A previously well 54- year-old woman presented with a short history of diplopia, cognitive decline, hallucinations and hypersomnolence. The patient had progressive deterioration in short-term memory, ocular convergence spasm, tremor, myoclonus, gait apraxia, central fever, dream enactment and seizures. Results of investigations were normal including MRI brain, electroencephalogram, cerebrospinal fluid (CSF, including CSF prion protein markers) and brain biopsy. The patient died from pneumonia and pulmonary embolus. Brain postmortem analysis revealed neuropathological changes in keeping with Fatal familial insomnia (FFI); the diagnosis was confirmed on genetic testing. FFI is caused by an autosomal dominant and highly penetrant pathogenic Prion Protein gene PRNP. Although usually familial, fatal insomnia (FI) also occurs in a rare sporadic form. FI is a rare human prion disease with prominent sleep disturbance, autonomic, motor, cognitive and behavioural involvement. Patient management is with best supportive care and early suspected diagnosis allows for timely palliation.
IntroductionSuccessful Trainee Clinical Research Networks have been established since 2007. Our network in the peninsula, the SOuthwest Neurology Audit and Research Group (SONAR) is the first such Neurology trainee network in the UK. To enable development of cohesive collaborative working of the network we designed an audit which would be deliverable across three neurology centres within the peninsula.MethodWe audited management of suspected acute meningitis and meningococcal sepsis against national guidelines within a 4 week period in December. A standardised anonymsed data collation tool was used across the three centres and results were analysed at one centre.ResultsAll 9 registrars on the rotation contributed to audit methodology design and data analysis; seven contributed cases (from all three centres). Ten cases were included in the audit, 6 (Exeter), 3 (Plymouth), and 1 (Truro). Our audit highlighted deficiencies in timely senior review, delivery of antibiotics and steroids, inappropriate administration of acyclovir and delay in lumbar puncture.ConclusionThis was SONAR’s first collaborative project and demonstrated that as a group of trainees we can successfully conduct a project across multiple hospital sites. We plan to extend the scope and ambition of our future undertakings.
IntroductionSuccessful Trainee Clinical Research Networks have been established since 2007 and are primarily run by Surgical and Anaesthetic Trainees. In the southwest peninsula we have set up the first UK Neurology Trainee Audit and Research Collaborative to deliver clinical studies. Ensuring all trainees have appropriate training is a key requirement; we aimed to ascertain the training needs of our network members.MethodA survey was sent to all 9 neurology trainees in the Peninsula Deanery. It comprised 5 questions to establish trainee clinical research training and experience.ResultsResponse rate was 100%. Training level varied from ST3–5; 22% had previously completed higher degrees. 40% of trainees had not been involved in clinical research. One trainee had not had formal good clinical practice (GCP) training and none had formal Informed Consent training. Of those who had been involved in research, there had been limited involvement in project design, ethics approval processes, data analysis, manuscript preparation or findings presentation.ConclusionWe identified a training need in our Trainee Audit and Research Network. In order to address this, we have organised formal GCP and Informed Consent training; to broaden the research experience of network members, we are planning our first collaborative research project.
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