Harlequin Syndrome in Cluster Headache

Lehman, Katie; Kumar, Nandini; Vu, Quang; Wells, Rebecca Erwin

doi:10.1111/head.12852

Cited by 5 publications

(6 citation statements)

References 3 publications

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“…One cluster headache patient was described in the literature as experiencing his first bout at the age of 60 years. The harlequin symptoms, however, were not simultaneous with the cluster attacks and both had started after a complicated ipsilateral dental root procedure suggesting an iatrogenic cause 5 . The patient in this case report is the second such case described, and the first one with primary cluster headache and the harlequin sign.…”

Section: Discussionmentioning

confidence: 58%

“…Some patients with harlequin syndrome also report headaches, as was first described by Lance et al 1 Although rare, this co‐morbidity enables us to hypothesize about the underlying pathophysiology 8 . The literature has so far described a total number of 10 patients with both harlequin syndrome and a co‐morbid primary headache disorder 2,3,5,9,10 . Recently, an overview of the clinical characteristics of 9 out of these 10 patients has been presented 4 .…”

Section: Discussionmentioning

confidence: 95%

“…Nowadays, the harlequin syndrome is considered to be a condition within the spectrum of cranial autonomic disorders that also includes Holmer‐Adie's and Horner's syndromes. In the literature, a total of approximately 90 cases of harlequin syndrome have been reported 3‐6 . The majority are primary or idiopathic.…”

Section: Discussionmentioning

confidence: 99%

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Patient with Cluster Headache and Harlequin Sign – Related or Not?

Oosterhout

2020

Headache

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Background The harlequin sign or syndrome is a rare cranial autonomic condition characterized by unilateral diminished flushing and sweating of the face (and sometimes arm), in response to heat or exercise. It results from autonomic, mainly sympathetic dysfunction. Although the idiopathic form is the most common, underlying structural abnormalities in the head, neck, and thorax need to be excluded. Methods and Results Here, we describe the first case of the combination of primary cluster headache and the harlequin syndrome in a 49‐year‐old female patient. The patient presented with a first bout of cluster headache with persisting Horner syndrome and new harlequin sign after the bout. Additional neuroimaging did not reveal underlying pathologies. This report provides an overview of cases from the literature and then discusses the association between primary headaches and the harlequin syndrome. Relevant anatomy, diagnostic tools, and therapeutic options are all considered. Conclusion This case reports a rare combination of cluster headache and harlequin sign that warrants further testing to exclude pathology in the brain, neck or mediastinum. It also illustrates the underlying anatomy and physiology of the autonomic nervous system.

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Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 95%

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Patient with Cluster Headache and Harlequin Sign – Related or Not?

Oosterhout

2020

Headache

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“…In most presented cases as well as in our case, migraine started years before the development of Harlequin syndrome and autonomic symptoms were not simultaneous with headache attacks [ 28 , 30 ]. Two case reports raised the possibility of the association between cluster and Harlequin syndrome [ 28 , 31 ]. Although, a dysfunction of the sympathetic nervous system is a plausible mechanism for both disorders, a shared underlying structural cause cannot be fully excluded in these cases [ 28 ].…”

Section: Discussionmentioning

confidence: 99%

Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Navickaitė,

Ališauskienė,

Petrauskienė

et al. 2023

Medicina

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Background and Objectives: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. Case presentation: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. Conclusions: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.

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“…22 Así mismo, se ha observado mayor preponderancia en pacientes con migraña y cefalea en racimos. 23,24 El diagnóstico es principalmente clínico: con presentación de palidez transitoria y anhidrosis del lado afectado y rubor del lado contralateral. 25 Generalmente afecta la hemicara, aunque puede afectar el brazo y tórax.…”

Section: Discussionunclassified

Síndrome de Arlequín

Alvis-Castaño¹,

Arellano-Lozano²,

Vieyra-Medina³

2022

revecuatneurol

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Se trata de una mujer de 37 años, quien acudió por presentar eritema hemifacial derecho y diaforesis posterior a la realización de ejercicio y eventos estresantes de un año de evolución. Mediante la exploración física y los estudios paraclínicos se descartaron causas estructurales. Por lo tanto, se otorgó el diagnóstico de síndrome de arlequín. Se explicaron las medidas terapéuticas a la paciente y se le dio seguimiento.

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Harlequin Syndrome in Cluster Headache

Cited by 5 publications

References 3 publications

Patient with Cluster Headache and Harlequin Sign – Related or Not?

Patient with Cluster Headache and Harlequin Sign – Related or Not?

Sarcoidosis-Associated Sensory Ganglionopathy and Harlequin Syndrome: A Case Report

Síndrome de Arlequín

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