Increased incidence of Susac syndrome: a case series study.

BACKGROUND: Susac syndrome (SuS) is a rare condition characterized by a clinical triad of sensorineural hearing loss, branch artery occlusion and encephalopathy. This study reports an increased incidence of SuS in Israel. We describe the clinical characteristics of these patients, diagnostic procedures and the use and subsequent outcomes of newly published treatment guidelines. METHODS: This is a single center retrospective study. Patients who were diagnosed with SuS between July 2017 and August 2018 were enrolled in this study. RESULTS: Seven patients were diagnosed with SuS according to the diagnostic criteria in a time period of 13 months. The annual incidence was recently evaluated in Austria to be 0.024/100000, therefore, our case series represent at least a 5.4- fold increase in the annual incidence of SuS expected in Israel and a 7-fold increase in the annual incidence expected in our medical center. Mean time from the onset of the symptoms to diagnosis was three weeks and follow-up time was twenty four months. Recent exposure to cytomegalovirus was serologically evident in three patients and one patient had high titer of anti-streptolysin antibody. All patients underwent brain MRI, fluorescein angiography and audiometry. All patients were treated according to the newly recommended guidelines. All patients achieved clinical and radiological stability. CONCLUSIONS: We report of an increased incidence of SuS in Israel. Infectious serological findings may imply a post infectious mechanism. The use of the recommended diagnostic procedures reduced the time to diagnosis. Newly published treatment guidelines led to favorable clinical outcomes.

Macular findings on optical coherence tomography in cat-scratch disease neuroretinitis.

PURPOSE: To describe the macular findings on optical coherence tomography (OCT) in patients with cat-scratch disease (CSD) neuroretinitis. METHODS: Medical records of all patients diagnosed with CSD neuroretinitis at the Tel Aviv Medical Center between April 2006 and May 2010 were retrospectively reviewed. All patients underwent Stratus OCT macular examination. RESULTS: Eight eyes of seven patients with confirmed CSD neuroretinitis, (mean age 33 ± 9.9 years, range 6-48 years) were included in the study. All patients presented clinically with optic nerve swelling and macular edema or macular exudates. OCT demonstrated flattening of the foveal contour, thickening of the neurosensory retina, and accumulation of subretinal fluid (SRF) in all studied eyes. Retinal exudates appeared as multiple hyper-reflective foci in the outer plexiform layer. The average central macular thickness was 460 µm (range 170-906 µm) and the average maximal retinal thickness was 613 µm (range 387-1103 µm), at presentation. The macula appeared normal on repeated exams during follow-up. CONCLUSION: Similar OCT findings were demonstrated in patients with CSD neuroretinitis. SRF was found in all eyes, although was not visible on clinical examination or fluorescein angiography. OCT may be used as an adjunct imaging tool in the diagnosis and follow-up of patients with CSD neuroretinitis.

[Therapeutic penetrating keratoplasty for microbial keratitis].

BACKGROUND: Microbial keratitis is a potentially sight threatening disease. Most cases respond well to antimicrobial therapy. However, in cases that progress despite intensive medical therapy, an urgent therapeutic penetrating keratoplasty (TPKP) is required. AIM: To evaluate the indications and results of TPKP in Israel. METHODS: A retrospective study reviewed the TPKP performed at the Goldschleger Eye Institute, Sheba Medical Center, between 1990-2003. The study included 18 cases of at least one-year follow-up. RESULTS: The indications for TPKP included severe infectious keratitis unresponsive to medical treatment in 33% of the patients and severe corneal destruction in 66% of them. The infectious keratitis was diagnosed as bacterial keratitis in 44% of the patients, unidentified pathogen in 39%, mycotic in 11% and acanthamoeba in 6% of the patients. Risk factors in the patients with microbial keratitis requiring TPKP included: previous ocular disease in 39%, previous ocular surgery in 66%, systemic disorders in 28% and ocular risk factors in 28% patients. TPKP was successful in bacterial and acanthamoeba keratitis as far as the transparency of the graft and elimination of the infection and improvement of visual acuity. However, TPKP failed in mycotic and unidentified keratitis. The risk factors for failure included: previous ocular disease or surgeries, systemic disorders or large corneal grafts. CONCLUSIONS: Therapeutic penetrating keratoplasty is an important and effective therapeutic tool for intractable bacterial and acanthamoeba keratitis. Prognostic factors for graft success include lack of ocular disease or previous surgeries, lack of systemic disorders or small corneal graft size.