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Harlequin syndrome in a patient with probable hemicrania continua and exertional headache - is there a link? a case report.
Miedl, Markus; Baumgartner, Philipp; Disse, Leah Raffaela; Weber, Konrad Peter; Pohl, Heiko; Wegener, Susanne.
Afiliação
  • Miedl M; Department of Neurology, University Hospital Zurich, Zurich, Switzerland.
  • Baumgartner P; Department of Neurology, University Hospital Zurich, Zurich, Switzerland.
  • Disse LR; Department of Neurology, University Hospital Zurich, Zurich, Switzerland.
  • Weber KP; Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland.
  • Pohl H; Department of Neurology, University Hospital Zurich, Zurich, Switzerland.
  • Wegener S; Department of Ophthalmology, University Hospital Zurich, Zurich, Switzerland.
BMC Neurol ; 24(1): 247, 2024 Jul 17.
Article em En | MEDLINE | ID: mdl-39020271
ABSTRACT

BACKGROUND:

The harlequin syndrome is a rare disorder of the autonomic nervous system characterized by unilateral diminished flushing and sweating of the face following exposure to heat or physical activity. It results from sympathetic dysfunction and most commonly occurs idiopathically. A secondary development due to an underlying pathology (e.g., carotid artery dissection, tumors) must be excluded at first appearance. There is evidence that the cranial autonomic system is involved in the pathophysiology of trigeminal autonomic headaches like hemicrania continua. Therefore, an overlap in the pathophysiology of harlequin syndrome and trigeminal autonomic headache disorders seems plausible. However, the association of a harlequin syndrome with hemicrania continua was never reported. CASE PRESENTATION This work describes the case of a 42-year-old female patient presenting to our headache unit. The patient reported persisting unilateral headache of the right side of dragging or squeezing character accompanied by trigeminal autonomic symptoms, including lacrimation, nasal congestion, conjunctival injection and Horner's syndrome, and was responsive to treatment with 75mg/d indomethacin. Five months after the initial consultation, the patient noted that the upper right quadrant of her face was pale after jogging. A harlequin syndrome was diagnosed. Further, she developed a short-lasting, bilateral headache of pulsatile character during strenuous exercise consistent with exertional headache. Comprehensive diagnostic evaluations, encompassing cranial and cervical MRI scans, laboratory tests, and biopsies, culminated in the diagnosis of Sjögren's syndrome. This finding suggests that the trigemino-autonomic dysfunction may either be idiopathic or a direct manifestation of Sjögren's syndrome.

CONCLUSIONS:

This report documents the case of a rare combination of a headache resembling probable hemicrania continua and the harlequin syndrome (and even exertional headache). It illustrates the underlying anatomy of the autonomic nervous system in a clinical context and emphasizes the hypothesis of a pathophysiological link between abnormal sympathetic activity and trigeminal autonomic headaches.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doenças do Sistema Nervoso Autônomo / Rubor / Hipo-Hidrose Limite: Adult / Female / Humans Idioma: En Ano de publicação: 2024 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doenças do Sistema Nervoso Autônomo / Rubor / Hipo-Hidrose Limite: Adult / Female / Humans Idioma: En Ano de publicação: 2024 Tipo de documento: Article