Giant cell arteritis in patients of Indian Subcontinental descent in the UK.

BACKGROUND: GCA in the Indian Subcontinent (ISC) is rare. Our centre in London, UK, serves an ethnically diverse population, including a significant population of patients of ISC descent. We hypothesise that patients of ISC descent are no less likely than others to present with symptoms suggestive of GCA and therefore to undergo temporal artery biopsy (TAB). METHOD: A retrospective audit of all TABs performed at our institution over an 8 year period, to identify ethnicity (white, black, ISC, other, unknown) and biopsy result. We compared the proportion of all patients of ISC descent attending the ED to the proportion of ISC patients undergoing TAB. We compared the proportion of positive TABs among ISC patients with positive TABs among white patients. We also compared the proportion of TAB in ISC patients with all non-ISC ethnicities combined. RESULTS: The proportion of patients undergoing TAB who were of ISC descent (16.3% of 92) was comparable to the proportion of A&E attendances made up by ISC patients [p = 0.1339]. 3.8% (1/26) of positive biopsies were among patients of ISC descent. White patients were significantly more likely to have a positive biopsy than patients of ISC ethnicity (33% of 61 white patients vs. 7% of 15 ISC [p = 0.0456]), as were patients of non-ISC ethnicity (32.5% of 77 non-ISC patients vs. 7% of 15 ISC patients [p = 0.0464]). DISCUSSION: At our centre, biopsy proven GCA occurs in patients of ISC descent, but rarely. Full investigation for GCA continues to be appropriate where it is suspected, regardless of ethnicity.

Uterotonics for Non-emergent Caesarean Section: Protocol Change During UK-Licensed Drug Shortage.

The aim was to assess the efficacy of Syntometrine ® (500 micrograms ergometrine with 5 units oxytocin) as an appropriate alternative first-line uterotonic for use in elective caesarean section (CS) during a national shortage of UK-licensed IV oxytocin from April-June 2014. An observational study was performed involving 2 groups of 22 women undergoing elective CS in a UK DGH during this period. Primary endpoints included mean estimated blood loss (EBL), haemoglobin drop post-operatively and transfusion requirement. Secondary endpoints were use of antiemetics and mean post-operative nausea and vomiting (PONV) score. Results for Syntometrine ® groups and syntocinon groups respectively: mean EBL (ml) 527.3 vs. 550.0 (p=0.5820), mean haemoglobin drop (g/dL) 0.977 vs. 0.982 (p=0.98), blood transfusion 1/22 vs. 0/22 (p=1). Intra-operative antiemetics 20/22 vs. 6/22 (p=<0.001), post-operative antiemetics 2/22 vs. 2/22 (p=1), mean PONV score 11.5 vs. 3.5 (p=0.099). As no significant difference in primary endpoints or PONV scores was observed between regimes, we conclude Syntometrine ® was a safe first-line haemostatic agent for elective CS during oxytocin shortage.

LETTER TO THE EDITOR Screening for Fingolimod Associated Macular Oedema: Experience Versus Guidelines.

We report adherence to United Kingdom national guidelines on surveillance for Fingolimod associated macular oedema (FAME) and the impact on clinical services at our unit. We conducted a 9-month study, measuring referral interval, visual function and performing OCT scans for all patients referred for FAME surveillance. 38 patients in total were seen, representing 9% of all new ophthalmic referrals during the study period. 26% were seen between 2 and 4 months after starting Fingolimod treatment, 74% between 3 and 4 months after starting Fingolimod treatment. The impact on clinical services is discussed.

Parinaud's syndrome - A rare presentation of clinically isolated syndrome.

We present a 26 year old Pakistani lady with first presentation of a demyelinating event, presenting as Parinaud's syndrome. The video demonstrates a convergence-retraction nystagmus on upgaze and failure of accommodation, and her brain imaging confirms a corresponding pre-tectal contrast enhancing T2 hyperintense lesion suggestive of demyelination. A review of the literature is presented.

The Incidence of Blindness Due to Idiopathic Intracranial Hypertension in the UK.

PURPOSE: To determine the incidence of blindness secondary to idiopathic intracranial hypertension (IIH) in the United Kingdom. METHODS: New cases of blindness occurring secondary to IIH were identified prospectively through the British Ophthalmological Surveillance Unit (BOSU) from October 2005 to November 2006. Only idiopathic cases of intracranial hypertension and those meeting the World Health Organisation`s definition of blindness were included. Cases that were already blind or had already been blind registered before the study period were excluded. RESULTS: There were 24 new cases of registerable blindness secondary to IIH reported during the 12 month period. Questionnaires were completed for 19 cases. Of these 19 cases, 3 were not truly idiopathic and 3 cases did not fulfil the strict criteria for blindness. One case was a duplicate report. There were 12 definite cases of blindness secondary to IIH giving a UK incidence of blindness secondary to IIH of 0.6-2% (assuming a UK population of 63.2 million and an incidence of IIH of 1-3/100,000). If the 5 cases reported as blind but without a completed questionnaire are assumed to be true cases then the incidence of blindness would be 1-3%. CONCLUSIONS: The results of this study suggest that approximately 1-2% of new cases of IIH are likely to become blind in a given year. This contrasts with rates of between 4-10% reported previously in hospital-based studies, but may be a more accurate figure for the population as a whole. Under-ascertainment and improving standards of care may also have contributed to the lower figure than previously reported.

Management of suspected appendicitis in children.

Acute appendicitis is the most important cause of abdominal pain in children and is the commonest that requires emergency surgery. Prompt diagnosis, immediate referral and expeditious surgery should be undertaken to reduce the risk of a perforated appendix. The initial presentation may be atypical or be similar to another pathological process which makes the diagnosis extremely difficult. Risk assessment forms an important part in the management of these cases. We consider the difficulties in making the diagnosis including: (A) the pitfalls of clinical examination; (B) the usefulness of laboratory testing; (C) the relevance of clinical scoring systems; and (D) the controversial issue of imaging.

Defining Meyer's loop-temporal lobe resections, visual field deficits and diffusion tensor tractography.

Anterior temporal lobe resection is often complicated by superior quadrantic visual field deficits (VFDs). In some cases this can be severe enough to prohibit driving, even if a patient is free of seizures. These deficits are caused by damage to Meyer's loop of the optic radiation, which shows considerable heterogeneity in its anterior extent. This structure cannot be distinguished using clinical magnetic resonance imaging sequences. Diffusion tensor tractography is an advanced magnetic resonance imaging technique that enables the parcellation of white matter. Using seed voxels antero-lateral to the lateral geniculate nucleus, we applied this technique to 20 control subjects, and 21 postoperative patients. All patients had visual fields assessed with Goldmann perimetry at least three months after surgery. We measured the distance from the tip of Meyer's loop to the temporal pole and horn in all subjects. In addition, we measured the size of temporal lobe resection using postoperative T(1)-weighted images, and quantified VFDs. Nine patients suffered VFDs ranging from 22% to 87% of the contralateral superior quadrant. In patients, the range of distance from the tip of Meyer's loop to the temporal pole was 24-43 mm (mean 34 mm), and the range of distance from the tip of Meyer's loop to the temporal horn was -15 to +9 mm (mean 0 mm). In controls the range of distance from the tip of Meyer's loop to the temporal pole was 24-47 mm (mean 35 mm), and the range of distance from the tip of Meyer's loop to the temporal horn was -11 to +9 mm (mean 0 mm). Both quantitative and qualitative results were in accord with recent dissections of cadaveric brains, and analysis of postoperative VFDs and resection volumes. By applying a linear regression analysis we showed that both distance from the tip of Meyer's loop to the temporal pole and the size of resection were significant predictors of the postoperative VFDs. We conclude that there is considerable variation in the anterior extent of Meyer's loop. In view of this, diffusion tensor tractography of the optic radiation is a potentially useful method to assess an individual patient's risk of postoperative VFDs following anterior temporal lobe resection.

Feasibility of a structured 3-minute walk test as a clinical decision tool for patients presenting to the emergency department with acute dyspnoea.

INTRODUCTION: Emergency department (ED) physicians face frequent decisions on whether to admit patients with congestive heart failure (CHF) or acute exacerbation of chronic obstructive pulmonary disease (COPD). This feasibility study evaluated the potential of a structured 3-minute walk test as a clinical decision tool for admission and correlated its performance with poor clinical outcomes. It also aimed to gather evidence and directions for the design of a multicentre study to derive clinical guidelines. METHODS: In this prospective cohort study, a convenience sample was enrolled of 40 adult patients who presented to a tertiary care ED with CHF, COPD, or stable chest pain and were being considered for discharge. Patients walked at their own pace and their dyspnoea, respiratory rate, heart rate and oxygen saturation were recorded each minute for 4 minutes. The primary outcome was "poor clinical outcome" defined as admission to hospital, the need for biphasic positive airway pressure, the need for intubation, relapse, or death. RESULTS: 85.0% successfully completed the test and 30.0% had poor clinical outcomes. Of those with poor clinical outcomes, 41.7% were unable to complete the test compared with only 3.6% of those with good clinical outcomes (p<0.01). Significant differences were noted in the dyspnoea (p = 0.04) and respiratory rate (p = 0.03) as well as oxygen saturation measurements at 3 minutes. CONCLUSIONS: The 3-minute walk test is a non-resource intensive, simple procedure with applicability in most ED for discharge decisions in patients with cardiopulmonary conditions. Multicentre studies are being planned to validate these findings and establish guidelines for admission and discharge of patients with CHF or acute exacerbation of COPD.

Orbital apex lesion due to Aspergillosis presenting in immunocompetent patients without apparent sinus disease.

We present two cases in which Aspergillus infection was identified at a late stage in the clinical evaluation as the cause for a painful, progressive and indolent orbital apex syndrome in the absence of any clinical or radiological sign of sinus involvement. Surgical investigation was undertaken with serious subsequent morbidity. Although treatment is often satisfactory, neurological outcome is without exception poor. A review of the literature has revealed that the risk of such investigations is high, and advice is provided to readers which may allow avoidance of such complications in the future.

Causes and outcomes for patients presenting with diplopia to an eye casualty department.

PURPOSE: To evaluate the causes and outcomes for patients presenting with diplopia to an eye casualty department. METHODS: Patients presenting with diplopia as a principal symptom, who were referred to the Orthoptic Department from Moorfields Eye Casualty over a 12-month period, were retrospectively investigated. RESULTS: One hundred and seventy-one patients were identified with complete records in 165 cases. There were 99 men and 66 women with an age range of 5-88 years. Monocular diplopia accounted for 19 cases (11.5%), whereas 146 patients (88.5%) had binocular diplopia. Cranial nerve palsies were the most common cause of binocular diplopia accounting for 98 (67%) of cases. Isolated sixth nerve palsy was the largest diagnostic group (n=45). Microvascular disease (hypertension or diabetes mellitus, or both) was present in 59% of patients with cranial nerve palsies, and of this group, 87% resolved spontaneously by 5 months rising to 95% by 12 months. CONCLUSION: Patients with clinically isolated single cranial nerve palsies associated with diabetes or hypertension are likely to recover spontaneously within 5 months and initially require observation only. However, patients with unexplained binocular diplopia and those who progress or fail to recover should be investigated to establish the underlying aetiology and managed as appropriate.

The influence of adaptation on visual motion detection in chronic sixth nerve palsy after treatment with botulinum toxin.

PURPOSE: To investigate changes in visual motion perception after treatment with botulinum toxin in patients with unilateral chronic lateral rectus muscle palsy. METHODS: Five patients and control subjects were asked to report the perceived drift direction of a sinusoidal grating that was initially stationary and then began to accelerate at 0.09 degrees /sec2 in a horizontal direction. The grating had a field size of 18.5 degrees and was presented monocularly with a contrast just above threshold for visibility for central vision. Both the paretic and non-affected eyes were tested. Psychophysical testing was performed under the following conditions: 1) before treatment and testing, patients occluded their paretic eye for at least three days to avoid diplopia. 2) After treatment with botulinum toxin, alignment was corrected and patients stopped occluding their paretic eye for at least three days before testing. The control subjects occluded their non-dominant eye for three days before testing. RESULTS: In condition 1, no differences in motion detection values between patients and control subjects were found. In condition 2, motion detection thresholds were raised approximately 0.15 degrees /sec as compared to pre-treatment values and compared to the control group. CONCLUSIONS: After treatment, a raised threshold for motion detection is one mechanism used to avoid oscillopsia and visuo-vestibular disorientation during head movements in patients with chronic paralytic squint. This study lends evidence that perceptual-adaptive, compensatory mechanisms develop to reduce oscillopsia and disorientation rather than being caused by abnormal cortical motion processing or defective eye muscle action.

Organ transplantation in hereditary apolipoprotein AI amyloidosis.

Patients with hereditary apolipoprotein AI (apoAI) amyloidosis often have extensive visceral amyloid deposits, and many develop end-stage renal failure as young adults. Solid organ transplantation to replace failing organ function in systemic amyloidosis is controversial due to the multisystem and progressive nature of the disease and the risk of recurrence of amyloid in the graft. We report the outcome of solid organ transplantation, including dual transplants in 4 cases, among 10 patients with apoAI amyloidosis who were followed for a median (range) of 16 (4-28) and 9 (0.2-27) years from diagnosis of amyloidosis and transplantation, respectively. Eight of 10 patients were alive, seven with a functioning graft at censor. Two patients died, one of disseminated cytomegalovirus infection 2 months after renal transplantation and the other of multisystem failure following severe trauma more than 13 years after renal transplantation. The renal transplant of one patient failed due to recurrence of amyloid after 25 years. Amyloid disease progression was very slow and the natural history of the condition was favorably altered in both cases in which the liver was transplanted. Failing organs in hereditary apoAI amyloidosis should be replaced since graft survival is excellent and confers substantial survival benefit.

The natural history of Vigabatrin associated visual field defects in patients electing to continue their medication.

PURPOSE: To determine the natural history of visual field defects in a group of patients known to have Vigabatrin-associated changes who elected to continue the medication because of good seizure control. METHODS: All patients taking Vigabatrin alone or in combination with other antiepileptic drugs for at least 5 years (range 5-12 years) were entered into a visual surveillance programme. Patients were followed up at 6-monthly intervals for not less than 18 months (range 18-43 months). In all, 16 patients with unequivocal defects continued the medication. Following already published methodology (Eye 2002; 16;567-571) monocular mean radial degrees (MRDs) to the I/4e isopter on Goldmann perimetry was calculated for the right eye at the time of discovery of a visual field defect and again after not less than 18 months follow-up. RESULTS: Mean right eye MRD at presentation was 36.98 degrees (range 22.25-51.0), compared to 38.40 degrees (range 22.5-49.75) after follow-up; P=0.338 unpaired t-test. Only one patient demonstrated a deterioration in visual field during the study period and discontinued treatment. CONCLUSION: Established visual field defects presumed to be due to Vigabatrin therapy did not usually progress in spite of continuing use of the medication. These data give support to the hypothesis that the pathogenesis of Vigabatrin-associated visual field defects may be an idiosyncratic adverse drug reaction rather than dose-dependent toxicity.