CME: Idiopathische intrakranielle Hypertension

Zusammenfassung. Die idiopathische intrakranielle Hypertension ist eine sekundäre Kopfschmerzerkrankung und Optikusneuropathie bei erhöhtem intrakraniellem Druck. Sie betrifft vorwiegend adipöse Frauen im gebärfähigen Alter und stellt sowohl diagnostisch als auch therapeutisch eine interdisziplinäre Herausforderung dar. Häufigstes Symptom dieser Erkrankung sind von Photo- und/oder Phonophobie begleitete Kopfschmerzen, die semiologisch oftmals einer Migräne sehr ähnlich sind, gefolgt von transienten visuellen Obskurationen und pulssynchronem Tinnitus. Während dem Erhalt von Visus und Gesichtsfeld die oberste Therapiepriorität zukommt, spielt auch die adäquate Behandlung der oftmals hartnäckigen und chronischen Kopfschmerzen eine zentrale Rolle. Ein konservatives Vorgehen mittels Gewichtsreduktion und medikamentöser Therapie ist in den meisten Fällen ausreichend. Im Falle fulminanter Verläufe oder relevanter Visuseinschränkung kommen zusätzlich interventionelle Verfahren zum Einsatz. Das Management der Kopfschmerzen richtet sich nach der vorherrschenden Semiologie. CME: Idiopathic Intracranial Hypertension Abstract. Idiopathic intracranial hypertension is a pressure-induced secondary headache disorder and optic neuropathy. It primarily affects obese women of childbearing age and poses an interdisciplinary challenge both diagnostically and therapeutically. The most common symptom of this disorder are headaches frequently accompanied by photo- and/or phonophobia, whose semiology often resembles that of migraine, followed by transient visual obscurations and pulsatile tinnitus. While protection of visual acuity and visual fields are of first therapeutical priority, adequate headache treatment also plays a key role. In the majority of cases, conservative treatment including weight loss and pharmacological therapy is sufficient. In case of a fulminant disease course or loss of visual function, interventional strategies can be applied additionally. Headache treatment is guided by the predominant semiology

Apraclonidine—An eye opener

Pharmacological testing with apraclonidine eye drops induces a typical reversal of anisocoria in patients with Horner’s syndrome. Moreover, apraclonidine was observed to have an elevating effect on the upper eyelid in Horner’s syndrome as well as in healthy subjects, which is thought to be mediated by alpha-1 adrenergic receptors present in the Muller’s muscle. We aim to quantitatively investigate the effect of apraclonidine on eyelid position in patients with Horner’s syndrome compared to physiological anisocoria based on infrared video recordings from pupillometry. We included 36 patients for analysis who underwent binocular pupillometry before and after apraclonidine 1% testing for the evaluation of anisocoria. Vertical eyelid measurements were taken from infrared videos and averaged from multiple pupillometry cycles. Receiver operating characteristic curves were calculated to determine the optimal cutoff value for change in eyelid aperture pre- and post-apraclonidine. A decrease of inter-eye difference in the aperture of >0.42 mm was discriminative of Horner’s syndrome compared to physiological anisocoria with a sensitivity of 80% and a specificity of 75%. Our data confirm an eyelid- elevating effect of the apraclonidine test, more pronounced in eyes with a sympathetic denervation deficit. Measuring eyelid aperture on pupillometry recordings may improve the diagnostic accuracy of apraclonidine testing in Horner’s syndrome

Multiple use of preservative-free single dose unit dexamethasone 0.1% eye drops is safe within 24 hours

Background Unpreserved single-dose unit (SDU) eye drops are commonly used to avoid benzalkonium chloride-related toxicity. Although intended for single use, many patients report off-label repeated use of SDUs over a prolonged period. We investigated whether repeated use of dexamethasone 0.1% SDUs in the same patient increases the bacterial contamination rate. Methods We prospectively enrolled patients scheduled for inpatient corneal and glaucoma surgery receiving dexamethasone 0.1% SDU four times per day from the same vial. To assess contamination rates, one drop from the vial was cultured immediately after opening the SDU (t0), 10 hours later after four drop applications (t10) and 24 hours after opening without further drop applications (t24). Conjunctival swabs were taken before and after drop application. Contamination rate was assessed with a standard clinical culturing protocol without introducing a positive control. Results 110 eyes of 109 patients were evaluated. Drops collected immediately after opening the SDU (t0) were contaminated in 9/110 cultures (8.1%). At t10, 13/110 cultures were contaminated (11.8%; p=0.267) and 11/110 at t24 (10.0%; t24 vs t0; p=1.00). In 5 of 21 cases of contaminated drops at t10 and/or t24, the same isolates were cultured from the initial conjunctival swab and the SDU. In three cases, the same bacterial species was found in consecutive samples. Conclusion The contamination rate of the SDU did not increase after multiple use within 24 hours. Contamination from fingertip flora was more likely than from ocular surface flora. Reuse of dexamethasone 0.1% SDU in the same patient within 24 hours appears to be safe

Long-Term Follow-Up in Children with Anisocoria: Cocaine Test Results and Patient Outcome

Background Evaluation of anisocoria including pharmacological testing for Horner's syndrome in the pediatric population is challenging in view of potential serious underlying disease. We describe cocaine test results, outcome of systemic investigation, and long-term follow-up in children with anisocoria. Methods Retrospective review of medical records and phone interview of consecutive pediatric patients (<18 years old) who underwent cocaine testing from August 2007 to July 2015 at a tertiary referral centre. Results A total of 35 patients were included with a positive, negative, or inconclusive cocaine test in 12/35, 19/35, and 4/35, respectively. Systemic investigation was performed in 11 of the patients with a positive and in 2 of the patients with an inconclusive cocaine test result. Mediastinal Hodgkin lymphoma was found in one patient with an inconclusive cocaine test result. Two other cases were presumably related to birth trauma and surgical trauma. None of the other children further developed any pathology during the follow-up period of 34.8 months (range 0-106.6). Conclusions In most children with anisocoria and a positive cocaine test result, systemic investigation did not reveal any underlying etiology. The only malignant disease was diagnosed in a patient with a suspicion of Horner's syndrome but with an inconclusive cocaine test result in our cohort

Diagnosing Myasthenia Gravis With Repetitive Ocular Vestibular Evoked Myogenic Potentials

Timely and accurate diagnosis of myasthenia gravis, particularly in patients with fluctuating, isolated ocular involvement, remains challenging. Serological antibody testing and repetitive nerve stimulation of peripheral muscles usually have low sensitivity in these patients. Edrophonium testing may cause adverse events, single-fiber electromyography (SFEMG) is time-consuming and both tests are often unavailable outside specialized institutions. Repetitive ocular vestibular evoked myogenic potential (roVEMP) stimulation has recently been introduced to facilitate the diagnosis of myasthenia gravis. Similar to repetitive nerve stimulation, roVEMPs detect muscle decrements with the benefit of being non-invasive and allowing for direct measurement of the extraocular muscles. This review summarizes the clinical evidence of the diagnostic value of roVEMP for myasthenia. Prospective clinical trials have demonstrated high sensitivity and specificity. RoVEMPs are of particular interest in challenging myasthenia subgroups with isolated ocular involvement, negative serology, and/or negative conventional electrophysiological results. Optimal roVEMP repetition rates of 20-30 Hz have been identified. This promising novel diagnostic tool merits further attention and investigation to establish its value as a clinical test for myasthenia

Treatment of Esophageal Variceal Hemorrhage with Self-Expanding Metal Stents as a Rescue Maneuver in a Swiss Multicentric Cohort

Acute esophageal variceal bleeding in patients with portal hypertension remains a complication with a high mortality today. In cases refractory to standard therapy including endoscopic band ligation and pharmacological therapy, traditionally balloon tamponade has been used as salvage therapy. However, these techniques show several important limitations. Self-expanding metal stents (SEMS) have been proposed as an alternative rescue treatment. The use of variceal stenting in 7 patients with a total of 9 bleeding episodes in three different Swiss hospitals is demonstrated. While immediate bleeding control is achieved in a high percentage of cases, the 5-day and 6-week mortality rate remain high. Mortality is strongly influenced by the severity of the underlying liver disease. Accordingly, our data represent a high-risk patient collective. Thanks to their safety and easy handling, SEMS are an interesting alternative to balloon tamponade as a bridging intervention to definitive therapy including the pre-hospital setting

Convergence on VOR Gain: Normal and Abnormal

Vestibular compensatory eye movements provide a mechanism to maintain steady fixation on targets during head movement. The anatomic pathways that mediate a normal horizontal vestibulo-ocular reflex (h-VOR), when lesioned, cause spontaneous nystagmus. While the effect of convergence on spontaneous nystagmus has been reported in various populations, the response to adaptation to near targets in patients with peripheral or central vestibular lesions has not been studied. We aim to clarify potential vestibular and near-reflex interactions by comparing the near and far h-VOR in normal volunteers to patients with unilateral peripheral and central vestibular lesions

Using Smartphone Exophthalmometry to Measure Eyeball Protrusion

ImportanceThe current clinical criterion standard for measuring abnormal eyeball protrusion is still the historic Hertel exophthalmometer, which is prone to reading errors. Therefore, a smartphone application has been developed to measure exophthalmos.ObjectiveTo evaluate a relatively simple noninvasive measurement method for exophthalmos using a smartphone.Design, Setting, and ParticipantsThis cross-sectional study compared smartphone exophthalmometry with the existing reference standard, the Hertel exophthalmometer, or a professional high-resolution 3-dimensional scanner. Participants were patients with exophthalmos due to Graves orbitopathy and other intraorbital conditions and healthy volunteers who were recruited between June 2019 and January 2022 from the Department of Ophthalmology, University Hospital Zurich.InterventionsAll participants were examined twice by 3 different operators using 3 different methods (smartphone, high-resolution scanner, or Hertel exophthalmometer) at an interval of a minimum of 2 weeks or after exophthalmos-changing treatment.Main Outcome MeasuresAccuracy and precision, test-retest reliability, and interoperator reliability of eyeball protrusion measurements obtained with the smartphone compared to the Hertel exophthalmometer and the high-resolution face scanner.ResultsOf 39 participants, 23 patients (median [IQR] age, 54 [44-59] years; 15 [65%] female and 8 [35%] male) showed a mean difference in eyeball protrusion of 3.3 mm and 16 healthy volunteers (median [IQR] age, 32 [30-37] years; 11 [69%] female and 5 [31%] male) of 0.8 mm without any significant difference between the 3 methods. Accuracy and precision agreement between exophthalmos measures with the smartphone and the Hertel exophthalmometer showed an intraclass correlation coefficient (ICC) of 0.89 (95% CI, 0.80-0.94) and 0.93 (95% CI, 0.83-0.97) for the high-resolution scanner. Interoperator agreement was highest for the high-resolution scanner (ICC, 0.99 [95% CI, 0.98-0.99]), followed by the smartphone (ICC, 0.95 [95% CI, 0.92-0.97]) and the Hertel exophthalmometer (ICC, 0.91 [95% CI, 0.85-0.95]). Test retest reliability was similarly high for the smartphone (ICC, 0.93 [95% CI, 0.82-0.95]), the Hertel exophthalmometer (ICC, 0.92 [95% CI, 0.83-0.96]), and the high-resolution scanner (ICC, 0.95 [95% CI, 0.89-0.97]).Conclusions and RelevanceThe findings of this study demonstrate relatively high accuracy and precision, interoperator reliability, and test-retest reliability for smartphone exophthalmometry. These data support the use of a smartphone in place of a Hertel exophthalmometer for measuring exophthalmos in the future

Widespread White Matter Alterations in Patients With Visual Snow Syndrome

Background: Visual snow is considered a disorder of central visual processing resulting in a perturbed perception of constant binocular flickering or pixilation of the whole visual field. The underlying neurophysiological and structural alterations remain elusive. Methods: In this study, we included patients (final n = 14, five dropouts; five females, mean age: 32 years) with visual snow syndrome (VSS) and age- and sex-matched controls (final n = 20, 6 dropouts, 13 females, mean age: 28.2 years). We applied diffusion tensor imaging to examine possible white matter (WM) alterations in patients with VSS. Results: The patient group demonstrated higher (p-corrected < 0.05, adjusted for age and sex) fractional anisotropy (FA) and lower mean diffusivity (MD) and radial diffusivity (RD) compared to controls. These changes were seen in the prefrontal WM (including the inferior fronto-occipital fascicle), temporal and occipital WM, superior and middle longitudinal fascicle, and sagittal stratum. When additionally corrected for migraine or tinnitus-dominant comorbidities in VSS-similar group differences were seen for FA and RD, but less pronounced. Conclusions: Our results indicate that patients with VSS present WM alterations in parts of the visual cortex and outside the visual cortex. As parts of the inferior fronto-occipital fascicle and sagittal stratum are associated with visual processing and visual conceptualisation, our results suggest that the WM alterations in these regions may indicate atypical visual processing in patients with VSS. Yet, the frequent presence of migraine and other comorbidities such as tinnitus in VSS makes it difficult to attribute WM disruptions solely to VSS

The "Eyelet Sign" as an MRI Clue for Inflammatory Brown Syndrome

BACKGROUND Brown syndrome is characterized by a restrictive elevation deficit of the affected eye in adduction. Besides the well-known congenital form, different acquired etiologies including inflammation, trauma, and surgery may prevent the superior oblique (SO) tendon from gliding freely through the trochlea on attempted upgaze. We present MRI findings in pediatric and adult patients with inflammatory acquired Brown syndrome. METHODS Retrospective review of clinical and MRI findings of 6 patients (4 children: median age 8.4 years [range 6.1-8.7]; 2 adults: age 46.4 and 51.1 years). Median follow-up was 23 months (range 1-52). RESULTS In all 6 patients, orbital MRI demonstrated inflammatory changes of the SO tendon-trochlea complex. A striking feature was circumferential contrast enhancement of the trochlea with central sparing where the tendon passes, reminiscent of an eyelet. In all cases, the motility restriction improved either spontaneously or with systemic anti-inflammatory treatment. Although both adult patients had a history of known seronegative spondyloarthritis, there was no associated systemic condition in the children in our series. CONCLUSIONS Both in children and in adults, MRI can provide evidence of inflammatory changes located at the trochlea-tendon complex in acquired Brown syndrome here referred to as the "eyelet sign," which may be helpful in confirming the clinical diagnosis and guide appropriate treatment