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Unilateral loss of facial flushing and sweating with contralateral anhidrosis: Harlequin syndrome or Adie's syndrome?

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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.

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References

  1. 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.

    Google Scholar 

  2. Drummond PD, Edis RH. Loss of facial sweating and flushing in Holmes-Adie syndrome.Neurology 1990;41: 847–849.

    Google Scholar 

  3. 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.

    Google Scholar 

  4. McLeod JG, Tuck RR. Disorders of the autonomic nervous system: Part 2. Investigation and treatment.Ann Neurol 1987;21: 519–529.

    PubMed  Google Scholar 

  5. Baba M, Watahiki Y, Matsunaga M, Takebe K. Sympathetic skin response in healthy man.Electromyogr Clin Neurophysiol 1988;28: 277–283.

    PubMed  Google Scholar 

  6. Brody IA, Wilkins RH. Adie's syndrome.Arch Neurol 1968;18: 710–715.

    PubMed  Google Scholar 

  7. Adie WJ. Tonic pupils and absent tendon reflexes, a benign disorder sui generis: its complete and incomplete forms.Brain 1932;55: 98–113.

    Google Scholar 

  8. Russell GFM. Accommodation in Holmes-Adie syndrome.J Neurol Neurosurg Psychiat 1958;21: 290–296.

    Google Scholar 

  9. Harriman DGF, Garland H. The pathology of Adie's syndrome.Brain 1968;91: 401–418.

    PubMed  Google Scholar 

  10. 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.

    PubMed  Google Scholar 

  11. Spector RH, Rachman DL. Bilateral Adie's tonic pupil with anhidrosis and hyperthermia.Arch Neurol 1984;41: 342–343.

    PubMed  Google Scholar 

  12. 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.

    PubMed  Google Scholar 

  13. Petajan JH, Danforth RC, D'Allesio D, Lucas GJ. Progressive sudomotor denervation and Adie's syndrome.Neurology 1965;15: 172–176.

    PubMed  Google Scholar 

  14. Lucy DD, Van Allen MW, Thompson HS. Holmes-Adie syndrome with segmental hypohidrosis.Neurology 1967;17: 763–769.

    PubMed  Google Scholar 

  15. Johnson RH, Robinson BJ. Local autonomic failure affecting a limb.J Neurol Neurosurg Psychiat 1987;50: 738–742.

    PubMed  Google Scholar 

  16. 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.

    PubMed  Google Scholar 

  17. Laruelle L. La structure de la moelle épinière en coupes longitudinales.Rev Neurol (Paris) 1937;67: 695–725.

    Google Scholar 

  18. Hardin WB, Gay AJ. The phenomenon of benign areflexia.Neurology 1965;15: 613–621.

    PubMed  Google Scholar 

  19. Miyasaki JM, Ashby P, Sharpe JA, Fletcher WA. On the cause of hyporeflexia in the Holmes-Adie syndrome.Neurology 1988;38: 262–265.

    PubMed  Google Scholar 

  20. Ulrich J. Morphological basis of Adie's syndrome.Eur Neurol 1980;19: 390–395.

    PubMed  Google Scholar 

  21. Selhorst JB, Madge G, Ghatak NR. The neuropathology of the Holmes-Adie syndrome.Ann Neurol 1984;16: 138.

    Google Scholar 

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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

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  • DOI: https://doi.org/10.1007/BF01829012

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