Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma.
Authors: Jeon YJ, Son J, Cho JH Abstract Harlequin syndrome is a rare disorder of the sympathetic nervous system characterized by unilateral facial flushing and sweating. Although its etiology is unknown, this syndrome appears to be a dysfunction of the autonomic nervous system. To the best of our knowledge, thus far, very few reports on perioperative Harlequin syndrome after thoracic surgery have been published in the thoracic surgical literature. Here, we present the case of a 6-year-old patient who developed this unusual syndrome following the resection of a posterior mediastinal mass. PMID: 283...
Source: Korean Journal of Thoracic and Cardiovascular Surgery - April 7, 2017 Category: Cardiovascular & Thoracic Surgery Tags: Korean J Thorac Cardiovasc Surg Source Type: research

Harlequin sign concomitant with Horner syndrome after anterior cervical discectomy: a case of intrusion into the cervical sympathetic system.
Abstract Harlequin syndrome is a rare autonomic disorder referring to the sudden development of flushing and sweating limited to one side of the face. Like Horner syndrome, associating miosis, ptosis, and anhidrosis, Harlequin syndrome is caused by disruption of the cervical sympathetic pathways. Authors of this report describe the case of a 55-year-old female who presented with both Harlequin sign and Horner syndrome immediately after anterior cervical discectomy (C6-7) with cage fusion and anterior spondylodesis. They discuss the pathophysiology underlying this striking phenomenon and the benign course o...
Source: Journal of Neurosurgery.Spine - March 9, 2017 Category: Neurosurgery Authors: Fringeli Y, Humm AM, Ansorge A, Maestretti G Tags: J Neurosurg Spine Source Type: research

A pediatric case of idiopathic Harlequin syndrome.
Authors: Kim JY, Lee MS, Kim SY, Kim HJ, Lee SJ, You CW, Kim JS, Kang JH Abstract Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He h...
Source: Korean Journal of Pediatrics - December 28, 2016 Category: Pediatrics Tags: Korean J Pediatr Source Type: research

Harlequin Syndrome after Stellate Ganglia Neuroblastoma Resection
A localized MYCN nonamplified neuroblastoma arising from the left stellate ganglia was discovered in a 9-month-old girl. After 4 courses of neoadjuvant chemotherapy, the tumor was removed via left thoracotomy. Residue from the upper part of the initial mass located behind the left subclavian and vertebral arteries was followed serially. There was an increase in size up to 37 × 20 × 23 mm; foraminate extension in T1-T2 22 months later prompted an iterative surgical resection through a left transmanubrial osteomuscular-sparing approach (Figure 1). (Source: The Journal of Pediatrics)
Source: The Journal of Pediatrics - June 21, 2016 Category: Pediatrics Authors: Sabine Irtan, Anne Auvrignon Tags: Rediscovering the Physical Exam Source Type: research

Harlequin Syndrome after Stellate Ganglia Neuroblastoma Resection
A localized MYCN nonamplified neuroblastoma arising from the left stellate ganglia was discovered in a 9-month-old girl. After 4 courses of neoadjuvant chemotherapy, the tumor was removed via left thoracotomy. Residue from the upper part of the initial mass located behind the left subclavian and vertebral arteries was followed serially. There was an increase in size up to 37  × 20 × 23 mm; foraminate extension in T1-T2 22 months later prompted an iterative surgical resection through a left transmanubrial osteomuscular-sparing approach (Figure 1). (Source: The Journal of Pediatrics)
Source: The Journal of Pediatrics - June 21, 2016 Category: Pediatrics Authors: Sabine Irtan, Anne Auvrignon Tags: Rediscovering the Physical Exam Source Type: research

Harlequin Syndrome in Cluster Headache.
PMID: 27238298 [PubMed - as supplied by publisher] (Source: Headache)
Source: Headache - May 29, 2016 Category: Neurology Authors: Lehman K, Kumar N, Vu Q, Wells R Tags: Headache Source Type: research

Harlequin Syndrome in Cluster Headache
(Source: Headache: The Journal of Head and Face Pain)
Source: Headache: The Journal of Head and Face Pain - April 30, 2016 Category: Neurology Authors: Katie Lehman, Nandini Kumar, Quang Vu, Rebecca Wells Tags: Resident and Fellows Source Type: research

Sympathetic Dysfunction Inter-Ictally in Cluster Headache (P6.097)
Conclusion: The strictly LEFT sided facial flushing that occurred with exertion in this patient demonstrates the patient’s loss of sweating on the RIGHT, non-flushed side of the face, suggestive of abnormal sympathetic activation on the RIGHT and consistent with Harlequin syndrome. Although cluster patients typically exhibit unilateral ipsilateral autonomic dysfunction during a cluster attack, ipsilateral sympathetic dysfunction may also occur inter-ictally. While most cases of Harlequin syndrome are benign, care must be taken to rule out a lesion along the sympathetic tract, especially along the postganglionic cervi...
Source: Neurology - April 3, 2016 Category: Neurology Authors: Lehman, K., Kumar, N., Vu, Q., Wells, R. Tags: Headache: Basic Science and Clinical Presentation/Diagnosis Source Type: research

[Harlequin phenomenon associated with neurological abnormalities: A case report].
We present the case of a child in whom the syndrome was associated with Horner's syndrome, epilepsy, mental and psychomotor retardation. PATIENTS AND METHODS: A 9-year-old boy presented with right unilateral hemifacial erythema on effort, with normal colouring and Horner's syndrome on the left side of the face. His medical history revealed generalized myoclonic epilepsy, psychomotor delay and mental retardation. No underlying anomalies were identified. Harlequin phenomenon was diagnosed. DISCUSSION: Despite its stereotypical clinical features, Harlequin phenomenon is a poorly known disease. However, clin...
Source: Annales de Dermatologie et de Cenereologie - March 23, 2016 Category: Dermatology Authors: Lucas P, Legendre L, Pauwels C, Mazereeuw-Hautier J Tags: Ann Dermatol Venereol Source Type: research

Harlequin Syndrome After Thoracic Paravertebral Block
We present a case of Harlequin syndrome without Horner syndrome in a patient with unilateral right-sided facial flushing that started shortly after a left-sided thoracic paravertebral nerve block for a mastectomy. We discuss the interruption of the sympathetic and parasympathetic nervous system and the levels of spinal nerve block associated with a thoracic paravertebral nerve block. (Source: A&A Case Reports)
Source: A&A Case Reports - February 1, 2016 Category: Anesthesiology Tags: Case Reports: Clinical Care Source Type: research

Botulinum toxin for treatment of Harlequin syndrome
We described a patient with Harlequin syndrome, a rare neurological condition, characterized by unilateral facial sweating and flushing, who had a good response to botulinum toxin application. She had been submitted to sympathectomy a few years, however she still complained of excessive sweating in the regions mentioned and intense flushing. (Source: Parkinsonism and Related Disorders)
Source: Parkinsonism and Related Disorders - December 17, 2015 Category: Neurology Authors: Roberta K.J.V. Manhães, Mariana Spitz, Luiz Felipe Vasconcellos Tags: Correspondence Source Type: research

Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy
The author presents a patient with Harlequin's and Horner's syndrome as part of an autoimmune autonomic ganglionopathy and suggests implication for work-up and management. In general, Harlequin's and Horner's syndromes are reported to be caused by either a structural lesion of the sympathetic pathway or, when no structural lesion is found, are presumed to be idiopathic. In this paper, a 76year old man developed a Harlequin's and Horner's syndrome in the setting of subacute autonomic failure and other systemic features. (Source: Autonomic Neuroscience: Basic and Clinical)
Source: Autonomic Neuroscience: Basic and Clinical - December 9, 2015 Category: Neuroscience Authors: Chafic Karam Source Type: research

Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy.
Abstract The author presents a patient with Harlequin and Horner syndromes as part of an autoimmune autonomic ganglionopathy and suggests implication for work-up and management. In general, Harlequin and Horner syndromes are reported to be caused by either a structural lesion of the sympathetic pathway or, when no structural lesion is found, are presumed to be idiopathic. In this paper, a 76year old man developed a Harlequin and Horner syndromes in the setting of subacute autonomic failure and other systemic features. The patient's symptoms improved with a short course of intravenous methylprednisolone. An...
Source: Autonomic Neuroscience - December 9, 2015 Category: Neuroscience Authors: Karam C Tags: Auton Neurosci Source Type: research

Systemic sarcoidosis presenting as Harlequin and Horner syndrome
We report a rare case of Harlequin and Horner syndromes attributed to systemic sarcoidosis. (Source: Nature Clinical Practice)
Source: Nature Clinical Practice - December 7, 2015 Category: Neurology Authors: Wong, J. C., Sparks, J. A., Doyle, T. J., Paulson, V. A., Milligan, T. A., Prasad, S. Tags: All Clinical Neurology, All Medical/Systemic disease, All Neuro-ophthalmology Cases Source Type: research

Physical and Psychological Consequences of Left Cardiac Sympathetic Denervation in Long-QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia [Original Articles]
Conclusions— Despite significant morbidity resulting from left cardiac sympathetic denervation, patients with LQTS and CPVT have high levels of postoperative satisfaction. (Source: Circulation: Arrhythmia and Electrophysiology)
Source: Circulation: Arrhythmia and Electrophysiology - October 20, 2015 Category: Cardiology Authors: Waddell-Smith, K. E., Ertresvaag, K. N., Li, J., Chaudhuri, K., Crawford, J. R., Hamill, J. K., Haydock, D., Skinner, J. R., on behalf of the Cardiac Inherited Disease Group New Zealand* Tags: Other diagnostic testing Original Articles Source Type: research