In cases where the individual may feel socially embarrassed, contralateral sympathectomy may be considered, although compensatory flushing and sweating of other parts of the body may occur. In contralateral sympathectomy, the nerve bundles that cause the flushing in the face are interrupted. This procedure causes both sides of the face to no longer flush or sweat. Since symptoms of Harlequin syndrome do not typically impair a persons daily life, this treatment is only recommended if a person is very uncomfortable with the flushing and sweating associated with the syndrome. Research
In August 2016, researchers at the Instituto de Assistência dos Servidores do Estado do Rio de Janeiro used botulinum toxin as a method to block the acetylcholine release from the presynaptic neurons. Although they have seen a reduction in one sided flushing, sweating still occurs.There have been case studies of individuals who have experienced this syndrome after an operation. Two female patients with metastatic cancer, ages 37-years-old and 58-years-old, were scheduled for placement of an intrathecal pump drug delivery system. After the intrathecal pump was placed, certain medications were given to the patients. Once the medications were administered, both patients had one sided facial flushes, closely resembling Harlequin Syndrome. Patients were given neurological exams to confirm that their nerves were still intact. An MRI was performed and showed no significant evidence of bleeding or nerve compression. After close observation for 16 hours, symptoms of the Harlequin syndrome was diminished and both patients did not have another episode.
A phase II clinical trial was completed in 2012, which showed that a cocktail of drugs that included lonafarnib and two other drugs met clinical efficacy endpoints that improved the height and diminished the rigidity of the bones of progeria patients. References
External links
"Lonafarnib". Drug Information Portal. U.S. National Library of Medicine. "Experimental Drug Is First To Help Kids With Premature-Aging Disease", NPR, September 24, 2012
Clinical trial number NCT00425607 for "Phase II Trial of Lonafarnib (a Farnesyltransferase Inhibitor) for Progeria" at ClinicalTrials.gov
Clinical trial number NCT00916747 for "Study of Zoledronic Acid, Pravastatin, and Lonafarnib for Patients With Progeria" at ClinicalTrials.gov