Cerebroretinal microangiopathy with calcifications and cysts (CRMCC).

Extensive intracranial calcifications and leukoencephalopathy are seen in both Coats plus and leukoencephalopathy with calcifications and cysts (LCC; Labrune syndrome). Coats plus syndrome is additionally characterized by the presence of bilateral retinal telangiectasia and exudates while LCC shows the progressive formation of parenchymal brain cysts. Despite these apparently distinguishing features, recent evidence suggests that Coats plus and LCC represent the same clinical entity with a common primary pathogenesis involving a small vessel obliterative microangiopathy. Here, we describe eight previously unreported cases, and present an update on one of the original Coats plus patients to highlight the emerging core clinical features of the "cerebroretinal microangiopathy with calcification and cysts" (CRMCC) phenotype.

Degree, duration, and causes of visual loss in uveitis.

BACKGROUND/AIMS: Uveitis is a major cause of visual morbidity in the working age group. The authors investigated the duration, degree, and causes of visual loss in uveitis patients with the aim of better defining the visual morbidity and identifying potential risk factors. METHODS: A retrospective, non-interventional, observational survey of 315 consecutive patients attending a tertiary referral uveitis service. RESULTS: The mean duration of follow up was 36.7 months. Reduced vision (< or =6/18) was found in 220/315 (69.95%) of the patients with a subset of 120 patients having vision < or =6/60. Unilateral visual loss occurred in 109 (49.54%), while 111 (50.45%) had bilateral loss. The mean duration of visual loss was 21 months. Of the 148 patients with pan-uveitis, 125 (84.45%) had reduced vision, with 66 (53%) having vision < or =6/60. Main causes of visual loss were cystoid macular oedema (CMO) (59/220, 26.8%), cataract (39/220, 17.7%), and combination of CMO and cataract (44/220, 20%). The following were predictive of a poorer visual prognosis: pan-uveitis (p = 0.0005), bilateral inflammation (p = 0.0005), increasing duration of reduced vision (p = 0.0005), an Indian or Pakistani ethnic background (p = 0.004), and increasing patient age (p = 0.02). CONCLUSION: Prolonged visual loss occurred in two thirds of uveitis patients, with 70 (22%) patients meeting the criteria for legal blindness at some point in their follow up. Older patients with bilateral inflammation and an increasing duration of reduced vision are at the greatest risk of severe visual loss (< or =6/60). CMO and cataract were responsible for visual loss in 64.5% of patients.

Deep intramuscular methylprednisolone for the treatment of cystoid macular oedema in uveitis.

PURPOSE: To study the use of intramuscular methylprednisolone in the treatment of cystoid macular oedema in patients with uveitis. METHODS: A total of 32 patients with various types of uveitis with unilateral cystoid macular oedema were recruited. Patients received 160 mg of intramuscular methylprednisolone into the thigh and were reassessed 6-8 weeks later. In 17 patients in whom there had been no significant improvement, a further injection of methylprednisolone was given; a total of 49 injections. RESULTS: After a first injection a significant improvement in vision (an increase of > or = 2 Snellen lines) was seen in 15 of 32 eyes (47%), and 17 of 32 eyes (53%) showed no significant change. After the second injection 7 of 17 eyes (41%) showed a significant improvement but there was no change in 9 of 17 eyes (53%). Only one eye deteriorated more than 2 lines of Snellen acuity. There were minimal ocular or systemic side effects associated with this form of treatment. CONCLUSIONS: Despite limited success, deep intramuscular methylprednisolone may have a role as an alternative mode of treatment in uveitis patients with unilateral cystoid macular oedema, thus avoiding the potential hazards of periocular injections. In comparison with orally administered systemic corticosteroids, an injection ensures patient compliance and is associated with fewer side effects.