The ODDS Consortium recommendations are important in the process of establishing a reliable and consistent diagnosis of ODD using OCT for both clinicians and researchers.
Idiopathic intracranial hypertension (IIH) primarily affects young obese females, and potentially causes visual loss and severe headache. The aim of this experiment is to examine relapse rate and long-term outcome in IIH patients. The methods involved in this experiment include a prospective controlled study of 18 newly diagnosed IIH patients followed for a mean observation period of 21.1 (±8.0) months. Treatment regime included diuretics, dietary recommendations and check-up visits at a dietician. Baseline and follow-up included neurological examination, detailed headache history and comprehensive neuro-ophthalmological examination, including fundus photography, Humphrey visual fields, and measurement of the retinal thickness (RT) and retinal nerve fiber layers (RNFL) by optical coherence tomography (OCT). Relapse was defined as recurrence of either: (1) papilledema or (2) symptoms and demonstrated raised ICP. The result of this experiment is that relapse was found in 28%. Visual function improved from baseline to follow-up and was generally favorable. In patients without relapse of papilledema RT and RNFL were significantly thinner than in healthy controls (p = 0.003 and 0.02), although atrophy was clinically detectable in only one patient. Headache was still present in 67% of the patients at follow-up. Headache was heterogenic and unrelated to relapse. After an initial reduction, weight increased again in the relapse group compared to reduced weight in the non-relapse group (p = 0.013). Thus, the conclusions drawn are that headache was persistent, difficult to classify, and equally represented in relapse and non-relapse patients. Headache was thus a poor marker of active disease. Relapse rate was high and clinically undetectable optic disc atrophy was discovered in apparently well treated IIH patients.
Although headache in 43% of patients responded well to ICP management, sustained long-term headache was seen in the remaining patients, despite resolution of papilledema. Headache in IIH may thus be attributed to more complex mechanisms than ICP elevation alone. High ICP and young age were associated with better headache outcome. Early treatment according to standard guidelines seems sufficient to ensure excellent visual outcome in the vast majority of patients.
Peripapillary RNFL thickness correlates with anatomic location (superficial or buried) of ODD. Frequency and extent of visual field defects corresponded with anatomic location of ODD and peripapillary RNFL thickness, suggesting increased axonal damage in patients with superficial ODD.
Peripapillary OCT is a promising objective examination modality for optic disc evaluation in IIH, and may improve the identification of subtle disc swellings. Underestimation of RNFLT for increasing severities of papilledemas is suspected, making PART a more reliable parameter compared to RNFLT. Future larger validation studies are needed.
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Purpose: This study set out to investigate retinal nerve fibre layer (RNFL) thickness and best corrected visual acuity (BCVA) in relation to age in healthy subjects and patients with OPA1 autosomal dominant optic atrophy (DOA).
Methods: We carried out a cross‐sectional investigation of RNFL thickness and ganglion cell layer density in 30 healthy subjects and 10 patients with OPA1 DOA using optical coherence tomography (OCT). We then performed a regression analysis of RNFL thickness and BCVA versus age.
Results: Both healthy subjects and DOA patients demonstrated a gradual reduction in RNFL thickness with age; the relationship was best described statistically by a model that assumed a constant offset between the two groups. Best corrected VA decreased significantly with age in DOA patients, in whom BCVA was correlated with peripapillary RNFL thickness in the inferior and superior peripapillary quadrants and with total macular thickness at eccentricities of 500–3000 μm. The observations were best described by a constant offset of 41.9 μm separating the two groups and an annual decrease in RNFL thickness of 0.48 μm (p < 0.0001). In patients with DOA, increasing age was associated with decreasing BCVA (p = 0.046).
Conclusions: This cross‐sectional study found evidence of comparable age‐related decreases in RNFL thickness in healthy subjects and in DOA patients, where the deficit in DOA patients is best described using a model that assumes the deficit between the groups does not vary with age. The gradual reduction of BCVA with age may be a consequence of a relative deficit in RNFL thickness that is established before the second decade of life.
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