The Merck Manual-Home Edition.2nd ed. Digre KB, Smoker WR, Johnston P, Tryhus MR, Thompson HS, Cox TA, Yuh WT. Injury to the axillary nerveand resulting paralysis of the deltoid muscleusually results from shoulder trauma such as dislocation and/or fracture. Acquisition of data: M. A. Sabbagh, L. B. Evaluation and neuroimaging of the Horner syndrome. Anisocoria due to Horner syndrome associated with pain should be treated as an emergency warranting immediate recognition. You may need to be referred to a doctor who specializes in vision problems related to the nervous system (neuro-ophthalmologist). 1982;39:108111. Maloney WF, Younge BR, Moyer NJ. Disclaimer. We did not assign cluster headache as a diagnosis unless carotid imaging was negative and the patient had periodic headache with at least a suggestion of other trigeminal autonomic symptoms. 6. Some reports have indicated that when a cause for Horner syndrome can be determined, it is most often chest and neck tumors and stroke. Among the 159 apraclonidine-confirmed cases, clinical examination and apraclonidine testing were conducted at the University of Michigan Medical Center under the supervision of the 3 faculty neuro-ophthalmologists in 158 cases. Horner syndrome occurs due to interruption of the oculosympathetic chain that begins in the hypothalamus, travels through the spinal cord to the thorax, and ascends along the internal carotid artery to the orbit. Parsonage-Turner Syndrome (PTS), also referred to as idiopathic brachial plexopathy or neuralgic amyotrophy, is a rare disorder consisting of a complex constellation of symptoms with abrupt onset of pain in the shoulder, which is followed by progressive neurologic increasing of motor weakness, a reduced sense of touch, and numbness. These are the four classic signs of the disorder. affect pupil, Systemic diseases associated with third nerve Arch Neurol. 2000;15:124-28. The .gov means its official. However, no genes associated with the condition have been identified. Drooping of the upper eyelid in the affected eye Facial Center for Brachial Plexus and Traumatic Nerve Injury, About Brachial Plexus and Traumatic Nerve Injury, Brachial Plexus Doctors, Surgeons and Specialists, Seeing a Physician at the Center for Brachial Plexus and Traumatic Nerve Injury, Symptoms of Brachial Plexus and Serious Nerve Injury. The earliest apraclonidine-confirmed case occurred in the year 2005. In the cohort of 159 apraclonidine-confirmed cases of Horner syndrome, a cause was identified in 97 (61%). Referred to as burners or stingers, they make up approximately10 percent of all cervical spine neurologic injuries, and two-thirds of all college football players experience at least one of these injuries. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. In a cohort of 159 cases of Horner syndrome not tested with apraclonidine because the clinical diagnosis appeared firm, procedures again accounted for the largest percentage, but tumor was the next most common cause. The 50% cut point for diagnosed cases occurred in 2014. In our study of 159 pharmacologically-confirmed cases, a cause remained undetermined in nearly half of them, whereas in the 159 pharmacologically-unconfirmed cases, a cause was found in nearly all cases. Traumatic brachial plexus injuries, which are most commonly sustained in high-speed motor vehicle accidents or while engaged in sporting events, affect the sensibility and muscle power in part of or the entire limb. All strokes had produced multiple neurologic manifestations, among which Horner syndrome was a minor component. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Comparisons may be useful for a differential diagnosis. There were 2 cases each of carotid-cavernous fistula and congenital Horner syndrome, along with a case each of Aspergillus infection in the neck and sarcoidosis in the neck. De Lott, and J. D. Trobe; b. It was retrospective, so that data were originally gathered as part of a clinical rather than a research assessment. Neurol Clin. The data were drawn from just 1 institution, including only a small number of children. The most common area is a pinched nerve in the cervical spine as a result of arthritis and disc disease. 2000;51:329-47. These patients require evaluation to look for these problems. Procedures in the neck, chest, skull base, and paraspinal region accounted for most of the identified causes, with cervical carotid dissection the next most common cause. Journal of Neuro-Ophthalmology40(3):362-369, September 2020. https://accesspediatrics.mhmedical.com/content.aspx?bookid=527§ionid=41074817. 2001;29:411-15. Copyright illness.com. Hanauer DA, Mei Q, Law J, Khanna R, Zheng K. Supporting information retrieval from electronic health records: a report of University of Michigan's nine-year experience in developing and using the Electronic Medical Record Search Engine (EMERSE). New York, NY; 1997:538. Although congenital Horner syndrome can be passed down in families, no associated genes have been identified. The underlying causes can vary enormously, from a snake or insect bite to a neck trauma made by a blunt instrument. Carotid dissection accounted for 14 patients, of which 7 were unprovoked and 7 were associated with sudden neck strain (motor vehicle accident4, chiropractic neck manipulation1, yoga exercises1, and heavy lifting1). We presume that carotid dissection caused Horner syndrome in some of the cases attributed in earlier reports to neck trauma. In the pharmacologically-unconfirmed cases, it was not possible to determine reliably whether the diagnosis was first made in the outpatient or inpatient setting. In the pharmacologically-confirmed cases, the diagnosis of Horner syndrome was made on outpatients in 79% of cases, an expected result because testing with apraclonidine is more practical in the outpatient setting. (For more information on this disorder, choose Adie Syndrome as your search term in the Rare Disease Database.). See Privacy Policy for more information. 5) Tumors account for an important proportion of causes; if a tumor has not already been identified at the time of Horner diagnosis, appropriate imaging is justified. Two recent reports (8,15) mention carotid dissection as the explanation for 40% (8) and 18% (15) of identifiable causes of Horner syndrome. It is characterized by a drooping eyelid (ptosis), decreased pupil size (myosis), and dryness of the eye (anhidrosis). 2pp. However, in an important minority of cases where the cause was not yet known, the identification of Horner syndrome was valuable in leading to important diagnoses such as carotid dissection and tumor. 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 2015;50:107111. The brachial plexus refers to a complex web of large nerves that exit from the spinal cord in the neck and direct the movement and sensation of the entire upper limb. We used strict definitions of causation. not react at all, referral is also indicated. Leuchter I, Becker M, Mickel R, et al. 2022 May 23;14(5):e25242. In both groups, when a cause for Horner syndrome could be identified, that cause was nearly always known before Horner syndrome was identified. It results from damage to the oculosympathetic pathway, which extends from the hypothalamus to Horner syndrome that occurs very early in life can lead to iris heterochromia because the development of the pigmentation (coloring) of the iris is under the control of the cervical sympathetic nerves. Causes of Horners Syndrome. The international classification of headache disorders, 3rd edition. Approximately 15% of brachial plexus injuries have an injury to the blood supply of the arm as well, and emergency surgery may be indicated. particularly if it is variable. Patients with double crush syndrome have injuries or compression in two separate areas. 3. Cluster headache accounted for about 10% of causes in our study, as it did in the studies of Sadaka et al (7) and Maloney et al (4), but not in other studies. McGraw-Hill Companies. The cause of Horners syndrome often depends on which of the three sympathetic nervous system pathways are disrupted. Carotid dissection was the second most common identifiable cause of Horner syndrome in our study. Tumors accounted for 5% of identifiable causes among our pharmacologically-confirmed cases, yet 28% in pharmacologically-unconfirmed cases. In both groups, when a cause for Horner syndrome could be identified, that cause was nearly always known before Horner syndrome was identified. By contrast, when the Horner diagnosis depends on pharmacologic confirmation, as occurs often in outpatients, its cause will often remain unknown. Careers. 2003;23:22-23. 2pp. Causes Horner syndrome can be caused by any interruption in a set of nerve fibers that start in the part of the brain called the hypothalamus and travel to the face and eyes. 2012;20:145152. Before Would you like email updates of new search results? Patients with injuries to the brachial plexus or other major nerve injuries near the neck or shoulder may have complete or partial loss of function in the arm, with or without sensation. Horners syndrome caused by a thoracic dumbbell-shaped schwannoma: sympathetic chain reconstruction after a one-stage removal of the tumor. older children, may result from serious diseases such as neuroblastoma. due to scarring of the iris may be the first abnormality noted. Este programa de asistencia, primero en su tipo, est diseado para los cuidadores de un nio o adulto diagnosticado con un trastorno raro. 2008;48:467470. Disruption of First-Order Neurons. Bethesda, MD 20894, Web Policies Curr Opin Ophthalmol. Horners syndrome is often the result of an interruption to this group of nerves through physical injury. Horner syndrome classically consists of ptosis, miosis, and sometimes anhidrosis. Note: Both Erbs palsy and Klumpkes palsy may occur in adults. J Neuroophthalmol. Third-order Horner syndrome results from damage to the nerve path that travels from your neck to your middle ear and eye. Problems may result from: Lesions on your carotid artery. Middle ear infections. Injury to the base of your skull. Migraine or cluster headaches. Raeder paratrigeminal syndrome. McKusick VA, Ed. 10. For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office: For information about clinical trials sponsored by private sources, contact: Salvesen R. Horner Syndrome. When surgical intervention is necessary, good outcomes can be achieved in appropriately screened patients. Descriptive statistics were used to describe patient demographics. It may occur with a lack of color (pigmentation) of the iris (colored part of the eye). 185.6.9.159 Wallenberg Syndrome is a rare disorder caused by a blood clot. 2010 Mar;30(1):12-7. doi: 10.1097/WNO.0b013e3181b1b41f. Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder. Absent or poor reflexes are also associated with this disorder. Cluster headache was the cause in 12 patients, determined only after carotid imaging was negative. Cases were accrued through a search that detected all text mentions of Horner syndrome with and without pharmacologic confirmation, allowing for a broader inclusion than studies dependent on personal files or discharge diagnoses. Where the cause is not yet known, localization to the central, preganglionic, or postganglionic segment of the oculosympathetic pathway by pupil reactions to topical adrenergic agents is not sufficiently accurate to be useful (10). 9. 2pp. Depending on the suspected cause, tests may be done, such as: There are no direct complications of the syndrome itself. The yield of diagnostic imaging in patients with isolated Horner syndrome. Our study included 9 patients with neck trauma and negative carotid artery imaging in whom we presume the mechanism was contusion of the Horner fibers adjacent to the carotid wall. Among apraclonidine-unconfirmed cases, more than 2,000 cases were identified, so that we included only the first 159 cases that met inclusion criteria. Children These cases are referred to as idiopathic. Therefore, it often goes undocumented. 15. Prompt evaluation is necessary to detect and treat potentially life 2022 Nov 21;2022:8670350. doi: 10.1155/2022/8670350. Apraclonidine for the pharmacologic confirmation of acute Horner syndrome. side, Unequal pupil color in congenital or early acquired cases, Exposure to topical medications or plants that De Lott, and J. D. Trobe. Chandrasekhar S, Peterfreund RA. The main ones include 3: Central causes hypothalamic, thalamic or brainstem ischemia (e.g. The numbered bands specify the location of the thousands of genes that are present on each chromosome. Trauma, mostly to the neck, accounted for an important proportion of Horner syndrome in our study, as it has in other studies. Genetic counseling may be of benefit for patients and their families if they have the genetic form of this disorder. 2003;14:357363. Asegurarse de que los pacientes y los cuidadores estn equipados con las herramientas que necesitan para vivir su mejor vida mientras manejan su condicin rara es una parte vital de la misin de NORD. 14. Some cases of Horner syndrome occur for no other apparent reason or unknown cause (idiopathically). Stroke was an infrequent cause of Horner syndrome in our study. Some patients with winged scapula are still able to use the arm on the affected side, but prompt treatment is recommended, even in instances when the condition is not caused by traumatic injury. Each chromosome has a short arm designated p and a long arm designated q. In some of these patients, abnormal pupils If an adequate diagnostic evaluation failed to determine a probable cause of Horner syndrome, we labeled the cases idiopathic. If an adequate evaluation had not been performed, or we lacked sufficient information to assign a reasonable cause for Horner syndrome, we considered the cause to be uncertain. Cases that were idiopathic or uncertain were considered to be of indeterminate cause. We used strict definitions of all causes. 2) When a cause for Horner syndrome is found, our study suggests that it will most often be a procedure damaging the oculosympathetic pathway and it will almost always be known before the diagnosis of Horner syndrome is made, making Horner syndrome frequently a clinical afterthought. The symptoms only occur on one side and may include weakness and paresthesia (numbness or tingling) in the involved extremity for several seconds to several minutes. It is characterized by miosis (a constricted pupil), partial ptosis (a weak, droopy eyelid), apparent anhidrosis (decreased sw Horner syndrome. Treatment of these injuries by many different specialists addresses both the underlying condition and associated pain. may email you for journal alerts and information, but is committed Among the reports that have described the causes and evaluation of Horner syndrome in at least 50 cases (28), a 1958 study of 216 patients, based on discharge summaries, found that tumorsoften malignant and mostly in the chest and neckwere responsible for more than one-third of cases (2). Pairs of human chromosomes are numbered from 1 through 22, and the sex chromosomes are designated X and Y. Damage to the cervical sympathetic nerves can be caused by a direct injury to the nerves themselves, which can result from trauma that might occur during a difficult birth, surgery, or accidental injury. The incidence of Horner syndrome that appears later is unknown, but it is considered an uncommon disorder. Asia Pac J Ophthalmol. The sympathetic and spinothalamic pathways in the brain stem lie throughout their course Among apraclonidine-confirmed cases, 159 met inclusion criteria. Pseudo-Horner's syndrome. Pediatric Horners syndrome due to a cervical thymic rest. We surmise that when a diagnosis of Horner syndrome is made without pharmacologic confirmation, it is often based on a cause identified in inpatients. 2010;30:7072. https://accesspediatrics.mhmedical.com/content.aspx?bookid=527§ionid=41074817. In the 159 patients with pharmacologically-unconfirmed Horner syndrome, most cases (91%) had an identifiable cause (Fig. Tumors accounted for 28%, the second highest proportion. We applied a novel search engine to the inpatient and outpatient electronic medical record text in a single large tertiary care academic center, dividing the cohorts into those with and without pharmacologic confirmation. Miller PR, Fabian TC, Croce MA, et al. WebPhysiological anisocoria does not cause any problems with development of vision. Please click Continue to continue the affiliation switch, otherwise click Cancel to cancel signing in. Inclusion of more children might have altered the causation prevalence. Nguyen MTB, Farahvash A, Zhang A, Micieli JA. Others have suggested that Horner syndrome is more frequently caused by surgical procedures in the neck and chest. Among them were 3 acute carotid dissections without recent neck pain or trauma and 2 tumorsan anaplastic thyroid cancer and a cervical neuroblastoma. About 1 in 6,250 babies are born with this syndrome. We further acknowledge that the prevalence of causes might have been biased because the imaging protocol did not always include the intracranial space or the orbit and the sensitivity of imaging improved over the study period. Selective MR imaging approach for evaluation of patients with Horner's syndrome. All individuals carry a few abnormal genes. Philadelphia, PA. 2003:648. Clinical clues will be more useful, as they were in some of our cases (6). Recognizing Horners syndrome. For pharmacologic confirmation, we relied on topical apraclonidine, a now more commonly used pharmacologic indicator of Horner syndrome than cocaine, epinephrine, or hydroxyamphetamine, which were used in prior studies. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. Most patients in those reports had not undergone definitive vascular imaging. In the pharmacologically-confirmed Horner cohort, 97 (62%) had Horner syndrome on the right side and 59 (38%) on the left side. Terms of Use Among the 159 pharmacologically-unconfirmed cases, only 13 had been examined under neuro-ophthalmologic supervision. When a cause of Horner syndrome was found in both cohorts, it was usually known before Horner syndrome was discovered, making Horner syndrome an afterthought. Among pharmacologically-confirmed cases, the cause was often undetermined or due to a preceding neck or chest procedure. When Horner syndrome follows a procedure or stroke that could have damaged the oculosympathetic pathway, there is no need for further evaluation. Stroke can also cause central Horners and can present alongside other cranial nerve palsies. The most common stroke syndrome is Wallenbergs (lateral medullary) syndrome affecting the posterior inferior cerebellar artery. This commonly presents as Horners with ipsilateral ataxia, dysphagia, hoarseness and reduced gag reflex. 5 In brachial plexus injuries, the term avulsion is used to describe a condition where the roots of the nerves are torn away from the spinal cord. This site needs JavaScript to work properly. The cause of Horners syndrome often depends on which of the three sympathetic nervous system pathways are disrupted. severe inflammation. Among pharmacologically-confirmed cases, the cause was often undetermined or due to a preceding neck or chest procedure. Others have suggested that Horner syndrome is more frequently caused by surgical procedures in the neck and chest. Your message has been successfully sent to your colleague. An unstable shoulder may result in other conditions such as bursitis and chronic pain, and difficulty raising the shoulder. See this image and copyright information in PMC. Clinical Ophthalmology. 1) When the diagnosis of Horner syndrome is based on pharmacologic confirmation, it is often an isolated abnormality and a definite cause will often not be found even in the face of adequate investigation. However, in an important minority of cases, mostly involving carotid dissection or tumor, the identification of Horner syndrome was critical to the discovery of those conditions. 3) If the cause is not known at the time of Horner syndrome diagnosis, carotid dissection may be the cause even if acute neck or face pain is not described. Am J Ophthalmol. Sadaka A, Schockman SL, Golnik KC. Fetzer SJ. The evaluation of Horner syndrome. Treatment may be administered in stages, addressing the more significant compression first. Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. 2002;236:386-93; discussion 393-35. Human body cells normally have 46 chromosomes. Horner's syndrome: an analysis of 216 cases. Compression or stretching of a nerve root or the brachial plexus can cause a sharp, burning pain that may radiate into the shoulder and down the arm to the hand. In these 318 cases, we documented patient demographics, ophthalmologic features, imaging, underlying cause, and whether the cause was discovered before or after the diagnosis of Horner syndrome.
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