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Purpose A hospital-based epidemiology study to describe herpes zoster ophthalmicus (HZO) prevalence, and identify risk factors for recurrent and chronic disease. Design Retrospective, hospital-based cohort study Participants All patients evaluated in the Broward and Miami VA Healthcare System (MIAVHS) during the study period. Methods Retrospective medical record review of patients seen in the MIAVHS from January 1, 2010 and December 31, 2014 with a HZO clinical diagnosis. Assessment of the patient's clinical course was defined by the following: an acute episode of HZO was defined as quiescence of disease within 90 days of initial presentation; HZO recurrence was defined as any recurrent eye disease or rash >90 days after quiescence disease was noted off therapy; chronic HZO was defined as active disease persisting greater than 90 days from initial presentation. Main Outcome Measures 1) Frequency of HZ involving the V1 dermatome (HZO) with and without eye involvement; 2) HZO recurrence rates 3) Risk factors for recurrent or chronic HZO. Results 90 patients with HZO were included in the study. The mean age at incident episode of HZO was 68±13.8 years (range, 27-95 years). The majority of patients were white (73%), immune competent (79%), and did not receive zoster vaccination at any time point in their follow up (82%). Patients were followed for a mean of 3.9±5.9 years, (range, 0-33 years). The period prevalence of HZ in any dermatome was 1.1%, the frequency of HZ involving V1 (HZO) was 0.07% and the frequency of HZO with eye involvement was 0.05%. The overall 1, 3, and 5-year recurrence rates for either recurrent eye disease or rash were 8%, 17%, 25%, respectively. Ocular hypertension (HR 4.6, 95% Cl 1.3-16.5; OR 6.7, 95% Cl 1.5-31.2) and uveitis (HR 5.7, 95%CI 1.7-19.0; OR 6.7, 95% C1 1.5-31.2) increased the risk of recurrent and chronic disease. Conclusion This study supports newer data that a significant proportion of patients experience recurrent and chronic HZO. Further study is needed to guide preventative and therapeutic approaches to recurrent and chronic HZO.

Autosomal recessive intellectual disability (ID) is characterized by extensive genetic heterogeneity. Recently, three mutations in SZT2 were reported in two unrelated children with unexplained infantile epileptic encephalopathy with severe ID. Here we report a European American family with three children having non-syndromic mild or moderate ID without seizures. Whole-exome sequencing of three affected siblings revealed a three base pair deletion (c.4202_4204delTTC) located in a 19 mb autozygous region on chromosome 1, leading to an amino acid deletion (p.Phe1401del) in SZT2. All three children were homozygous for the deletion and their parents were heterozygous as expected in autosomal recessive inheritance. SZT2 is highly expressed in neuronal tissues and regulates seizure threshold and neuronal excitation in mice. We conclude that the disruption of SZT2 with some residual function might lead to mild or moderate ID without seizures.