

Appl Neuropsychol Adult. 2013 Oct-Dec;20(4):306-312. doi: 10.1080/09084282.2012.753075. Epub 2013 Jun 14.

Abstract

Postural orthostatic tachycardia syndrome (POTS), estimated to affect 500,000 people in the United States alone, is a growing source of impairment and disability. Symptoms often consist of physical, cognitive, and psychological impairment. The symptoms are driven by fluctuations in cerebral blood flow. Cerebral perfusion is tightly controlled and linked to brain function as changes in posture can result in quick hemovascular regulation by the autonomic nervous system. Any delay in hemoregulation and cerebral blood flow can result in neurological symptoms. POTS is associated with chronic autonomic dysregulation, and people with POTS suffer differing degrees of cerebral hypoperfusion causing variability in symptoms. This article reviews the cases of two siblings (22 years old and 19 years old) who both suffer from POTS. They suffered physical symptoms along with difficulty organizing thoughts, sustaining attention, thinking quickly, multitasking, and recalling recent information, as well as depression and anxiety. These sisters have Full-Scale IQs (FSIQ) above average, yet they received academic accommodations and have ongoing cognitive dysfunction that prevents them from furthering their true potential. These cases illustrate the need for neuropsychologists and other treating providers to understand POTS, the related pathophysiology, and how cognitively debilitating POTS can be, even in the context of intact and high FSIQ scores.

Keywords: cognition; neuropsychology; postural orthostatic tachycardia syndrome; rehabilitation.