En kvinne i 60-årene som ikke lenger gjenkjente det hun så.

BACKGROUND: Neurological disorders can present with a vast array of visual disturbances. The constellation of symptoms and findings in this patient prompted workup for unusual causes of both stroke and neurodegenerative disorder. CASE PRESENTATION: A woman in her sixties presented with visual disturbances, followed by weakness in her right arm and aphasia three days later. Her close acquaintances had suspected progressive cognitive decline during the previous year. CT and MRI showed an occluded left posterior cerebral artery with a subacute occipito-temporal infarction. The finding of extensive white matter lesions and segmental arterial vasoconstriction necessitated further workup of vasculitis and hereditary small vessel disease, which were ruled out. The stroke aetiology was considered to be atherosclerotic intracranial large vessel disease. FDG-PET scan revealed decreased metabolism in the left hemisphere, and cerebrospinal biomarkers had slightly decreased beta-amyloid. The findings were suggestive of early Alzheimer's disease or primary progressive aphasia, but currently inconclusive. INTERPRETATION: Based on clinical-anatomical correlation, the patient's visual disturbances, in this case right hemianopsia and object agnosia, were solely related to the stroke and not to a neurodegenerative disorder. Knowledge and interpretation of visual agnosias can in many cases be clinically valuable.

The Value of Macular Optical Coherence Tomography in Watchful Waiting of Suprasellar Masses: A 2-Year Observational Study.

BACKGROUND: A possible benefit of optical coherence tomography (OCT) in the approach to tumors involving the optic chiasm may be the ability to foresee visual deterioration. This study investigated the value of OCT in watchful waiting for compressive optic neuropathy as the primary management of suprasellar masses. METHODS: The research was conducted as a 2-year observational study of a patient cohort with conservatively managed mass lesions involving the optic chiasm on MRI. Threshold perimetry and macular OCT were performed at baseline and each follow-up examination. Univariate Cox regression was used to determine the effect of baseline and longitudinal covariates upon development of visual field (VF) loss compatible with chiasmal dysfunction. RESULTS: Nineteen eyes of 19 patients were included. The optic chiasm-tumor relationship on baseline MRI was abutment in 6 cases and compression in 13 cases. Seven eyes developed VF loss. None of the baseline covariates were predictors of VF loss. The longitudinal decrease in mean macular ganglion cell complex (mGCC) thickness on OCT was 2.5 µm/yr for eyes that developed VF loss and 0.2 µm/yr for eyes that did not develop VF loss (P = 0.02). The hazard ratio for VF loss per 1-µm/yr decrease in mGCC thickness was 1.30 (95% confidence interval [CI] 1.04-1.62; P = 0.02) for the inferior nasal quadrant and 1.45 (95% CI 1.02-2.07; P = 0.04) for the inferior temporal quadrant. CONCLUSIONS: OCT offers a valuable complement to perimetry in monitoring for compressive optic neuropathy. Longitudinal mGCC thinning can anticipate VF loss.

Transient smartphone blindness. / Transitorisk smarttelefonblindhet.

BACKGROUND: The aetiology of transient monocular vision loss is not necessarily thromboembolic, and careful history-taking is crucial in making the correct diagnosis. CASE PRESENTATION: A previously healthy woman in her thirties was referred to our outpatient stroke clinic on suspicion of recurring amaurosis fugax in her right eye. The ophthalmic and neurologic examinations were normal. A review of the medical history revealed that her symptoms occurred in connection with using her smartphone in bed, typically while lying on her left side with the left eye covered by the pillow. When standing up from the bed again with the lights turned off in the bedroom, she noticed vision loss in her right eye. INTERPRETATION: We concluded that the patient had experienced transient smartphone blindness. This phenomenon can occur when monocularly using a smartphone under scotopic lighting conditions; only the occluded eye adapts to darkness, and the other eye is perceived to be blind if looking away from the bright smartphone screen.

A woman in her forties with unilateral blurred vision and pain upon eye movement. / En kvinne i 40-årene med ensidig tåkesyn og smerter ved øyebevegelse.

BACKGROUND: Toxoplasmosis is a relatively common cause of infectious papillitis and neuroretinitis, which may affect both immunocompetent and immunodeficient patients. CASE PRESENTATION: A previously healthy woman in her mid-forties presented with subacute onset of unilateral blurred vision and retrobulbar pain exacerbated by eye movements. Ophthalmological assessment revealed decimal best-corrected visual acuity of 0.75, an ipsilateral swollen disc and a peripapillary infiltrate. Serology indicated acute infection with Toxoplasma gondii. Cerebral MRI showed a periventricular lesion, and oligoclonal bands were detected in the cerebrospinal fluid. INTERPRETATION: This case illustrates that even when symptoms, MRI and cerebrospinal fluid findings suggest demyelinating disease, differential diagnoses must be considered in order to mitigate the negative therapeutic and prognostic consequences of a misdiagnosis.

Off-label use of bevacizumab for wet age-related macular degeneration in Europe.

PURPOSE: To analyse current off-label use of bevacizumab for wet age-related macular degeneration (AMD) in Europe. METHODS: The study was conducted as a combined survey and literature review. It included the 22 most populous countries in Europe. In each country, ophthalmologists with particular knowledge about off-label treatment responded to a questionnaire. RESULTS: Answers were obtained from twenty European countries. The off-label use of bevacizumab for wet AMD greatly differed between nations; the bevacizumab proportion varied from non-existent (0%) to very high (97%). There were also large disparities within single countries (e.g. 0-80%), which were attributable to differences in regional decision-making. Both governmental institutions and national ophthalmological societies expressed highly diverging opinions on the use of off-label treatment. Intravitreal administration of bevacizumab had been a matter of legal dispute in several countries. The question about responsibility for off-label therapy mainly remained unanswered. CONCLUSIONS: There was a highly varying utilization of bevacizumab between European countries. Despite an intention of a consistent approach to medical regulations, Europe has not yet reached a professional or political consensus on the ophthalmic off-label use of bevacizumab.

Anterior Ischemic Optic Neuropathy Due to Calciphylaxis.

A 72-year-old woman experienced anterior ischemic optic neuropathy in her left eye. The funduscopic and fluorescein angiographic findings were strongly suggestive of giant cell arteritis. Temporal artery biopsy revealed extensive calcification in the vessel wall consistent with calciphylaxis. This unusual disorder should be considered in the differential diagnosis of anterior ischemic optic neuropathy, particularly the arteritic form.

A teenager with acute bilateral visual loss. / En tenåring med akutt bilateralt synstap.

BACKGROUND: The diagnosis of acute optic neuropathy is made clinically. In young patients demyelinating optic neuritis is the most common cause. However, other autoimmune diseases, infections and other non-inflammatory conditions may also cause inflammation. Careful clinical workup is necessary to establish the correct diagnosis and treatment. We describe the clinical approach to a case of acute optic neuropathy with several atypical features. The same case was published in the Journal of Neuro-Ophthalmology. CASE PRESENTATION: A male teenager developed acute and painless bilateral visual loss. Fundoscopy revealed optic disc hypaeremia with telangiectasia. Magnetic resonance imaging demonstrated contrast enhancement of the optic nerves and chiasm without evidence of demyelinating disease. There was no visual improvement after methylprednisolone treatment. Genetic analysis for the 3 common Leber hereditary optic neuropathy (LHON) mutations was negative. However, idebenone treatment was followed by a marked improvement in visual function. Whole mitochondrial genome sequencing eventually detected a rare LHON mutation. INTERPRETATION: There are many different causes of acute optic neuropathy. Making the correct diagnosis is important, as clinical management differs. Idebenone is now a treatment option for LHON. Whole mitochondrial genome sequencing is sometimes necessary to confirm the diagnosis.

The first Norwegian doctorate in multiple sclerosis. / Den første norske doktorgraden om multippel sklerose.

The story of research on multiple sclerosis in Norway starts in 1945 at a private Catholic hospital in the town of Hamar, where the ophthalmologist Marius Haarr was treating patients with retinal periphlebitis and neurological symptoms. He completed his doctoral degree while raising two young children as a widowed father.

A health economic pilot study comparing two diabetic retinopathy screening strategies.

To compare two screening strategies for diabetic retinopathy (DR), and to determine the health-economic impact of including optical coherence tomography (OCT) in a regular DR screening. This cross-sectional study included a cohort of patients (≥ 18 years) with type 1 or 2 diabetes mellitus (T1D or T2D) from a pilot DR screening program at Oslo University Hospital, Norway. A combined screening strategy where OCT was performed in addition to fundus photography for all patients, was conducted on this cohort and compared to our existing sequential screening strategy. In the sequential screening strategy, OCT was performed on a separate day only if fundus photography indicated diabetic macular edema (DME). The presence of diabetic maculopathy on fundus photography and DME on OCT was determined by two medical retina specialists. Based on the prevalence rate of diabetic maculopathy and DME from the pilot, we determined the health-economic impact of the two screening strategies. The study included 180 eyes of 90 patients. Twenty-seven eyes of 18 patients had diabetic maculopathy, and of these, 7 eyes of 6 patients revealed DME on OCT. When diabetic maculopathy was absent on fundus photographs, OCT could not reveal DME. Accordingly, 18 patients (20%) with diabetic maculopathy would have needed an additional examination with OCT in the sequential screening strategy, 6 (33%) of whom would have had DME on OCT. In an extended healthcare perspective analysis, the cost of the sequential screening strategy was higher than the cost of the combined screening strategy. There was a weak association between diabetic maculopathy on fundus photography and DME on OCT. The health economic analysis suggests that including OCT as a standard test in DR screening could potentially be cost-saving.

Primary vitrectomy or intravitreal antibiotics followed by early vitrectomy for acute endophthalmitis: A prospective observational study.

BACKGROUND/AIMS: To compare outcomes of acute endophthalmitis (EO) managed with either primary vitrectomy (PV) or primary intravitreal antibiotics (vancomycin and ceftazidime) followed by early vitrectomy (PIAEV) combined with polymerase chain reaction (PCR)-based diagnostics. METHODS: This was a prospective, comparative observational study of acute EO cases admitted to a regional vitreoretinal service over 18 months. Depending on whether immediate vitrectomy (within 6 h) was achievable, the EO cases were treated with either (1) PV or (2) PIAEV. Microbiology samples were collected either (A) before or (B) after administration of intravitreal antibiotics. The samples were analysed with broad-range 16S PCR and culture. RESULTS: The study included 41 EO cases. There were 19 post-injection EO, 18 post-cataract EO, three post-vitrectomy EO, and one blebitis-related EO. Fifteen of 19 PV cases and 15 of 21 PIAEV had a clinically meaningful improvement in best-corrected visual acuity (BCVA) of at least 15 letters at 3 months (p = 0.58). One patient was lost to follow-up. Twenty-three cases were culture- and PCR-positive, and seven additional cases were culture-negative but PCR-positive (p = 0.02). PCR increased the diagnostic yield for samples collected both before and after administration of intravitreal antibiotics. CONCLUSION: Primary vitrectomy or PIAEV allowed for vitrectomy for all cases of acute EO in a large region. Most eyes in both groups achieved a clinically meaningful improvement in BCVA. By combining culture with PCR in connection with the vitrectomy procedure, intravitreal antibiotics could be injected before microbiological sampling, thereby improving the door-to-treatment time without sacrificing microbial identification.