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Objective Deferiprone is an iron chelator that has recently been used to treat patients with infratentorial superficial siderosis (iSS). It is considered to have a generally favourable safety profile but concerns have been raised due to the risk of agranulocytosis. We aimed to evaluate the safety and tolerability of oral deferiprone as a treatment for patients with iSS. Methods We present a case series of 10 consecutive patients presenting with classical iSS treated with deferiprone. Results Ten patients were followed up for a mean period of 2.3 years (range 0.5–5.5 years). Four patients (40%) were withdrawn from treatment because of treatment-related side effects. The reasons for treatment discontinuation were neutropenic sepsis (n = 3) and fatigue (n = 1). In 2 out of the 3 cases of neutropenic sepsis, patients initially developed neutropenia without sepsis. The mean time to neutropenic sepsis following deferiprone was 1.2 years (range 0.3–2.5) with mean neutrophil count of 0.4 (range 0.3–0.5). Six patients (60%) reported no change in neurological function while on treatment, and four patients (40%) reported that their condition deteriorated. Conclusions Deferiprone was poorly tolerated, with 40% of patients withdrawing from treatment, most commonly due to neutropenic sepsis, after an average of 2 years on treatment. This study increases the number of reported cases of agranulocytosis in patients with iSS treated with deferiprone. Clinicians treating iSS patients with deferiprone should be aware that this drug has a potentially life-threatening side effect of neutropenic sepsis, and should ensure that appropriate haematological monitoring is in place.

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.

Evaluate long-term efficacy and safety of ITB in treating MS-related spasticity over ∼ 20 years of service provision in a single centre.A single centre prospective observational cohort study was performed. Eligible subjects underwent ITB trial by bolus dose via lumbar puncture and responders proceeded to pump implantation. Demographics, spasticity scores (Ashworth), spasm score (Penn), stiffness, pain and discomfort (Visual Analogue Scale), mobility (10 M walk), spasticity treatment, and ITB doses were analysed longitudinally.106 people were included with 568 patient years of data. Ashworth, Penn and VAS/NRS mean scores improved post-trial compared with baseline (p 0.001). Sustained efficacy was reported on Ashworth, Penn and VAS scores over time. After 1 year, 73 (69%) discontinued all oral antispasticity medications. Complication rates were low at 0.05 complications per pump year and mostly mechanical (usually catheter) related. In 8 ambulatory subjects, 7 (87%) continued to walk one year after pump insertion, 5 (62%) were still walking at time of analysis (mean follow up 3.4 years).ITB is an effective and safe long term treatment for refractory MS related spasticity. Efficacy was sustained over time and the majority of subjects subsequently discontinued systemic medications. In a small cohort, ability to walk was preserved, indicating ITB should be considered earlier in this cohort.

In contrast to peer-assisted learning (PAL) in clinical training, there is scant literature on the efficacy of PAL during basic medical sciences teaching for preclinical students. A group of senior medical students aimed to design and deliver clinically oriented small-group tutorials after every module in the preclinical curriculum at a United Kingdom medical school. Twenty tutorials were delivered by senior students throughout the year to first- and second-year students. A baseline questionnaire was delivered to inform the development of the program followed by an end-point questionnaire the next year ( n = 122). Quizzes were administered before and after five separate tutorials to assess changes in mean student scores. Additionally, each tutorial was evaluated via a questionnaire for participants ( n = 949). All five posttutorial quizzes showed a significant improvement in mean student score ( P < 0.05). Questionnaires showed students found the program to be relevant and useful for revision purposes and appreciated how tutorials contextualized basic science to clinical medicine. Students appreciated the interactive nature of the sessions and found receiving personalized feedback about their learning and consolidating information with someone familiar with the material to be useful. With the inclusion of the program, students felt there were now an adequate number of tutorials during the year. In conclusion, this study shows that senior medical students can design and deliver a program that adds value to the mostly lecture-based formal preclinical curriculum. We hope that our study can prompt further work to explore the effect of PAL on the teaching of basic sciences during preclinical studies.

Peer-led teaching is an established paradigm with benefits for student teachers, learners and the wider medical community. Students are increasingly taking ownership of such teaching, which has fuelled the creation of new peer-led medical education societies at universities around the UK. Students wishing to undertake such an endeavor must contend with concerns over the quality of peer-led teaching, logistical challenges, lack of senior support and difficulties accessing relevant resources to design and appraise their initiatives. Peer-led medical education societies represent a relatively novel concept, and students may struggle to find practical information on how to approach these challenges. We propose that these obstacles can be overcome by thorough event planning, understanding the role and features of high quality peer-led education in supplementing medical school curricula, maintaining a strong working relationship with local medical faculty, and learning from the wider medical education community.