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Mucous membrane pemphigoid is a chronic autoimmune disease that mainly affects mucous membranes. Skin lesions occur in 10–43% of patients. The conjunctiva is involved in about 70% of patients. The component of the disease that affects the conjunctiva, regardless of involvement of nonocular sites, is commonly called ocular cicatricial pemphigoid. It is a bilateral, asymmetric, sight-threatening disease characterized by progressive conjunctival cicatrization associated with trichiasis, eyelid entropion, symblepharon, secondary corneal neovascularization, and scarring. It is characterized by IgG, IgA, or C3 (complement component) deposition in a linear fashion along the epithelial basement membrane zone. The disease usually presents between the ages of 30 and 90 years; it affects males twice as often as females.

Eye pain is a common complaint, but no previous studies have determined the most common causes of this presenting symptom. Our objective was to determine the most common causes of eye pain in 2 ophthalmology and neurology departments at academic medical centers.This was a retrospective cross-sectional analysis and chart review at the departments of ophthalmology and neurology at the University Hospital Zurich (USZ), University of Zürich, Switzerland, and the University of Utah (UU), USA. Data were analyzed from January 2012 to December 2013. We included patients aged 18 years or older presenting with eye pain as a major complaint.Two thousand six hundred three patient charts met inclusion criteria; 742 were included from USZ and 1,861 were included from UU. Of these, 2,407 had been seen in an ophthalmology clinic and 196 had been seen in a neurology clinic. Inflammatory eye disease (conjunctivitis, blepharitis, keratitis, uveitis, dry eye, chalazion, and scleritis) was the underlying cause of eye pain in 1,801 (69.1%) of all patients analyzed. Although only 71 (3%) of 2,407 patients had migraine diagnosed in an ophthalmology clinic as the cause of eye pain, migraine was the predominant cause of eye pain in the neurology clinics (100/196; 51%). Other causes of eye pain in the neurology clinics included optic neuritis (44 patients), trigeminal neuralgia, and other cranial nerve disorders (8 patients).Eye pain may be associated with a number of different causes, some benign and others sight- or life-threatening. Because patients with eye pain may present to either a neurology or an ophthalmology clinic and because the causes of eye pain may be primarily ophthalmic or neurologic, the diagnosis and management of these patients often requires collaboration and consultation between the 2 specialties.

OBJECTIVE Migraine is associated with several important visual symptoms, during both acute attacks and headache-free intervals. The purpose of this investigation was to use validated vision-related quality of life instruments to assess the effect of migraine on visual quality of life. BACKGROUND Many migraineurs experience visual aura, increased photophobia during and between headache attacks, and increased symptoms of dry eye with structural changes in corneal nerve endings. Other visual symptoms associated with migraine include positive persistent visual phenomenon (visual snow) and transient vision changes. Previous research looking at the disability associated with migraine has shown that patient-reported quality of life data can be useful in determining the severity of disease burden. Recent published literature has suggested that visual symptoms related to migraine represent a proportionally minor burden to patients, compared to other manifestations of migraine, but no previous studies have assessed how migraine affects visual quality of life. METHODS In this cross-sectional quantitative survey, visual quality of life in individuals with chronic and episodic migraine was assessed using the National Eye Institute Visual Function Questionnaire-25, and the 10-item National Eye Institute Visual Function Questionnaire-25 Neuro-Ophthalmic Supplement. Overall headache severity and impact was assessed using the Migraine-specific Quality of Life Questionnaire (Version 2.1) and the Headache Impact Test-6. Participants were recruited from Headache and Neuro-ophthalmology subspecialty clinics. The target sample size was 30 participants per subgroup. The results were compared to those from disease-free controls and to results from other neuro-ophthalmic disease quality of life studies. RESULTS Among 29 participants with chronic migraine, vision-specific quality of life scores were all statistically significantly decreased compared to disease-free controls. The National Eye Institute Visual Function Questionnaire-25 median composite score was 85 for chronic migraineurs and 96 for controls (P

Previous evidence suggests optical treatments hold promise for treating migraine and photophobia. We designed an optical notch filter, centered at 480 nm to reduce direct stimulation of intrinsically photosensitive retinal ganglion cells. We used thin-film technology to integrate the filter into spectacle lenses. Our objective was to determine if an optical notch filter, designed to attenuate activity of intrinsically photosensitive retinal ganglion cells, could reduce headache impact in chronic migraine subjects. For this randomized, double-masked study, our primary endpoint was the Headache Impact Test (HIT-6; GlaxoSmithKline, Brentford, Middlesex, UK). We developed two filters: the therapeutic filter blocked visible light at 480 nm; a 620 nm filter was designed as a sham. Participants were asked to wear lenses with one of the filters for 2 weeks; after 2 weeks when no lenses were worn, they wore lenses with the other filter for 2 weeks. Of 48 subjects, 37 completed the study. Wearing either the 480 or 620 nm lenses resulted in clinically and statistically significant HIT-6 reductions. However, there was no significant difference when comparing overall effect of the 480 and 620 nm lenses. Although the 620 nm filter was designed as a sham intervention, research published following the trial indicated that melanopsin, the photopigment in intrinsically photosensitive retinal ganglion cells, is bi-stable. This molecular property may explain the unexpected efficacy of the 620 nm filter. These preliminary findings indicate that lenses outfitted with a thin-film optical notch filter may be useful in treating chronic migraine.

Purpose Orbital myositis is characterized by pain with eye movements, gaze restriction, diplopia, and enlargement of extraocular muscles on imaging. Varicella zoster virus (VZV) is an extremely rare cause of the disease in the elderly and has never been reported in a patient younger than forty-five years old such as the adolescent described herein. We present this case to raise awareness of an entity that will likely become more prevalent due to current vaccine strategies. Observation We present the case of a 13-year-old girl with VZV-associated orbital myositis and meningitis that had a quick and complete recovery following IV acyclovir and oral steroids. Conclusions and importance In conclusion, orbital myositis is an extremely rare complication of facial VZV infections. Our case highlights the importance of prompt detection and treatment in the pediatric population.

Benign essential blepharospasm (BEB) generally is considered a disorder of adults; however, it rarely can present in childhood or adolescence. The main purpose of this study was to determine the prevalence of BEB in children and adolescents. Our research question was whether blepharospasm is seen in children or adolescents as well as in the adult population. We conducted a retrospective chart review at the University of Utah and Johns Hopkins University. We reviewed our databases for diagnoses of blepharospasm and tic disorder over the past 10 years in patients of all ages. Charts then were reviewed to confirm the diagnosis, and a questionnaire was sent to subjects whose blepharospasm had apparently begun before age 20 years. We identified 26 patients diagnosed with eyelid spasms that had begun while under the age of 20. We confirmed BEB in four of these cases. Of these individuals, all had developed symptoms in adolescence or before and all were still symptomatic but had noted improvement in the severity and frequency of their symptoms. Although rare, BEB can develop in the first decade of life, producing symptoms and signs that are similar to adults, with persistence into adulthood.

Background We used in vivo corneal confocal microscopy to investigate structural differences in the sub-basal corneal nerve plexus in chronic migraine patients and a normal population. We used a validated questionnaire and tests of lacrimal function to determine the prevalence of dry eye in the same group of chronic migraine patients. Activation of the trigeminal system is involved in migraine. Corneal nociceptive sensation is mediated by trigeminal axons that synapse in the gasserian ganglion and the brainstem, and serve nociceptive, protective, and trophic functions. Noninvasive imaging of the corneal sub-basal nerve plexus is possible with in vivo corneal confocal microscopy. Methods For this case–control study, we recruited chronic migraine patients and compared them with a sex- and age-similar group of control subjects. Patients with peripheral neuropathy, a disease known to be associated with a peripheral neuropathy, or prior corneal or intraocular surgery were excluded. Participants underwent in vivo corneal confocal microscopy using a Heidelberg Retinal Tomography III confocal microscope with a Rostock Cornea Module. Nerve fiber length, nerve branch density, nerve fiber density, and tortuosity coefficient were measured using established methodologies. Migraine participants underwent testing of basal tear production with proparacaine, corneal sensitivity assessment with a cotton-tip applicator, measurement of tear break-up time, and completion of a validated dry eye questionnaire. Results A total of 19 chronic migraine patients and 30 control participants completed the study. There were no significant differences in age or sex. Nerve fiber density was significantly lower in migraine patients compared with controls (48.4 ± 23.5 vs 71.0 ± 15.0 fibers/mm2, P

Background Most patients with migraine report photophobia associated with headache; a subset report interictal photophobia. These patients are light sensitive even during headache-free periods. The objective of this case–control study was to assess the prevalence of symptoms of anxiety and depression in migraine patients with and without interictal photophobia. Methods We recruited 16 subjects with migraine and interictal photophobia, 16 age- and gender-matched migraine subjects without interictal photophobia, and 16 age- and gender- matched controls. Migraine subjects met International Headache Society classification criteria. Participants completed a photophobia questionnaire, Beck Depression Inventory (BDI-II), and Beck Anxiety Inventory (BAI). Chi-square analyses and two-tailed Wilcoxon rank sum tests were used for the analyses. Results Subjects with interictal photophobia had significantly higher scores on the photophobia questionnaire compared to subjects without interictal photophobia. Subjects with interictal photophobia had significantly higher scores on the BDI-II and BAI compared to subjects without interictal photophobia. Conclusions Migraine patients with interictal photophobia are more likely to manifest symptoms of depression and anxiety compared to migraine patients without interictal photophobia. Care providers should be aware of increased prevalence of these symptoms in this population and consider appropriate referrals. Future research could assess whether treatment of photophobia leads to improvements in symptoms of depression and anxiety in migraine patients.

Ultrasound is often used as an adjuvant in the workup of ocular pathology in children. It is particularly useful when ophthalmoscopic examination is limited, such as in the presence of extreme miosis or opaque ocular media. Other indications include assessment of a mass seen at ophthalmoscopy, elucidation of a mass underlying a retinal detachment, ocular trauma, and biometry. In this article, we review normal sonographic technique and ocular anatomy. The sonographic features of common pediatric ocular conditions are presented, with an emphasis on distinguishing features. Correlation, where pertinent, is made with other imaging modalities, such as computed tomography and magnetic resonance imaging. Ultrasound plays a critical role in the clinical diagnostic evaluation of globe pathology.

Infection, inflammation, and systemic diseases affecting the globe encompass a broad range of pathologies which may ultimately lead to progressive vision loss. Clinical symptomatology varies from the inexorably silent progressive visual loss to an acute presentation of ocular pain and/or red eye. Most are diagnosed by clinical ophthalmologic examination with selective use of ultrasound, computed tomography, and magnetic resonance imaging for confirmation of the diagnosis, assessment of disease extent, and signs of associated systemic disease. Knowledge of the differential diagnoses of vision loss, ocular pain, and redness makes imaging analysis of this diverse group of processes more precise.

Ocular cross-sectional imaging is usually obtained as an adjunct to clinical ophthalmologic examination and ocular ultrasound. Computed tomography/magnetic resonance imaging (CT/MRI) are complimentary for ocular imaging and are performed for evaluation of the vitreous cavity, choroid, retina, sclera, and potential spaces and for the assessment of extension of disease beyond the globe into the orbit or brain. CT has superior spatial resolution aided by the natural contrast between bone, soft tissues, air, and fat. The short scanning time is advantageous to reduce motion effects and the need for sedation. CT is also the modality of choice for evaluation of traumatic injury and for visualization of foreign bodies. Potential clinical indications for MRI include staging of retinoblastoma and other causes of leukocoria, assessment of retinal or choroidal detachments for underlying retinal mass or hemorrhage, uveal melanoma, ocular metastases, choroidal hemangioma, and buphthalmus, staphyloma, and coloboma. Last, but not least, MRI has the advantage of no ionizing radiation.

Interpretation of globe imaging after ophthalmologic surgical intervention, just like postoperative imaging in any location, can create a diagnostic dilemma if the radiologist is not familiar with the type of surgery performed and the nature and location of any implanted devices. Certain implants and devices may create artifacts on computed tomography or magnetic resonance imaging, and some contain ferromagnetic components that may be damaged if inadvertently placed in the magnetic resonance imaging. We review several common ocular surgical procedures, as well as a few orbital surgical procedures and discuss many implants and devices and their appearance on cross-sectional imaging. Familiarity with these procedures and their imaging appearance can diminish the chance of misinterpretation.

A 47-year-old healthy man presented with symptoms of double vision and drooping of the right upper eyelid. Examination disclosed signs of vertical diplopia and right upper-eyelid ptosis. An MRI delineated a well circumscribed, ring enhancing mass within the right orbit. The mass with peripheral enhancement located within the orbit, generated a broad differential diagnosis. After an excisional biopsy was performed, the mass was revealed to be fibrosis with extramedullary hematopoiesis. Current differential diagnoses of ring enhancing orbital lesions do not include extramedullary hematopoiesis, but we present a unique presentation of extramedullary hematopoiesis presenting as a ring enhancing orbital mass.

Ocular neoplasms, both primary and metastatic, may present with visual disturbance or vision loss and often are asymptomatic. Clinical ophthalmologic examination may demonstrate leukocoria, abnormal pupillary light reflex, or a mass lesion with or without retinal detachment or hemorrhage. Retinoblastoma in children and uveal melanoma and ocular metastases in adults are the most important ocular malignant neoplasms referred for imaging to aid with diagnosis and staging. Familiarity with their common imaging appearances, the common patterns of spread, and the diagnostic findings of greatest concern to the ocular oncologist will enhance accuracy of imaging interpretation. Clinical ophthalmologic examination and imaging using B-scan ultrasound, A-scan ultrasound, fluorescein angiography, computed tomography and magnetic resonance imaging have complementary roles in ocular tumor staging and treatment assessment.