Brown syndrome is a rare disorder of the eye distinguished by pathological changes of the superior oblique muscle and its tendon, resulting in the inability to elevate the eye while being in adduction. Both congenital and acquired forms are described, and the diagnosis can be made only after a thorough physical examination and a detailed patient history.
Presentation
Brown syndrome is a relatively rare condition of the eye apparatus, in which various factors render the superior oblique muscle unable to perform its respective function - elevation of the eye while being in adduction, principally as a result of mechanical limitation [1] [2] [3] [4]. Numerous conditions have been implicated in the pathogenesis of this ocular disorder, including juvenile and rheumatoid arthritis, systemic diseases (scleroderma, Sjogren's syndrome, systemic lupus erythematosus), orbital metastatic deposits, but also trauma, inflammation, and surgery, all leading to restriction of superior oblique tendon movement [1] [2] [3] [5]. On the other hand, many individuals develop Brown syndrome from birth, indicating that congenital forms of the disease also exist [1] [2] [4] [5]. Furthermore, familial Brown syndrome, estimated to occur in approximately 1 in 20,000 live births, was also recognized in the literature [1] [3]. The clinical presentation is characterized by the inability of patients to elevate the eye in adduction, producing predominantly unilateral (although 10% of cases exhibit bilateral) changes in eye movement [1] [2] [3]. Consequently, a slight downward deviation of the eye on adduction is noted [1] Studies did not show a predilection toward gender or either of the eyes [1]. In the setting of the congenital Brown syndrome, spontaneous resolution is seen in 11-76% of children, but complications such as hypertropia, amblyopia, and other visual deficits may develop [1] [4] [6].
Entire Body System
- Disability
For example, Timmons and Brown (1997) have indicated how teenagers who are disabled are required to go to bed much earlier than teenagers who are not so disabled. [library.down-syndrome.org]
Documents World Down Syndrome Day (A/RES/66/149) UN Disability Inclusion Strategy Convention for the Rights of People with Disabilities Principles for the protection of persons with mental illness and the improvement of mental health care Promotion and [un.org]
Although adaptive mechanisms, such as an anomalous head posture, may develop, patients are left with a permanent disability that could not be reversed. One should scrupulously adhere to the indications for surgery in Brown syndrome. [ncbi.nlm.nih.gov]
One study, for example, found SLC9A6 mutations in two of 200 people suspected of having X-linked intellectual disability. [news.brown.edu]
Having an intellectual disability Back to top Down syndrome is the most common cause of intellectual disability that we know of. Everyone who has Down syndrome will have some level of intellectual disability. [downsyndrome.org.au]
- Fever
There was no history of trauma, any previous surgery, any systemic illnesses, joint pain, skin rashes, or fever. On examination, she had a chin up posture with a left head tilt. Her visual acuity was 20/20, N6 in both eyes. [ojoonline.org]
Fever. While having a fever doesn't cause Brugada syndrome by itself, a fever can irritate the heart and stimulate a Brugada-triggered faint or sudden cardiac arrest, especially in children. [mayoclinic.org]
These include: a high temperature (fever) – if you develop a fever of 38C (100.4F) or above, take painkillers such as paracetamol to bring it down; get medical advice as soon as possible if this doesn't help drinking too much alcohol – avoid drinking [nhs.uk]
However, PANDAS patients test positive for a recent streptococcal infection, such as strep throat, peri-anal strep or scarlet fever. [med.stanford.edu]
Frequent infections and fever Frequent infections and fever may be caused by low levels of white blood cells, also known as neutropenia. A low white blood cell count is known as leukopenia. [healthline.com]
- Pathologist
Pathology, University of Chicago James Bowman, MD is a member of the following medical societies: Alpha Omega Alpha, American Society for Clinical Pathology, American Society of Human Genetics, Central Society for Clinical Research, and College of American Pathologists [emedicine.medscape.com]
[…] epilepsy; therefore, neurologic evaluation may be needed Orthopedic specialist Child psychiatrist - A child psychiatrist should lead liaison interventions, family therapies, and psychometric evaluations Physical and occupational therapist Speech-language pathologist [jbiomedsci.biomedcentral.com]
Cardiovascular
- Chest Pain
Women are more likely than men to experience the sudden, intense chest pain — the reaction to a surge of stress hormones — that can be caused by an emotionally stressful event. [heart.org]
Some people experience: blackouts fits (seizures) occasional noticeable heartbeats (palpitations), chest pain, breathlessness, or dizziness These symptoms can occur at any time, but are sometimes triggered by something such as a high temperature (fever [nhs.uk]
Other symptoms of anemia include: pale skin lightheadedness, dizziness cold hands and feet general weakness irregular heartbeat headache chest pain Symptoms of anemia tend to get worse over time. 2. [healthline.com]
No fever, loss of appetite, abdominal pain, jaundice, behavioral changes were noted. There were no other constitutional symptoms like night sweats, chills, chest pain and easy fatigability suggestive of Lymphoma. [clinmedjournals.org]
[…] duodenal web or atresia Absence of stools secondary to Hirschsprung disease Delay in cognitive abilities, motor development, language development (specifically expressive skills), and social competence Arrhythmia, fainting episodes, palpitations, or chest [emedicine.medscape.com]
Musculoskeletal
- Fracture
When repairing medial orbital wall fracture that causes Brown syndrome, surgeons should always be careful of entrapment of the superior oblique muscle if the implant is inserted without identifying the superior and posterior margin of the orbital fracture [ncbi.nlm.nih.gov]
None has been observed in such a high-energy fracture/dislocation pattern. Despite the severity of the fracture, the patient recovered fully at 6 months follow-up. [asianjns.org]
- Muscle Weakness
The most frequent presenting symptoms are cranial neuropathy, sensory ataxia, muscle weakness and respiratory insufficiency due to diaphragmatic paralysis. [orpha.net]
In any child where there is evidence of muscle weakness with cranial nerve problems and/or respiratory problems (such as facial paralysis or vocal cord paralysis), BVVL should be considered a possible cause. [facialpalsy.org.uk]
Symptoms of an under-active thyroid gland can include: lethargy (lack of energy) weight gain slow physical and mental reactions muscle aches and weakness dry, flaky skin See the Health A-Z topic about Hypothyroidism for more information. [hse.ie]
Children presenting before 3 years of age present more commonly with respiratory distress, bulbar palsy (both 86%), muscle weakness, and hearing loss (both 67%).[1] It is hypothesized that the children who present very young also succumb early, thus precluding [pediatricneurosciences.com]
- Contusion
MRI scans were also taken, showing cord contusion but no clear epidural haematoma despite the presence of a Horner's syndrome. [emj.bmj.com]
[…] considered superior in detecting the extent of spinal cord injury, spinal alignment, damage of the intervertebral discs, and involvement of the surrounding soft tissue. [3] Signal cord hyperintensity was noted on T2-weighted sagittal MRI suggestive of a contusion [asianjns.org]
Orbital Fracture -- Blow-Out Fracture Traumatic contusion to the orbit (the bone surrounding the eyeball) may result in a fracture of the floor of the orbit known as a blow-out fracture. Diagnosis is best made with a CAT scan not a MRI. [strabismus.org]
- Neck Pain
Doctors participating in trauma management need to be thorough in their examination of the nervous system even in the absence of neck pain and a “normal” C-spine radiograph. [emj.bmj.com]
Often, people will experience symptoms like headaches, TMJ Dysfunction, all kinds of body pain ranging from joints, hips, sciatic, bladder leakage, shoulder, back, head, and neck pain. [therolfer.com]
pain and left-sided weakness of the upper and lower extremities. [asianjns.org]
- Joint Dislocation
“Because of this, those with EDS frequently have joint sprains and dislocations, as well as frequent joint aches, pains and arthritis,” Dr. Smith says. The other symptoms of EDS can cause issues as well. [self.com]
Neurologic
- Stroke
Stroke A neurological event, like a stroke, is the most common reason adults experience a turning of the eye, such as hypertropia. A blood clot that leads to a stroke can also damage the nerves that help control the movement of the eyes. [healthline.com]
We treat patients with complex strabismus arising from thyroid related eye disease, stroke and brain tumors as well as strabismus disorders following severe orbital and head trauma. [pedseye.com]
These occurred in 14 (70%) of 20 patients described by Maria et al. [22] Garcia et al reported recurrent thrombotic episodes. [18] Stroke also may occur. [emedicine.medscape.com]
Stroke. Spinal tumours. Investigations Laboratory studies may be useful with non-traumatic causes. Overall they are not usually necessary for diagnosis. [patient.info]
Other causes include local ophthalmological conditions, CN III dysfunction attributable to, for example, uncal herniation, posterior communicating artery aneurysm, tumours, strokes, diabetes, Lyme disease or alcoholism, and pharmacological glaucoma medications [emj.bmj.com]
- Paresthesia
SSN usually presents with asymmetric pain, paresthesia and dysesthesia type of numbness of the extremities. [clinmedjournals.org]
There were no associated visual field deficits, vertigo, facial weakness, or paresthesia. A review of laboratory values showed positive antinuclear antibody, elevated sedimentation rate, and C-reactive protein levels. [ncbi.nlm.nih.gov]
- Myelopathy
(Other studies, however, have demonstrated improved outcomes for patients with traumatic SCIs who were given high-dose steroids early in the clinical course. [16] ) Surgical treatment of stenosis with myelopathy or incomplete spinal cord injury, including [emedicine.medscape.com]
External links[edit] Case studies of Brown-Séquard syndrome Classification D ICD-10: G83.8 ICD-9-CM: 344.89 MeSH: D018437 DiseasesDB: 31117 External resources eMedicine: emerg/70 pmr/17 v t e Lesions of spinal cord and brain Spinal cord/ vascular myelopathy [en.wikipedia.org]
- Meningism
Neurology (1) A condition characterised by congenital analgesia with neurogenic anhidrosis, loss of deep and superficial pain sensation, dental dysplasia, meningeal thickening with cystic degeneration, hyperreflexia, mild mental retardation Lab Abnormal [medical-dictionary.thefreedictionary.com]
Infectious causes: eg, meningitis, empyema, herpes zoster virus, herpes simplex virus, tuberculosis, syphilis. Other causes: include gnathostomiasis (helminthic parasitic disease), and tropical spastic paraplegia (HTLV-1). [patient.info]
- Vertigo
Vertebral arteritis may result in auditory loss and vestibular dysfunction (vertigo). Abdominal claudication and bowel necrosis are reported. Erythrocyte sedimentation rate and C-reactive protein are elevated. [accessanesthesiology.mhmedical.com]
There were no associated visual field deficits, vertigo, facial weakness, or paresthesia. A review of laboratory values showed positive antinuclear antibody, elevated sedimentation rate, and C-reactive protein levels. [ncbi.nlm.nih.gov]
Workup
The diagnosis of Brown syndrome can be made only after an extensive workup. Because the initial diagnosis of an eye-related pathology might be made by a simple evaluation of eye movement, the role of a properly performed physical examination is pivotal in recognizing this condition. Apart from the obvious inability to perform eye elevation while being in adduction, several other features that are seen during the exam are a downward deviation in adduction, widening of the palpebral fissure when the patient adducts the eye and the absence of over-activation of the superior oblique muscle [1]. Additionally, the forced duction test, during which the limbus is grasped by a forceps with the attempt to move the eye mechanically, will be positive - restricted movement will be observed due to the mechanical nature of the disease. In that case, the diagnosis of Brown syndrome is almost definite [1]. To identify the exact cause of this condition, a thorough patient history is helpful in distinguishing between congenital and acquired forms. Age of onset, as well as a history of previous conditions or events that could lead to Brown syndrome, should be assessed. Imaging studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) have been regarded as potentially useful tools in solidifying the diagnosis [5]. Thickening of the superior oblique tendon on T1 and high signal intensity on T2-weighed imaging have been reported in this condition [5].
Treatment
Secondary cases, due to inflammation in the trochlear notch can benefit from steroid treatment. [eyewiki.aao.org]
Techniques and results of treatment for Brown syndrome at two institutions were reviewed. [ncbi.nlm.nih.gov]
Other treatment is symptomatic and supportive. × Treatment Generally treatment for individuals with BSS focuses on the underlying cause of the disorder. Early treatment with high-dose steroids may be beneficial in many cases. [ninds.nih.gov]
The various etiologic theories are reviewed and the spectrum of medical and surgical treatments are described and evaluated. [scienceopen.com]
Prognosis
Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. x Prognosis The prognosis for individuals with BSS varies depending on the cause of the disorder. [ninds.nih.gov]
If the cervical spinal cord lesion is incomplete, such as central cord or Brown-Séquard syndrome, younger patients with have a more favorable prognosis for recovery. [emedicine.medscape.com]
Prognosis The prognosis for Brown-Séquard's syndrome is generally poor although it may be better than other forms of spinal cord injury.[13] Aetiology can have a bearing on prognosis. [patient.info]
10–15% functional recovery; if no recovery is evident and progressive after 24 hours, prognosis is poor Vibration and proprioception are typically spared because of an intact dorsal column! [amboss.com]
Treatment and prognosis Prognosis of anterior cord syndrome is worst among all other spinal cord injury syndromes 5. It is associated with high mortality and poor functional outcome in terms of poor recovery of motor power and coordination. [radiopaedia.org]
Etiology
Acquired nonsuperior oblique Brown syndrome Etiology of acquired nonsuperior oblique Brown syndrome Acquired nonsuperior oblique etiologies: Inferior orbital fibrous adhesions to the posterior globe are caused by the following: orbital floor fracture [emedicine.com]
In terms of etiology, Brown syndrome has two distinct forms – congenital and acquired. [ojoonline.org]
Occurrences of Brown syndrome are rare, hence the difficulty in establishing the exact etiology. [ncbi.nlm.nih.gov]
Further workup may be needed in acquired Brown syndrome and often depends on the suspected underlying etiology. [eyewiki.aao.org]
Epidemiology
Terminology Also called sideropenic dysphagia Epidemiology Occurs mostly in Scandinavia and Great Britain Usually women ages 30+ years Treatment Iron supplements, mechanical dilation and monitor for upper GI carcinoma Clinical images Images hosted on [pathologyoutlines.com]
To mark the occasion of World Down Syndrome Day, the JRC publishes a report describing the epidemiology of Down syndrome in Europe over the last 25 years. [ec.europa.eu]
Epidemiology[edit] In Brown's original series there was a 3:2 predominance of women to men and nearly twice as many cases involved the right eye as the left. 10% of cases showed bilaterality. [en.wikipedia.org]
Pathophysiology
CT is a valuable tool in understanding the pathophysiology and management of acquired Brown syndrome, showing thickening and inflammatory changes of the reflected portion of the superior oblique tendon. [ncbi.nlm.nih.gov]
CT is a valuable tool in understanding the pathophysiology and management of acquired Brown syndrome, showing thickening and inflammatory changes of the reflected portion of the superior oblique tendon. Article History Published in print: 1985 [pubs.rsna.org]
In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. https://www.clinicalkey.com. Accessed May 12, 2017. AskMayoExpert. [mayoclinic.org]
Prevention
Surgical exploration revealed adhesions that prevented the normal sliding of the superior oblique tendon beneath the superior rectus muscle. Forced ductions became normal and downgaze improved after removal of the silicone band. [ncbi.nlm.nih.gov]
Prognosis and prevention[3, 12] Correction of iron deficiency will correct the condition if malignancy has not yet occurred. Prevention of iron deficiency prevents the disease. Historical notes[2] Henry Plummer (1874-1937) was an American internist. [patient.info]
Things You Can Do For Hand-Foot Syndrome: Prevention: Prevention is very important in trying to reduce the development of hand-foot syndrome. Actions taken to prevent hand-foot syndrome will help reduce the severity of symptoms should they develop. [chemocare.com]
[…] excavatum, pectus carinatum, and slipping rib syndrome Research Interests Pediatric trauma; injury prevention; chest wall deformities Academic Affiliation Professor, UC Department of Surgery Departments Colorectal Disorders, Injury Prevention, Surgery [cincinnatichildrens.org]
References
- Olusanya BA. Brown syndrome with severe amblyopia: a case report from Africa. Pan Afr Med J. 2015;20:56.
- Cho YA, Kim S, Graef MH. Surgical Outcomes in Correction of Brown Syndrome. Korean J Ophthalmol. 2006;20(1):33-40.
- Kenawy N, Pilz DT, Watts P. Familial unilateral Brown syndrome. Indian J Ophthalmol. 2008;56(5):430-434.
- Lambert SR. Late spontaneous resolution of congenital Brown syndrome. J AAPOS. 2010;14(4):373-375.
- Currie S, Goddard T. MR imaging features of acquired Brown syndrome. AJNR Am J Neuroradiol. 2009;30(9):1778-1779.
- Kent JS, Makar I. Hypertropia following Spontaneous Resolution of Brown’s Syndrome. Case Rep Ophthalmol. 2012;3(2):230-235.