Harlequin syndrome - one face of many etiologies

Wasner, Gunnar; Maag, R.; Ludwig, J.; Binder, Andreas; Schattschneider, Jörn; Stingele, Robert; Baron, Ralf

doi:10.1038/ncpneuro0040

Cited by 74 publications

(76 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Harlequin syndrome occurs with disruption of vasodilator and sudomotor sympathetic fibers by a lesion on the opposite side. 4 The side with preserved sympathetic innervation exhibits excessive, presumably compensatory, flushing and sweating. 1 Horner syndrome, including ptosis and miosis, frequently coexists with Harlequin syndrome, and is contralateral to the side demonstrating the flushing reactions.…”

Section: Discussionmentioning

confidence: 99%

Systemic sarcoidosis presenting as Harlequin and Horner syndrome

Wong

Doyle

Paulson³

et al. 2015

Neur Clin Pract

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Systemic sarcoidosis presenting as Harlequin and Horner syndrome

Wong

Doyle

Paulson³

et al. 2015

Neur Clin Pract

View full text Add to dashboard Cite

“…The first-order neuron originates in the posterior hypothalamus, the second in the intermediolateral cell column at spinal cord level C8-T2, and the third in the superior cervical ganglion near by the bifurcation of the common carotid artery. 4 Third-order neurons carry 2 different types of sympathetic fibers: oculosympathetic and vaso-/sudomotor fibers. Oculosympathetic fibers ascend along the ICA and innervate the iris dilator muscle and the superior tarsal muscle.…”

Section: Discussionmentioning

confidence: 99%

Internal Carotid Artery Dissection and Asymmetrical Facial Flushing

Drexler

Traenka

Hessling

et al. 2014

Stroke

View full text Add to dashboard Cite

A 47-year-old woman presented to her primary care physician (PCP) with severe left-sided headache localized in the retrobulbar and temporal region. The PCP suspected migraine and prescribed a nonsteroidal anti-inflammatory drug. But despite analgesic treatment, her headache continued, and she returned to her PCP 2 weeks later. At this visit, the PCP noticed new anisocoria with a miotic pupil on the left. Emergent magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of neck and brain revealed no acute or subacute cerebral lesions but a narrowing and semicircular wall hematoma of the left internal carotid artery (ICA) up the petrous segment (Figure 1). The wall hematoma resulted in almost complete occlusion of the arterial lumen. Hereafter, she was transferred to our stroke center, where we confirmed diagnosis of ICA dissection with incomplete Horner syndrome.Extracranial and intracranial duplex sonography revealed a resistance flow profile in the dissected ICA indicating distal occlusion. Collateral circulation through the left posterior communicating artery maintained the blood flow in the left anterior and middle cerebral artery. Six days later a repeated ultrasound showed improving blood flow with only local flow accelerations in the distal extracranial portion of the dissected ICA. The patient was treated with aspirin 300 mg daily for 2 weeks followed by a dose reduction to 100 mg daily. During hospitalization, she remained clinically stable and was discharged home after 8 days.At follow-up visit 3 months later, the patient reported fatigue, difficulty concentrating at work, and persistent left-sided headache. In addition, she had observed an asymmetrical flushing and sweating of her face during a strong hike (Figure 2). The neurological examination was otherwise normal, except for the preexisting left-sided miosis. Blood flow in the left ICA further improved but was still accelerated distally. The patient was told to continue the antiplatelet treatment with aspirin 100 mg daily. DiscussionWe present a patient with a rare, nonischemic symptom of ICA dissection, which has been termed Harlequin sign. 1Dissections of the carotid and vertebral artery are defined by the occurrence of an arterial wall hematoma. MRI with T1-weighted axial scan with fat-saturation technique is the preferred method to detect the mural hematoma in cervical artery dissection, but it can be missed within the first days after onset. 2About two thirds of patients with cervical artery dissection present with stroke or transient ischemic attack and headache or neckpain. Local symptoms (ie, Horner syndrome, cranial-nerve palsy, cervical root injury, and tinnitus) occur in about one third of all cases.3 These local symptoms are caused by an eccentric expansion of the mural hematoma, which leads to compression and stretching of nearby structures (sympathetic-nerve fibers, which proceed along the carotid artery, cranial-nerves, and cervical roots).2 A typical local sign is Horner syndrome, which is defined by the occurrence ...

show abstract

“…Avbrutt sympatisk innervasjon til ansiktet vil ofte gi ipsilateral blekhet og manglende svetteutsondring (hemifacial anhidrose) (8,9), men det man ofte legger merke til er den kontralaterale rødmen og svetten som blir ekstra kraftig for å opprettholde normal varmeregulering i ansiktet (10). Hemifacial anhidrose kalles gjerne Harlekins tegn (11).…”

Section: Pasienten Fortalte At Hun I Noen åR Var Blitt Rød Bare På Veunclassified

“…Vi klarte ikke å fastslå den anatomiske lokaliseringen av sympatikusdefekten. Fravaer av hemifacial anhidrose ved emosjonelt stress kan tyde på affeksjon av første ordens nevron (11), mens manglende Horners syndrom kan tyde på affeksjon av andre ordens nevron (preganglionaert) i 2. og 3. torakale rot eller i nedre del av den sympatiske grensestreng (9) (fig 1).…”

Section: Pasienten Fortalte At Hun I Noen åR Var Blitt Rød Bare På Veunclassified