Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He had an unremarkable birth history and no significant medical history. Complete ophthalmological and neurological examinations were performed, and no other abnormalities were identified. Magnetic resonance imaging was performed to exclude lesions of the cerebrum and cervicothoracic spinal cord, and no abnormalities were noted. His final diagnosis was classic idiopathic harlequin syndrome. Herein, we report the first pediatric case of idiopathic harlequin syndrome in Korea.
Cerebrum ; Child ; Diagnosis ; Flushing ; Hot Temperature ; Humans ; Korea ; Magnetic Resonance Imaging ; Male ; Neurologic Examination ; Pediatrics ; Reproductive History ; Spinal Cord ; Sweat ; Sweating

Dystonia is a symptom defined by involuntary and irregular contractions of the muscles, which cause movement disorders and postural problems. Deep brain stimulation (DBS) in globus pallidus interna (GPi) is a good option for controlling dystonia. DBS has already been shown to have significant effects on primary dystonia as well as Parkinson's disease. Dystonia is very difficult to manage, as seen in cerebral palsy (CP) mixed with spasticity. As CP patients grow, their musculoskeletal problems may require orthopedic surgery. However, the outcome of orthopedic surgery is not usually suitable due to dystonia. Therefore, we attempted to control dystonia through DBS initially and perform orthopedic surgery to correct musculoskeletal deformities after treatment of dystonia. Herein, we report a case that showed remarkable improvement in terms of the dystonia rating scale and gait pattern after combined therapy of DBS and orthopedic surgery.
Cerebral Palsy ; Congenital Abnormalities ; Contracts ; Deep Brain Stimulation ; Dystonia ; Dystonic Disorders ; Gait ; Globus Pallidus ; Humans ; Movement Disorders ; Muscle Spasticity ; Muscles ; Orthopedics ; Parkinson Disease

Drug-induced immune hemolytic anemia is a rare disease that occurs in 1 in 1 million individuals of the general population. Rifampin-induced immune hemolytic anemia is caused by drug-dependent antibodies and this can be treated without complication by drug cessation. Herein, we present a case of rifampin-induced immune hemolytic anemia in a patient with primary Sjogren's syndrome (pSS) which occurred during treatment of pulmonary tuberculosis. At admission, the patient's laboratory tests revealed hemolytic anemia and positive direct antiglobulin test result. Since the incidence of autoimmune hemolytic anemia (AIHA) in pSS is reported to be 3 percent, which is higher than that of the general population, differential diagnosis between AIHA and rifampin-induced immune hemolytic anemia was required for planning future anti-tuberculous treatment. We identified rifampin-dependent antibody by drug-induced immune complex test and diagnosed rifampin-induced immune hemolytic anemia. Based on this experience, if rifampin administration is considered in patients with systemic autoimmune disease such as pSS, which has a high incidence of AIHA, we suggest evaluating the presence and the cause of hemolytic anemia at baseline by testing serum lactate dehydrogenase, haptoglobin, and direct and indirect antiglobulin tests before drug administration to promptly identify the cause of hemolysis if hemolytic anemia develops.