Although this space is usually devoted to observations about patients and practice, this month’s column will describe an entirely new syndrome. Studying this condition may help broaden the horizons of neuroscience and deepen our understanding of the complex ways of the human brain.
Neurology recognizes many cognitive deficits, from the global (dysphasia – difficulty communicating) to the more limited (dysnomia – the inability to remember names and dyscalculia – problems counting.) I wish to describe a hitherto-unreported focal cognitive deficit, the selective inability to follow the procedures of the federal online registration program for isotretinoin, known as iPledge. I propose calling this condition DysiPledgeia.
Those who suffer from this disorder are for the most part young and generally healthy, alert and oriented, and unhampered by any linguistic or cultural barriers. When the patient is an adolescent living at home, the syndrome may be shared with parents (DysiPledgeia à deux, à trois, or, if grandparents are involved, DysiPledgeia avec toute la famille).
Of epidemiologic interest are two other observations: When iPledge requires pregnancy test reporting, most cases of DysiPledgeia are women. Another intriguing point is that nearly all cases also have severe acne.
Under the iPledge program, a registered female patient of childbearing age is required to:
• Have a negative pregnancy test, which her doctor enters into the system.
• Answer questions to demonstrate comprehension.
• Pick up isotretinoin within 7 days, bringing her 10-digit iPledge number to the pharmacy.
Some people have trouble at first following all three steps. There is debate over whether these patients have no disease, or whether they have a transient, abortive form of DysiPledgeia.
In the full-blown syndrome, patients who are otherwise cognitively intact cannot master the routine, and do not respond to educational efforts. They account for the preponderance of iPledge-related calls, which may be frightened ("I’m running out of pills!"), confused ("I don’t understand; the pharmacy won’t give them to me!"), or belligerent ("Your office didn’t do what it was supposed to!"). These calls are repeated at least monthly, and at times more often.
Explanatory responses from the medical staff are usually met with perplexity, remorse, blank incomprehension, or a combination of all three.
• Perplexity. "I waited 10 days to fill the prescription. Wasn’t somebody supposed to call me to go fill it?"
• Remorse. "I know I was supposed to get that pregnancy test!"
• Incomprehension. "Questions? What questions?"
• All three. "I gave them my number! Wait, you did say to use the one I got from the dermatologist in Texas 3 years ago, when he registered me but my insurance wouldn’t cover the medicine. What was that number again?"
Sometimes the calls can be alarming. "Wait! Which two contraceptive methods was I supposed to put down?"
The etiology and pathogenesis of DysiPledgeia are under investigation. Studies are being planned and will use imaging and neuropsychiatric testing to uncover anatomical and neurophysiologic deficits.
The intriguing correlation between DysiPledgeia and severe acne has not yet been clarified. A working hypothesis is that DysiPledgeia may be linked to a deficiency in the WBZ-1030 interleukin pathway, mediated through dystopic upregulation of mRNA caused by somatic mutations on the short arm of chromosome 14. It seems likely that deeper study of the acne-DysiPledgeia connection will help science better grasp the links between the nervous and integumentary systems. This in turn may provide insight into why dermatologists are so often handsome, charming, and highly intelligent.
Treatment for DysiPledgeia is not currently available, though fortunately this condition tends to be self-limited, rarely lasting more than 6 months. Treatment for the physician who takes care of many patients with DysiPledgeia is largely supportive: mild sedatives, long vacations, and contact sports have all been tried, with varying success.
Advances in understanding and treating this puzzling disorder, if any, will be covered in future columns. In the meantime, applications are in place to funding sources, as well as to the relevant committees overseeing ICD-9, 10, and 11. Readers with access to used CT-scanners or unemployed neuroscientists are urged to contact the editor.