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The authors have identified an error in thier manuscript and have incorrectly referred to the overall DISCERN score for SSc and RP as 2.21 and 1.99, respectively, in the abstract and discussion. The correct DISCERN quality scores are listed in Table 2.

Superficial siderosis describes haemosiderin deposition on the surface of the brain. When present on infratentorial structures, it can cause ataxia, sensorineural hearing loss and pyramidal signs. There is no proven treatment and patients experience slow progression of symptoms. Iron-chelating agents have been suggested as a therapeutic option and deferiprone is suited as it crosses the blood-brain barrier. However, deferiprone is reported to have a 1–2% risk of agranulocytosis. We performed a systematic review on treatment of infratentorial superficial siderosis with deferiprone based on PRISMA guidelines. Studies were included if in English or an English language translation was available, were about human subjects and referred to patients with ataxia. Studies were excluded if they did not possess an English translation, included animal studies or did not have ataxia. Studies were excluded if they discussed cerebral amyloid angiopathy or siderosis of other regions. Eleven papers were included. We identified 69 patients. Seventeen patients (25%) discontinued the drug. The most encountered adverse effect was anaemia (21.7%). Neutropaenia was observed in 8.7% and agranulocytosis in 5.8% of patients. Clinically, response varied, and stability or improvement was seen across neurological domains in 6 studies while 5 showed a mixed response. On imaging, 13 (28.9%) patients improved, 24 (53.3%) stabilised and 8 (17.8%) deteriorated. A prospective international centralised register of patients should be developed to inform the design and conduct of a multicentre, placebo-controlled, randomised clinical trial to evaluate the efficacy of deferiprone. The evidence from this systematic review is that deferiprone is a promising intervention.

Background In posterior circulation stroke, vertigo can be a presenting feature. However, whether isolated hemispheric strokes present with vertigo is less clear, despite a few single case-reports in the literature. Here we, a) explored the prevalence of vertigo/dizziness in acute stroke and, b) considered the cortical distribution of these lesions in relation to both the known vestibular cortex and evolution of these symptoms. Methods We conducted structured interviews in 173 consecutive unselected patients admitted to the hyperacute stroke unit at the University College London Hospitals. The interview was used to evaluate whether the patient was suffering from dizziness and/or vertigo before the onset of the stroke and at the time of the stroke (acute dizziness/vertigo), and the nature of these symptoms. Results 112 patients had subcortical lesions and 53 patients had cortical infarcts, of which 21 patients reported acute dizziness. Out of these 21, five patients reported rotational vertigo. 17 of the total 53 patients had lesions in known vestibular cortical areas distributed within the insular and parietal opercular cortices. Conclusions The prevalence of vertigo in acute cortical strokes was 9%, with no single locus of lesion overlap. There is growing evidence supporting a lateralised vestibular cortex, with speculation that cortical strokes affecting the right hemisphere, are more likely to cause vestibular symptoms than left-hemispheric strokes. We observed a trend for this association, with the right hemisphere affected in four of five patients who reported spinning vertigo at the onset of the stroke.

To define the neuropsychological and neuroimaging characteristics of classical infratentorial superficial siderosis (iSS), a rare but disabling disorder defined by hemosiderin deposition affecting the superficial layers of the cerebellum, brainstem and spinal cord, usually associated with a slowly progressive neurological syndrome of deafness, ataxia and myelopathy. We present the detailed neuropsychological and neuroimaging findings in 16 patients with iSS (mean age 57 years; 6 female). Cognitive impairment was present in 8/16 (50%) of patients: executive dysfunction was the most prevalent (44%), followed by impairment of visual recognition memory (27%); other cognitive domains were largely spared. Disease symptom duration was significantly correlated with the number of cognitive domains impaired (r = 0.59, p = 0.011). Mood disorders were also common (anxiety 62%, depression 38%, both 69%) but not associated with disease symptom duration. MRI findings revealed siderosis was not only in infratentorial brain regions, but also in characteristic widespread symmetrical supratentorial brain regions, independent of disease duration and degree of cognitive impairment. The presence of small vessel disease markers was very low and did not account for the cognitive impairment observed. Neuropsychological disturbances are common in iSS and need to be routinely investigated. The lack of association between the anatomical extent of hemosiderin and cognitive impairment or disease duration suggests that hemosiderin itself is not directly neurotoxic. Additional biomarkers of iSS disease severity and progression are needed for future research and clinical trials.

Background Interstitial lung disease (ILD) is a serious extra-articular manifestation of rheumatoid arthritis (RA). Risk factors include smoking, the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (CCP). Pulmonary function tests (PFT) show reduced carbon monoxide diffusion capacity (DLCO) early and reduced forced vital capacity (FVC) later in disease. HRCT is the gold standard diagnostic test while chest X-ray (CXR) has low sensitivity. PFT are routinely performed in the majority of RA patients at baseline at our tertiary centre. The aim of this study was to evaluate the frequency of abnormal PFT, specificity for ILD and influence on subsequent decision-making in patients newly diagnosed with RA. Methods A retrospective analysis was undertaken of patients with a new diagnosis of RA between January 2016 and December 2017. Patients meeting the ACR (2010) criteria for RA, with baseline PFT data available were included. Clinic letters and the hospital electronic records were used to obtain the data. Results 139 patients were included in the data analysis (Table 1). 23 patients had DLCO Conclusion This evaluation suggests baseline PFT are more sensitive than baseline CXR in detecting ILD but that a DLCO Disclosures A. Haque None. R. Kilding None. R. Smith None. S. Khalid None. R. Sandler None. M. Cox None. T. Hendry None. A. Flores-martin None. K. Lindop None. J. Maxwell None.

Patients are increasingly using internet-based information to inform healthcare utilization and treatment decisions. Our aim was to examine the quality and readability of internet-based information relating to Raynaud's phenomenon (RP) and systemic sclerosis (SSc).A systematic review of three commonly used search engines (Google®, Yahoo®, and Bing®) using the terms "Raynaud's phenomenon" and "systemic sclerosis" separately. The first 30 websites per search engine were examined. Quality was assessed using the DISCERN questionnaire and readability by the Flesch-Kincaid Grade Level, SMOG Index, Coleman-Liau index, and Flesch Reading Ease score.Fifty-two studies (30 RP and 22 SSc) were included after duplicates and exclusion criteria were applied. The overall quality of information was low for both SSc and RP (1.992.21), including in relation to reliability of the literature and information on treatment choices. Readability for RP and SSc was also poor (i.e., the texts were difficult to read) across all of the four methods examined.Overall, RP and SSc internet-based information is of low quality and inadequate readability. The RP and SSc international community should strongly consider developing an information standard for internet-based resources for healthcare users.• Patients with SSc and RP are increasingly using internet/online sources of information and support. • RP represents an important opportunity for the early diagnosis of SSc. • The overall quality and readability of internet-based RP and SSc information is poor. • Internet-based RP and SSc information requires improvement to facilitate early diagnosis and inform shared decision-making.

The authors have identified an error in thier manuscript and have incorrectly referred to the overall DISCERN score for SSc and RP as 2.21 and 1.99, respectively, in the abstract and discussion. The correct DISCERN quality scores are listed in Table 2.