To identify factors associated with plasma exchange response in neuromyelitis optica (NMO) spectrum disorders, the clinical and magnetic resonance imaging (MRI) features of 31 NMO-IgG-positive patients receiving plasma exchange for steroid-resistant exacerbations were analyzed. Functional improvement was observed in 65% of the patients. A lower baseline Expanded Disability Status Scale score was associated with favorable response (p = 0.040). Patients without cord atrophy had a higher success rate than patients with atrophy (p = 0.016). Levels of NMO-IgG did not differ between responders and non-responders before and after plasma exchange. In conclusion, a minimal pre-existing disability is the primary determinant of the effectiveness of plasma exchange.

Background and purposeThe loss of retinal ganglion cells observed in Alzheimer’s disease (AD) may be attributable to a neurodegeneration of the neuro-retinal structure. Amnestic mild cognitive impairment (aMCI) has been considered a prodromal stage of AD. We evaluated retinal thicknesses in patients with aMCI and AD compared to healthy controls using spectral-domain optical coherence tomography (OCT) to investigate whether changes in retinal thickness are correlated with the clinical severity of dementia.MethodsPatients with aMCI (n = 14), mild to moderate AD (n = 7), severe AD (n = 9), and age-matched controls (n = 17) underwent neuro-ophthalmologic examinations. Global deterioration scale (GDS), clinical dementia rating (CDR), and mini-mental status examination (MMSE) were used to evaluate the clinical overall severity of dementia. The thicknesses of the peripapillary retinal nerve fiber layer (RNFL), total macula, and macular ganglion cell-inner plexiform layer (GC-IPL) were measured using Cirrus HD-OCT.ResultsThe severe AD group had overall significantly thinner GC-IPL, total macula, and peripapillary RNFL compared to the controls (p<0.05). In the mild to moderate AD group, the total macula, average RNFL, and superior RNFL thickness were each significantly reduced compared to controls (p<0.05). The aMCI group had reduced total macula, average RNFL, and inferior RNFL thickness, but there were no significant differences compared to the controls. The GDS and CDR scores had a negative correlation with the thickness of the GC-IPL and the total macula. The MMSE scores had a positive correlation with both the total macular and average RNFL thickness, when adjusted for age (p<0.05).ConclusionsThis study confirmed that retinal thickness is decreased in AD patients. There is a correlation between reduced retinal thickness and the clinical severity of dementia.

PurposeTo report optical coherence tomography angiography (OCTA) of iris microhemangiomatosis.ObservationsA 75-year-old asymptomatic Caucasian man was found to have bilateral pupillary vascular lesions during cataract evaluation. Visual acuity was counting fingers in the right eye (OD) and 20/40 in the left eye (OS) with normal intraocular pressures in both eyes (OU). In each eye there were multifocal, round, dark red, pinpoint vascular tufts at the pupillary margin, randomly distributed and numbering 1 in OD and 7 in OS, each measuring 0.2–0.3 mm in diameter and without active bleeding or hyphema. Fundus examination OU was normal. By fluorescein angiography, the multifocal pupillary vascular tufts demonstrated mild staining without leakage. By OCTA, the tufts were clearly delineated and were fed by normal appearing radial iris vessels. OCT b-scan documented the optically dense vascular tufts at 0.1 mm in thickness and angio-overlay confirmed blood flow emanating from the deep iris stroma. Observation was recommended with the option of cataract surgery to improve vision.Conclusions and importanceNon-invasive imaging of iris microhemangiomatosis with OCTA delineates the vascular lesion with flow arising from the posterior iris stroma.