Harlequin syndrome: a rare sequelae of carotid dissection.

BackgroundThe 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 presentationThis 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.ConclusionsThis 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.

# {#article-title-2} To the Editor: We read the Teaching Neuro Image regarding Harlequin syndrome caused by internal carotid artery dissection (ICAD) with interest.1 We believe that this syndrome may not have been caused by ICAD. Harlequin syndrome due to ICAD generally affects the forehead and not the whole face because the innervation area of sympathetic vasomotor and sudomotor fibers traveling with the ICA is restricted to …

A 41-year-old woman presented to our hospital with a 12-year history of recurrent episodes of excessive sweating on her right face, neck, shoulder, and left calf after exercise (Figure). Cranial nerve examination was normal. Routine tests, thyroid function, ultrasound of carotid artery, MRI of the head, mediastinum, cervical and lumbar spine, and whole-body 18F-FDG PET/MRI were all negative. At her 3-year follow-up, her entire left leg was affected. She was finally diagnosed with Harlequin syndrome. Harlequin syndrome is attributed to sympathetic dysfunction.1 Contralateral involvement of autonomic nerves is relatively rare. Causes include syringomyelia, carotid artery dissection, tumors, or brainstem infarction, but many cases are idiopathic.1,2 Usually, Harlequin syndrome requires no treatment, but botulinum toxin can be tried in patients with severe symptoms.1

Hemifacial sweating after carotid artery dissection

Harlequin syndrome in spontaneous dissection of the cervical carotid artery A 52-year-old previously healthy man had a spontaneous dissection of the left cervical carotid artery (figure 1) with Horner syndrome.Three months later, when the patient restarted his running training, he noticed the symptoms of Harlequin syndrome (figure 2), which consist of unilateral facial flushing and sweating. 1The Harlequin syndrome affected only the left part of the patient's face and nose, because vasomotor and sudomotor sympathetic fibers traveling along the internal carotid artery innervate just the forehead and nose.This patient shows that physicians should also consider spontaneous carotid dissection as possible etiology of Harlequin syndrome.

Harlequin Syndrome with Ipsilateral Facial Flushing After Internal Carotid Artery Dissection: An Atypical Sympathetic Laterality Pattern.

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A comprehensive examination and dynamic observation of a patient with clinical manifestations of connective tissue dysplasia and dissection of the internal carotid arteries revealed a symptom complex characteristic of Harlequin syndrome. The description of this form of pathology is given for the first time in the national literature. A brief review presents the diagnostic criteria and mechanisms for the development of this rare autonomic dysfunction.