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Harlequin syndrome - one face of many etiologies

Nature Clinical Practice Neurology volume 1, pages 54–59 (2005)Cite this article

Abstract

Background A 55-year-old woman presented to hospital with a 3-month history of asymmetric facial flushing of the skin during exertion, and an 18-month history of left-sided ptosis and miosis. Detailed medical history analysis revealed that a palpable node measuring 0.8 × 1.2 × 1.2 cm (volume 1.1 ml) had been discovered 2 years previously, within the left lobe of an otherwise uncomplicated goiter that had been successfully managed for 20 years. Otherwise, the patient was healthy.

Investigations Neurological examination, autonomic testing, duplex ultrasonography, scintigraphy and MRI.

Diagnosis Harlequin syndrome following a lesion of the preganglionic sympathetic efferents, caused by neurovascular compression of the sympathetic chain between the stellate and superior cervical ganglion brought about by an elongated inferior thyroid artery.

Management Explanation of pathophysiology and benign nature of the condition.

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Figure 1: Patient after exertion.
Figure 2: Duplex ultrasonography showing the spatial relationship between the elongated left anterior thyroid artery, the thyroid gland and the common carotid artery.
Figure 3: Schematic diagrams showing sympathetic fibers innervating the face, and possible lesion sites.
Figure 4: Spatial anatomical relations between the left cervical sympathetic chain and the inferior thyroid artery in a cadaver preparation of the lateral neck.

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Acknowledgements

We thank Dr Peter Pätzel for collecting data and Martina Freyer and Angelika Troyke for technical assistance. We also thank Günther-Rudolf Klaws, Professor Ralf Lucius and Professor Michael Schünke from the Institute of Anatomy at the Christian- Albrechts University, Kiel, for cadaver preparation. This work was supported by the Alexander von Humboldt-Stiftung, the Deutsche Forschungsgemeinschaft (DFG Ba 1921/1–3), the German Ministry of Research and Education within the German Research Network on Neuropathic Pain (BMBF, 01EM01/04) and Pfizer Deutschland (unrestricted educational grant).

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Authors and Affiliations

  1. Department of Neurology and the Division of Neurological Pain Research and Therapy, Christian-Albrechts University, Kiel, Germany

    Gunnar Wasner

  2. Prince of Wales Medical Research Institute, University of New South Wales, Sydney, Australia

    Gunnar Wasner

  3. Division of Neurological Pain Research and Therapy, Assistant Neurologists, Christian-Albrechts University, Kiel, Germany

    Rainer Maag, Janne Ludwig, Andreas Binder & Jörn Schattschneider

  4. Head of the Division of Neurological Pain Research and Therapy, Christian-Albrechts University, Kiel, Germany

    Ralf Baron

  5. Head of the Stroke Unit and Neurocritical Care Unit of the Department of Neurology, Neurovascular Ultrasound Laboratory, Christian-Albrechts University, Kiel, Germany

    Robert Stingele

Authors
  1. Gunnar Wasner
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  2. Rainer Maag
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  3. Janne Ludwig
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  4. Andreas Binder
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  6. Robert Stingele
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  7. Ralf Baron
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Correspondence to Gunnar Wasner.

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The authors declare no competing financial interests.

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Wasner, G., Maag, R., Ludwig, J. et al. Harlequin syndrome - one face of many etiologies. Nat Rev Neurol 1, 54–59 (2005). https://doi.org/10.1038/ncpneuro0040

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