Measles and Eye Complications

Presentation A 37-year-old Polish male presented with headache, myalgia, rash and reduced visual acuity. Diagnosis Measles was confirmed on day 5 via throat swab, complicated by ocular keratitis which was diagnosed by slit lamp exam using fluorescein staining. Treatment Once the diagnosis was confirmed, the patient received supportive medical treatment and topical treatment with six - hourly preservative free dexamethasone and sodium hyaluronate to both eyes was prescribed. Conclusion Measles cases have increased by 244% in Ireland1 remaining an important public health concern. This case illustrates the difficulty in diagnosis, ocular manifestations and serves to remind health care professionals that this potentially devastating disease has not gone away.

The ophthalmic diagnosis and management of four siblings with Werner syndrome.

INTRODUCTION: Werner syndrome is a rare autosomal recessive disorder caused by mutations in the Werner syndrome WRN gene, on chromosome 8. Those affected manifest early the features of ageing. DISCUSSION: Cataract surgery is prone to post-operative complications in those with Werner syndrome. The development of cystoid macular oedema (CMO) is likely multifactorial. Patients with WS have diabetes mellitus type 2 which can contribute to macular oedema. There is a deposition of abnormal WRN proteins in the macula which also predisposes to macular oedema. The trauma of cataract surgery appears to be the main stimulus for the development of CMO. CMO may, as a result, be difficult to manage in Werner syndrome patients. CONCLUSION: Further study is needed to elucidate the precise role of retinal WRN protein expression in the development of CMO in those with Werner syndrome. A tailored and more successful approach to the treatment of CMO in such patients may result.

The interdisciplinary management of hypodontia patients in the UK: a national service evaluation.

Objective To examine the distribution of interdisciplinary clinics for hypodontia patients in the UK and to assess the provision of orthodontic-restorative care for hypodontia patients in units where this service is and is not available.Design Prospective, online-questionnaire.Setting Hospital orthodontic departments in the UK.Participants In total, consultants from 92 orthodontic departments completed the questionnaire.Methods Orthodontic consultants were asked to complete a questionnaire regarding the provision of orthodontic-restorative care for hypodontia patients in their units.Results Overall, 100% of teaching hospitals and 51% of district general hospitals (DGHs) that responded have an interdisciplinary clinic for hypodontia patients. In 51% of units, the majority of patients assessed on the interdisciplinary clinic undergo their restorative care at the same secondary care unit. In 59% of units where an interdisciplinary clinic is not available most of the restorative care for hypodontia patients is provided by the GDP, whilst in 38% of units a specialist restorative dentist in another secondary care unit provides most of the restorative care.Conclusions The provision of an interdisciplinary clinic for hypodontia patients varies amongst hospital units throughout the UK. The provision of orthodontic-restorative care for hypodontia patients also varies between these units.

Epidemiological, clinical and genetic aspects of adult onset isolated focal dystonia in Ireland.

BACKGROUND: Adult onset idiopathic isolated focal dystonia presents with a number of phenotypes. Reported prevalence rates vary considerably; well-characterized cohorts are important to our understanding of this disorder. AIM: To perform a nationwide epidemiological study of adult onset idiopathic isolated focal dystonia in the Republic of Ireland. METHODS: Patients with adult onset idiopathic isolated focal dystonia were recruited from multiple sources. Diagnosis was based on assessment by a neurologist with an expertise in movement disorders. When consent was obtained, a number of clinical features including family history were assessed. RESULTS: On the prevalence date there were 592 individuals in Ireland with adult onset idiopathic isolated focal dystonia, a point prevalence of 17.8 per 100 000 (95% confidence interval 16.4-19.2). Phenotype numbers were cervical dystonia 410 (69.2%), blepharospasm 102 (17.2%), focal hand dystonia 39 (6.6%), spasmodic dysphonia 18 (3.0%), musician's dystonia 17 (2.9%) and oromandibular dystonia six (1.0%). Sixty-two (16.5%) of 375 consenting index cases had a relative with clinically confirmed adult onset idiopathic isolated focal dystonia (18 multiplex and 24 duplex families). Marked variations in the proportions of patients with tremor, segmental spread, sensory tricks, pain and psychiatric symptoms by phenotype were documented. CONCLUSIONS: The prevalence of adult onset idiopathic isolated focal dystonia in Ireland is higher than that recorded in many similar service-based epidemiological studies but is still likely to be an underestimate. The low proportion of individuals with blepharospasm may reflect reduced environmental exposure to sunlight in Ireland. This study will serve as a resource for international comparative studies of environmental and genetic factors in the pathogenesis of the disorder.