A prospective cohort study was done to evaluate the role of surgery in patients with spontaneous supratentorial intracerebral haemorrhage (SICH) and to identify predictors of outcome including the use of invasive regional cortical cerebral blood flow (rCoBF) and microdialysis. Surgery consisted of craniotomy or decompressive craniectomy. The ventriculostomy for intracranial pressure (ICP) monitoring and drainage and regional cortical cerebral blood flow (rCoBF) and microdialysis were performed in all subjects. Pre and post operative information on subjects were collected. The study end points was functional outcome at 6 months based on a dichotomised Glasgow Outcome Scale (GOS).The selected clinical, radiological, biochemical and treatment factors that may influence the functional outcome were analysed for their significance. A total of 36 patients were recruited with 27(75%) patients had Glasgow Coma Score (GCS) between 5 to 8 on admission and 9(25%) were admitted with GCS of 9. At 6 months, 86 % had a poor or unfavourable outcome (GOS I-III) and 14% had good or favourable outcome (GOS IV-V). The mortality rate at 6 months was 55%. Univariate analysis for the functional outcome identified 2 significant variables, the midline shift (p=0.013) and mean lactate:pyruvate ratio (p=0.038). Multivariate analysis identified midline shift as the single significant independent predictor of functional outcome (p=0.013).Despite aggressive regional cortical cerebral blood flow (rCoBF) and microdialysis study for detection of early ischemia, surgical treatment for spontaneous intracerebral haemorrhage only benefited a small number of patients in terms of favourable outcome (14%) and in the majority of patients (86%), the outcome was unfavourable. Patients with midline shift > 5mm has almost 21 times higher chances (adj) OR 20.8 of being associated with poor outcome (GOS I-III).

We present a case of beta-propeller protein-associated neurodegeneration, a form of neurodegeneration with brain iron accumulation. The patient harbored a novel mutation in the WDR45 gene. A detailed video and description of her clinical condition are provided. Her movement disorder phenomenology was characterized primarily by limb stereotypies and gait dyspraxia. The patient's disability was advanced by the time iron-chelating therapy with deferiprone was initiated, and no clinical response in terms of cognitive function, behavior, speech, or movements were observed after one year of treatment.
Brain ; Chelation Therapy ; Cognition ; Extremities ; Gait Apraxia ; Humans ; Iron ; Movement Disorders

Introduction: Self-assessment is a mechanism to evaluate one’s own performance and identify strengths and weaknesses. However, there is paucity of information on the the validity of self-assessment in the literature. This study is planned to assess the validity of medical students’ self-assessment skills in clinical examinations with long case during their postings in Obstetrics and Gynaecology. Methods: A cross sectional study was conducted during Obstetrics and Gynaecology (O&G) rotation assessing the students performance in the clinical long case examination. Participants were 80 fourth year medical students who were in their last week of their O&G rotation between August and December 2019. Each student was given a random case from the obstetric ward for the clinical long case examination during their final week of posting. At the end of examination, both student and examiner were tasked to independently evaluate the performance with a standardized grading sheet. Students were assessed in three areas namely Case Presentation (4 items), Case Discussion (4 items) and Professionalism and Overall approach to the practice of medicine (2 items). Results: The correlation coefficient was identified to be 0.307, p<0.01, indicating validity in self-assessment in the context of a clinical examination. The overall bias index was -0.97. Students underrated themselves in all areas with bias indexes of -0.35 in case presentation, -0.26 for case discussion and -0.35 in the professionalism and overall approach to practice. The correlation coefficients were 0.186, 0.360 and 0.170 respectively, indicating that in isolation only the component of case discussion showed significant correlation (p<0.01). Conclusion: Self-assessment in clinical examination is shown to be a valid assessment method when multiple assessment items are combined.