Kanski JJ. In the original Raeder syndrome, the pain is trigeminal pain associated with hypoesthesia or anesthesia in the distribution of the trigeminal nerve (cranial nerve [CN] V). [1], Horner syndrome has a frequency of 1 per 6,250 population. 2009 Sep. 61(3):421-5. Please contact GARD if you need help finding additional information or resources on rare diseases, including clinical studies. Classic clinical signs include partial ptosis (drooping upper eyelid) and an abnormal pupil in the affected eye. The denervation supersensitivity results in pupillary dilatation and lid elevation on the abnormal side but no response or mild miosis on the normal side from alpha2-activity after apraclonidine administration. Kucur C, Ozbay I, Oghan F, Yildirim N, Zeybek Sivas Z, Canbaz Kabay S. A Rare Complication of Radiofrequency Tonsil Ablation: Horner Syndrome. Horner syndrome is typically caused by damage to the sympathetic nerves that serve your eyes. Hydroxyamphetamine stimulates the release of stored endogenous norepinephrine from the postganglionic axon terminals into the neuromuscular junction at the iris dilator muscles. Suite 310 Central nervous system and peripheral nervous system, Linking to and Using Content from MedlinePlus, U.S. Department of Health and Human Services, Injury to the carotid artery, one of the main arteries to the brain, Injury to nerves at the base of the neck called the brachial plexus, Tumor or infection in the top of the lung, between the lungs, and neck, Injections or surgery done to interrupt the nerve fibers and relieve pain (sympathectomy), Decreased sweating on the affected side of the face, Overreaction of the involuntary (autonomic) nervous system to stimulation (, Electromyography and nerve conduction studies if nerve root or brachial plexus injury suspected. Isolated Horner's syndrome and syringomyelia | Journal of Neurology National Center for Advancing Translational Sciences, Bernard-Horner Syndrome; Oculosympathetic Palsy. Von Passow syndrome is an association of Horner syndrome with iris heterochromia (heterochromia iridis). [16, 17, 18, 19, 20] ; for example, ptosis may rarely complicate injection of botulinum toxin for glabellar lines. To use the sharing features on this page, please enable JavaScript. There is no treatment for Horner syndrome itself. Talk to a trusted doctor before choosing to participate in any clinical study. NORD strives to open new assistance programs as funding allows. Horner syndrome - Symptoms and causes - Mayo Clinic Gregory Van Stavern, MD Attending Physician, Department of Ophthalmology and Neurology, Washington University School of Medicine A.D.A.M. Infants may have a lighter color iris in the affected eye. 2001 May. URL of this page: //medlineplus.gov/ency/article/000708.htm. [13] or coronary artery bypass grafting), Lymphadenopathy (eg, Hodgkin disease, leukemia, tuberculosis, or mediastinal tumors), Lesions of the middle ear (eg, acute otitis media). Our website services, content, and products are for informational purposes only. Lee JH, Lee HK, Lee DH et-al. [8], Horner syndrome can be congenital, acquired, or purely hereditary (autosomal dominant). The ophthalmic manifestations arise due to paralysis of the sympathetically innervated superior tarsal muscle (also known as Muller's muscle, which dilates the pupil) and thus . Horner's syndrome is characterised by the triad of ptosis (drooping eyelid), anhidrosis (lack of sweating) and miosis (constricted pupil) on the ipsilateral side (Figure 1). The empty hole in the middle, which allows light to enter the eye, is called the pupil. 2000 Jul. Carotid artery dissection. Other findings that may be noted include the following: Horner syndrome in the presence of pain merits special consideration. The fibers ascend through the sympathetic chain and synapse in the superior cervical ganglion at the level of the bifurcation of the common carotid artery (C3-C4). Vision Center is funded by our readers. If the affected eye doesnt dilate as much as the healthy eye, it confirms Horners syndrome. The prognosis and the complications to be expected depend on the underlying cause of the syndrome, as does treatment. Some cases of Horner syndrome occur for no other apparent reason or unknown cause (idiopathically). AJR Am J Roentgenol. Find resources for patients and caregivers that address the challenges of living with a rare disease. Careful direction of the history to rule out such life-threatening disorders is vital. Horner's Syndrome: Causes, Symptoms & Treatments, Incidence of pediatric Horner syndrome and the risk of neuroblastoma: a population-based study, Bilateral Horner's syndrome: detection and occurrence. 2008 Jan. 39(1):82-6. What are the common ocular abnormalities of Horner syndrome? Horner syndrome | definition of Horner syndrome by Medical dictionary The symptoms reported by the patient will depend on the underlying cause, as follows 2003;23:22-23. Oxford: 1924. May 27, 2008 Horner syndrome is a classic neurologic syndrome whose signs include miosis, ptosis, and anhidrosis. Its a fairly rare condition. Meanwhile, the healthy eye will not respond or experience mild miosis. For additional information visit Linking to and Using Content from MedlinePlus. This is a rare neurological disorder that also affects the eye. Herein, we review the clinical signs and symptoms that can aid in the diagnosis and localization of a Horner syndrome . Handbook of Ocular Disease Management. www.medhelp.org/forums/neuro/archive/XXXX.html. Gregory Van Stavern, MD is a member of the following medical societies: American Academy of Neurology, North American Neuro-Ophthalmology SocietyDisclosure: Nothing to disclose. With central first-order neuron lesions, anhidrosis affects the ipsilateral side of the body. PDF Horner's Syndrome - Manchester University NHS Foundation Trust J AAPOS. This site complies with the HONcode standard for trustworthy health information: verify here. Given that malignancy is a prominent feature of recently diagnosed preganglionic Horner syndrome, patients may avoid an unnecessary extensive workup for carcinoma if the lesion can be shown to be old or long-standing. Entry Number; 143000: Last Edit Date; 9/24/94. Postganglionic involvement has primarily benign causes (ie, usually a vascular headache). The sympathetic nervous system (SNS) triggers involuntary actions in response to dangerous or stressful situations. The most common aetiological . The term Horner syndrome is commonly used in English-speaking countries, whereas the term Bernard-Horner syndrome is common in France. This system allows your body to respond correctly to any changes in the environment around you. However, it may be a sign of oculosympathetic nerve damage. Quincy, MA 02169 Genetic counseling may be of benefit for patients and their families if they have the genetic form of this disorder. Note, GARD cannot enroll individuals in clinical studies. Third-order neuron lesions that may give rise to Horner syndrome include the following 2008 Feb. 44(2):116-7. A case of Ross syndrome presented with Horner and chronic cough According to a 2022 article, the condition can affect people of any age or ethnicity. When appropriate, a doctor may recommend surgical interventions to alleviate conditions such as aneurysms, carotid artery dissection, and tumors. [QxMD MEDLINE Link]. 2002;39:185-86. Review provided by VeriMed Healthcare Network. Horner syndrome | Radiology Reference Article | Radiopaedia.org Krasnianski M, Georgiadis D, Grehl H, Lindner A. 2013 Dec 30. 2003;74:245-56. Patients with spontaneous carotid artery dissection may present with Horner syndrome and ipsilateral headache. Horner's syndrome is a triad of features resulting from interruption of the sympathetic pathway from the hypothalamus to the orbit, as follows: slight ptosis pupillary miosis: due to paralysis of the sympathetically innervated Muller's muscle which normally dilates the pupil anhydrosis over the forehead Related pages: aetiology clinical features Learn about ulnar nerve palsy symptoms, causes, and treatment. Suite 500 Tumor or infection in the top of the lung, between the lungs, and neck. Last updated: Through her writing, Dr. Huang enjoys educating patients on how to lead healthier and happier lives. Horner's Syndrome: Causes, Symptoms, and Treatments - Healthline In many cases, however, no effective treatment is known. Learn more about A.D.A.M. 11(3):282-3. The ophthalmologist will perform an eye drop test to compare the reaction of both of your pupils. [12] chiropractic manipulation, Tests to determine the location and cause of nerve dysfunction include: These usually check for tumors, lesions, and structural abnormalities. [1, 26] The following pharmacologic tests document the presence or absence of an ocular sympathetic lesion and identify the level of involvement (ie, preganglionic or postganglionic). 2010 Apr. 2015 May 26. www.eyemdlink.com/Condition.asp?ConditionID=227. Check for errors and try again. J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, The Scientific Research Honor Society, Southern Medical Association, Pan-American Association of OphthalmologyDisclosure: Nothing to disclose. [11] carotid angiography, radiofrequency tonsil ablation, The pupil of one eye is much smaller than that of the other eye, and it will stay smaller. [1] : Pancoast tumor (tumor in the apex of the lung, most commonly squamous cell carcinoma), Birth trauma with injury to lower brachial plexus Patients with cluster headaches may develop ipsilateral Horner syndrome during an acute attack. This website also contains material copyrighted by 3rd parties. J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute 2002;64:49-52. Read on to learn about these, A pinched nerve in your butt can feel like pain, weakness, tingling or other sensations. 2010 Mar. Online Mendelian Inheritance in Man (OMIM). It may be due to an underlying condition that needs prompt medical treatment. Whitehouse Station, NJ: Merck Research Laboratories; 1999:1455. Wallenberg Syndrome is a rare disorder caused by a blood clot. 25(2):83-5. [citation needed] Diagnosis Mughal M, Longmuir R. Current pharmacologic testing for Horner syndrome. [QxMD MEDLINE Link]. Horner syndrome (Horner's syndrome or oculosympathetic paresis) results from an interruption of the sympathetic nerve supply to the eye and is characterized by the classic triad of miosis (ie, constricted pupil), partial ptosis, and loss of hemifacial sweating (ie, anhidrosis), as well as enophthalmos (sinking of the eyeball into the bony cavity that protects the eye). Diagnostic value of imaging in horner syndrome in adults. 2000;51:329-47. Horner's syndrome following obstetric neuraxial blockade - PubMed 74:1243-9. Horner syndrome typically occurs when an underlying medical condition disrupts the sympathetic nerve pathway in the head and neck. 69(5):236-41. [30, 27, 26, 31] Typically given in a 0.5% or 1% solution, it has little to no effect on a normal pupil but has a mydriatic effect on an abnormal pupil. In obstetric practice, the neurological signs of Horner's syndrome may cause anxiety amongst patients and healthcare staff, but more importantly may herald the onset of maternal hypotension. Curr Neurol Neurosci Rep. 2009 Sep. 9(5):384-9. Intermittent Horner syndrome is uncommon in both the adult and pediatric population. This disruption of nerve function can cause changes in pupil size, a drooping eyelid, and a lack of sweating. Horner's Syndrome - Symptoms, Causes, Treatment | NORD Philadelphia, PA. 2003:648. Pupils different sizes: Causes and when to see a doctor Ed. ORL J Otorhinolaryngol Relat Spec. 2003;15:217-19. Horner's syndrome, also known as oculosympathetic paresis, [1] is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. Enroll in databases to allow researchers from participating institutions to find you. 4th ed. [QxMD MEDLINE Link]. Cocaine instilled in an eye with intact sympathetic innervation causes the pupil to dilate. Usually, the pupil is larger in the affected eye. In children with the congenital form of Horner syndrome, the affected iris will remain the same blue-gray color it was at birth. In addition, they may perform a series of tests to confirm a positive diagnosis and identify the site of nerve damage. Causes of acquired Horner syndrome vary widely and may include: Congenital Horner syndrome is present at birth. Stroke. Zou ZY, Yao YT. Ultrasound diagnosis of spontaneous carotid dissection with isolated Horner syndrome. This additional testing will be used to find the underlying cause of the damage. Horner syndrome is uncommon. We describe a case of a pediatric patient with an intermittent Horner syndrome. Raeder described a combination of orbital pain, miosis, and ptosis and termed it paratrigeminal syndrome. Which specialist consultations are required for optimal management of the underlying cause of Horner syndrome? It is caused by damage to the sympathetic nerves of the face. In Horner syndrome, upregulation of alpha1-receptors increases apraclonidine sensitivity and causes denervation supersensitivity of the iris dilator muscle. Horner's syndrome, classically described as ipsilateral ptosis, pupillary miosis, enophthalmus and hemifacial anhidrosis occurs when there is disruption to the sympathetic chain. Phone: 203-744-0100 Horners Syndrome. ClinicalTrials.gov, an affiliate of NIH, provides current information on clinical research studies in the United States and abroad. This syndrome results from the invasion of a number of structures and tissues around the thoracic inlet and may be characterised by: Ipsilateral invasion of the cervical sympathetic plexus, leading to Horner's syndrome (miosis, enophthalmos, ptosis) in 14-50% of patients. Reference article, Radiopaedia.org (Accessed on 28 Jun 2023) https://doi.org/10.53347/rID-7463, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":7463,"questionManager":null,"mcqUrl":"https://radiopaedia.org/articles/horner-syndrome/questions/2215?lang=us"}, Figure 1: clinical photograph - Horner syndrome, View Yuranga Weerakkody's current disclosures, see full revision history and disclosures, Philips Australia, Paid speaker at Philips Spectral CT events (ongoing). Mnchener Medizinische Wochenshrift. There are three different sections of the pathway, called neurons, that may be damaged in Horners syndrome. Horner syndrome classically consists of ptosis, miosis, and sometimes anhidrosis. Watts P, Satterfield D, Lim MK. The pupils react normally to light and accommodation. Horners syndrome is also known as oculosympathetic palsy and Bernard-Horner syndrome. 2002. Washington, DC 20036 A Podcast For The Rare Disease Community, Rare Disease Cures Accelerator (RDCA-DAP), Policy Statements & Letters to Policymakers, Stay Informed With NORDs Email Newsletter, Launching Registries & Natural History Studies, https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/, https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/, https://rarediseases.org/patient-assistance-programs/caregiver-respite/, Learn more about Patient Assistance Programs >, https://rarediseases.org/non-member-patient/nih-national-eye-institute/, Learn more about Patient Organization & Membership >. The maximal response is seen 40-60 minutes after instillation of the drops. Further testing will allow your doctor to confirm this as your diagnosis. Horner syndrome classically presents as an ipsilateral enophthalmos,blepharoptosis, pupillary miosis and facial anhydrosis due to disruption at some point of the oculosympathetic pathway. Horners syndrome can affect people of any age. Horner syndrome is a rare disorder that affects the eye and face on one side of the body. Diagnosis. Causes of Horner syndrome include the following: Brainstem stroke or tumor or syrinx of the preganglionic neuron In one study, 33% of patients with brainstem lesions demonstrated Horner syndrome In some patients the pupil may be smaller than normal rather than dilated. The presence, absence, or location of anhidrosis is an important localizing sign. Horner's syndrome is a rare condition characterized by miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face). Ultrasonography may be considered but has not been found to be reliable for diagnosing carotid artery dissection in patients with isolated Horner syndrome. Use ClincalTrials.gov button below to search for studies by disease, terms, or country. Damage to this path may be caused by the following: The second-order neuron pathway runs from the spinal column, over the upper chest area, to the side of the neck. . Journal of Neurology, Neurosurgery, & Psychiatry, 1999. Damage to this path may be caused by the following: The third-order neuron pathway runs from the neck to the skin of the face and the muscles that control the iris and eyelids. See additional information. 2022 Jan. [QxMD MEDLINE Link]. Moster ML, Galiani D, Garfinkle W. False negative hydroxyamphetamine test in horner syndrome caused by acute internal carotid artery dissection. [32, 33] Accordingly, a negative apraclonidine test result, especially in acute settings, does not exclude Horner syndrome. Horner syndrome is a condition that affects the face and eye on one side of the body. Thoracoscopic sympathectomy for palmaris hyperhidrosis. This symptom (ie, harlequin effect) occurs with physical exercise in some patients. This can be corrected by cosmetic surgery or treated with eyedrops. Horner syndrome is a relatively rare disorder characterized by a constricted pupil (miosis), drooping of the upper eyelid (ptosis), absence of sweating of the face (anhidrosis), and sinking of the eyeball into the bony cavity that protects the eye (enophthalmos). Pupillary disorders. a tumor on the neurons protective outer covering, injury or damage to either the carotid artery or jugular vein on the side of your neck, severe headaches, including migraines and cluster headaches, infection or tumor at the base of your skull, injury during birth to their shoulders or neck, a defect of the aorta in the heart that they are born with, neck pain or a headache that is sudden and severe, weak muscles or inability to control your muscle movements. Ophthal Plast Reconstr Surg. They include: flushing on one side of the face not sweating in the face the eye on the same side has a smaller (constricted) pupil with a drooping or weak eyelid Diagnosing Pancoast tumours Pancoast tumours can be difficult to diagnose. [QxMD MEDLINE Link]. Matthews BD, Bui HT, Harold KL, et al. Symptoms of the following disorders can be similar to those of Horners Syndrome. Your doctor may be able to diagnose Horner syndrome based on your history and an assessment of your symptoms. Horner Syndrome: Symptoms, Causes, Diagnosis, and Treatment - WebMD Philadelphia, PA: Elsevier; 2022:chap 17. If stroke is suspected, computed tomography (CT) of the head should be performed. Children may not have redness or flushing on the affected side of their face. A benign form characterized by episodic retrobulbar or orbital pain, with miosis and ptosis but without associated cranial nerve findings, is considered a migraine variant (ie, type II Raeder paratrigeminal syndrome). Seek medical care if you develop Horner syndrome. 34 (6):1636-1640. If carotid artery dissection is suspected, especially if there are signs or symptoms of retinal ischemia, urgent neuroimaging studies (magnetic resonance imaging or magnetic resonance angiography) should be obtained along with neurologic consultation. Males have one X and one Y chromosome, and females have two X chromosomes. 2011 Sep. 31(3):214-6. 84 (21):e175-80. The incidence of central Horner's syndrome varies from 2.5% in ophthalmological reviews to 63% in the neurological literature. StatPearls. Comparisons may be useful for a differential diagnosis. Horner syndrome in the presence of axial, shoulder, scapula, arm, or hand pain may be indicative of compression by an apical lung tumor (Pancoast tumor). Although Horner syndrome is commonly an incidental finding related to a benign cause, it occasionally may be a manifestation of a serious and life-threatening disorder. Horner Syndrome: Overview, Anatomy, Pathophysiology - Medscape Horner syndrome can appear at any time of life; in about 5 percent of affected individuals, the disorder is present from birth (congenital). EyeMDLink. 2010 Mar. This is called anhidrosis. We would like to hear your feedback as we continue to refine this new version of the GARD website. J Neuroophthalmol. Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine, Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society, Jules E Harris, MD Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center, Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research, Disclosure: GlobeImmune Salary Consulting, Malvinder S Parmar, MB, MS, FRCP(C), FACP Assistant Professor (VPT), Faculty of Medicine, University of Ottawa Faculty of Medicine; Associate Professor, Department of Internal Medicine, Northern Ontario School of Medicine; Consulting Physician, Timmins and District Hospital, Ontario, Canada, Malvinder S Parmar, MB, MS, FRCP(C), FACP is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada, J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute, J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association, Philip Schulman, MD Chief, Medical Oncology, Department of Medicine, Memorial Sloan-Kettering Cancer Center; Clinical Professor, Department of Medicine, New York University School of Medicine, Philip Schulman, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology, and Medical Society of the State of New York, Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference. Getting a proper diagnosis and finding the cause is important. Early diagnosis of Horner syndrome using topical apraclonidine. Horner syndrome (Horners syndrome or oculosympathetic paresis) results from an interruption of the sympathetic nerve supply to the eye and is characterized by the classic triad of miosis (ie, constricted pupil), partial ptosis, and loss of hemifacial sweating (ie, anhidrosis), as well as enophthalmos (sinking of the eyeball into the bony cavity that protects the eye). is also a founding member of Hi-Ethics. GARD is not currently aware of organizations specific to this condition. What is the role of a topical apraclonidine test in the diagnosis of Horner syndrome? The underlying causes can vary enormously, from a snake or insect bite to a neck trauma made by a blunt instrument. Important aspects of the physical examination include the following: The pupil on the affected side may be round and constricted (ie, miosis). What is the anatomy relevant to Horner syndrome? It may occur at any age and in any ethnicity. It usually results from damage to the sympathetic nerve supply from the brain to the eyes. Horner's syndrome is a rare neurological condition seen in association with the disruption in the sympathetic nerve supply. Horner's syndrome is a rare condition characterized by miosis (constriction of the pupil), ptosis (drooping of the upper eyelid), and anhidrosis (absence of sweating of the face). In such cases, a cocaine test should be performed to exclude Horner syndrome. . Genetic and Rare Diseases Information Center, 2023. Horner syndrome can involve disruption along three different nerve pathways.