Neuropsychological and neuroimaging characteristics of classical superficial siderosis.

OBJECTIVE: 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. METHODS: We present the detailed neuropsychological and neuroimaging findings in 16 patients with iSS (mean age 57 years; 6 female). RESULTS: 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. CONCLUSION: 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.

Correction to: A systematic review of internet-based information for individuals with Raynaud's phenomenon and patients with systemic sclerosis.

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.

A systematic review of internet-based information for individuals with Raynaud's phenomenon and patients with systemic sclerosis.

INTRODUCTION: 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). MATERIAL AND METHODS: 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. RESULTS: 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 (1.99 & 2.21), 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. CONCLUSION: 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. KEY POINTS: • 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.