330 Fig. 1. Photograph of the patient, taken after a 30-min-run, shows facial flushing only in the right side. During this episode, the left side of her face remained dry without sweating. The preganglionic neurons of the sympathetic division originate in the intermediolateral cell column of the spinal gray matter, from the eighth cervical to the second lumbar segments. The preganglionic fibers synapse with the cell bod- ies of postganglionic neurons, which are collected in two large ganglionated chains or cords, one on each side of the verte- bral column (paravertebral ganglia), and in several single pre- vertebral ganglia. There are three cervical (superior, middle, and inferior or stellate), eleven thoracic, and four to six lum- bar sympathetic ganglia. The head receives its sympathetic innervation from the eighth cervical and the first two tho- racic cord segments, the fibers of which passing through the inferior and middle cervical ganglia, and synapsing at the superior cervical ganglia. Postganglionic fibers from the cells of the superior cervical ganglion follow the internal and exter- nal carotid arteries and innervate the blood vessels and smooth muscle as well as the sweat, lacrimal, and salivary glands of the head. The arm receives its postganglionic innervation from the inferior cervical and uppermost thoracic ganglia, which are fused to form the stellate ganglion. Our patient showed crossed sympathetic deficits in the left face and right arm, which suggested that the lesions were multifocal. Pre- viously, crossed sympathetic deficits involving the face, arm, and leg has been described only once, in a 37-yr-old woman (6). Caparros-Lefebvre et al. (7) reported another patient with unilateral loss of facial flushing and sweating, and with con- tralateral anhidrosis, which were induced by exercise. How- ever, she also had tonic pupils and areflexia, and was consid- S.Y. Moon, D.-I. Shin, S.-H. Park, et al. ered to have Ross syndrome (Adie syndrome with anhidrosis). The spectrum of pre- and postganglionic autonomic dys- function in patients with harlequin syndrome indicates a rela- tion with Holmes-Adie and Ross syndromes, and the per- sisting autonomic deficit in Guillain-Barre′syndrome (8-10). The clinical course of partial dysautonomias, such as Ross syndrome or Holmes-Adie syndrome, seems to be benign but may progress slowly. Each of these entities has a more widespread dysfunction than previously believed, with con- siderable overlap. There is increasing evidence that classic partial autonomic disorders may lie on a spectrum of gener- alized injury to the autonomic and dorsal root ganglia (11, 12). Because the sympathetic, parasympathetic, and dorsal root ganglia are all neural crest cell derivatives, it is conceiv- able that some property of these related tissues makes them more susceptible to injury, although the mechanism of gan- glionic damage or degeneration remains unknown. REFERENCES 1. Lance JW, Drummond PD, Gandevia SC, Morris JG. Harlequin syndrome: the sudden onset of unilateral flushing and sweating. J Neurol Neurosurg Psychiatry 1988; 51: 635-42. 2. Noda S. Harlequin syndrome due to superior mediastinal neurino- ma. J Neurol Neurosurg Psychiatry 1991; 54: 744. 3. Coleman PJ, Goddard JM. Harlequin syndrome following internal jugular vein catheterization in an adult under general anesthetic. Anesthesiology 2002; 97: 1041. 4. Charkoudian N. Skin blood flow in adult human thermoregulation: how it works, when it does not, and why. Mayo Clin Proc 2003; 78: 603-12. 5. Drummond PD. Sweating and vascular responses in the face: nor- mal regulation and dysfunction in migraine, cluster headache and harlequin syndrome. Clin Auton Res 1994; 4: 273-85. 6. Corbett M, Abernethy DA. Harlequin syndrome. J Neurol Neuro- surg Psychiatry 1999; 66: 544. 7. Caparros-Lefebvre D, Hache JC, Hurtevent JF, Dereeper O, Bille F, Petit H. Unilateral loss of facial flushing and sweating with contralat- eral anhidrosis: harlequin syndrome or Adie’s syndrome? Clin Auton Res 1993; 3: 239-41. 8. Kim JS, Yun CH, Moon CS. Bilateral tonic (Adie’s) pupils in Vogt- Koyanagi-Harada syndrome. J Neuroophthalmol 2001; 21: 205-6. 9. Choi MJ, Choi DG, Kim SY. A case of Ross’ syndrome with Horner’s syndrome. J Korean Ophthalmol Soc 1998; 39: 2201-5. 10. Kim KK. Tonic pupil in neuropathy. J Korean Neurol Assoc 2000; 18: 420-4. 11. Drummond PD, Edis RH. Loss of facial sweating and flushing in Holmes Adie syndrome. Neurology 1990; 40: 847-9. 12. Shin RK, Galetta SL, Ting TY, Armstrong K, Bird SJ. Ross syndrome plus: Beyond Horner, Holmes-Adie, and harlequin. Neurology 2000; 55: 1841-6.