Abstract
A 45-Year-Old woman presented with a 10 year history of asymmetrical facial flushing and sweating after exertion or in hot weather. During these episodes the right side of her face remained dry and white, while the left side normally flushed. Sweating was impaired on the left side in the limbs and trunk. She also had areflexia in the lower limbs and slow pupillary reactions to light and darkness, as seen in Adie's syndrome. The topography of the sweating disorder suggested that the lesion involved the sympathetic pathways at the level of spinal cord. The relationship with the harlequin syndrome and related disorders is discussed.
Similar content being viewed by others
References
Lance JW, Drummond PD, Gandevia SC, Morris JGL. Harlequin syndrome: the sudden onset of unilateral flushing and sweating.J Neurol, Neurosurg and Psychiatr 1988;51: 635–642.
Drummond PD, Edis RH. Loss of facial sweating and flushing in Holmes-Adie syndrome.Neurology 1990;41: 847–849.
Charlier J, Hache JC. Pupillary reflexia and presbyopia. In: Stark L, Obrecht G, eds.Presbyopia. Recent research and reviews from the IIIrd international symposium (1985). Fairchild, NY: Professional Press, 1987.
McLeod JG, Tuck RR. Disorders of the autonomic nervous system: Part 2. Investigation and treatment.Ann Neurol 1987;21: 519–529.
Baba M, Watahiki Y, Matsunaga M, Takebe K. Sympathetic skin response in healthy man.Electromyogr Clin Neurophysiol 1988;28: 277–283.
Brody IA, Wilkins RH. Adie's syndrome.Arch Neurol 1968;18: 710–715.
Adie WJ. Tonic pupils and absent tendon reflexes, a benign disorder sui generis: its complete and incomplete forms.Brain 1932;55: 98–113.
Russell GFM. Accommodation in Holmes-Adie syndrome.J Neurol Neurosurg Psychiat 1958;21: 290–296.
Harriman DGF, Garland H. The pathology of Adie's syndrome.Brain 1968;91: 401–418.
Bartin RH, Schmutz JL, Cuny JF, Vespignani H, Weber M, Beurey A. Le syndrome de Ross. Apropos d'une observation.Ann Dermatol Venereol 1990;117: 113–114.
Spector RH, Rachman DL. Bilateral Adie's tonic pupil with anhidrosis and hyperthermia.Arch Neurol 1984;41: 342–343.
Vanasse M, Molina-Negro P, Saint-Hilaire JM. Syndrome d'Adie associé à une hypohidrose segmentaire. Etude clinique et neurophysiologique.Rev Neurol 1974;130: 237–244.
Petajan JH, Danforth RC, D'Allesio D, Lucas GJ. Progressive sudomotor denervation and Adie's syndrome.Neurology 1965;15: 172–176.
Lucy DD, Van Allen MW, Thompson HS. Holmes-Adie syndrome with segmental hypohidrosis.Neurology 1967;17: 763–769.
Johnson RH, Robinson BJ. Local autonomic failure affecting a limb.J Neurol Neurosurg Psychiat 1987;50: 738–742.
Nathan PW, Smith MC. The location of descending fibres to sympathetic preganglionic vasomotor and sudomotor neurons in man.J Neurol Neurosurg Psychiat 1987;50: 1253–1262.
Laruelle L. La structure de la moelle épinière en coupes longitudinales.Rev Neurol (Paris) 1937;67: 695–725.
Hardin WB, Gay AJ. The phenomenon of benign areflexia.Neurology 1965;15: 613–621.
Miyasaki JM, Ashby P, Sharpe JA, Fletcher WA. On the cause of hyporeflexia in the Holmes-Adie syndrome.Neurology 1988;38: 262–265.
Ulrich J. Morphological basis of Adie's syndrome.Eur Neurol 1980;19: 390–395.
Selhorst JB, Madge G, Ghatak NR. The neuropathology of the Holmes-Adie syndrome.Ann Neurol 1984;16: 138.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Caparros-Lefebvre, D., Hache, J.C., Hurtevent, J.F. et al. Unilateral loss of facial flushing and sweating with contralateral anhidrosis: Harlequin syndrome or Adie's syndrome?. Clinical Autonomic Research 3, 239–241 (1993). https://doi.org/10.1007/BF01829012
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01829012