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Background and Purpose Malignant middle cerebral artery infarction(MMI) affects 5–10% of acute ischaemic stroke patients. Mortality of ∼80% if treated conservatively leaves decompressive surgery the choice treatment, halving mortality if undertaken early. We aimed to determine early clinical and CT based imaging factors that predict MMI. Methods We undertook a single centre retrospective study using an acute ischaemic stroke database that included CT head, CT angiography(CTA) and CT perfusion(CTP) scans 50% of MCA territory and space-occupying oedema on 24 h CT. Univariate and multivariate binary logistic regression identified predictive factors. Results Of 210 patients, 22 developed MMI. These had higher baseline NIHSS score, lower ASPECTS, larger ischaemic and infarct volumes, more large vessel occlusion and poor collateral circulation (all p= 75 ml on admission CTP, correctly classified 91.5% of patients. Conclusions CT imaging within 6 h of stroke onset can predict MMI. Multimodal CT allows early identification of those at risk of MMI.

Background and aims Intravenous Immunoglobulin (IVIG) is used to treat a variety of immunologically mediated neurological conditions.The Association of British Neurologists (ABN) published guidelines in 2005 for use of IVIG in neurological conditions. 1 These guidelines outline different indications for prescribing IVIG with research evidence and guidelines for monitoring patients on treatment. Our audit aimed to compare our practice in Aberdeen Royal Infirmary for the period of August 2010 to July 2011 to the ABN guidelines. We gathered data on indications of use of IVIG and 9 monitoring guidelines. Results A total of 36 Patients received IVIG and complete data were available for analysis for 32 patients. Indications for treatment included: Guillain–Barre Syndrome (GBS) (n=10), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) (n=8), Myasthenia Gravis (n=5), Multiple Sclerosis (MS) and Paraprotein associated neuropathy (n=2), Limbic encephalitis, Multifocal Motor Neuropathy (MMN), other CNS inflammation, Polyneuropathy, Organomegaly, Endocrinopathy, M–protein and Skin changes (POEMS) syndrome, and other demyelinating neuropathy (not CIDP) (n=1). In most of these patients other immunosuppressive treatments had been contraindicated or tried and failed, apart from the MMN group. In terms of the monitoring guidelines, we found that all patients had routine blood tests prior to the first infusion, but in nine patients pre–treatment IgA levels were not performed. 18 Patients received more than one infusion and of these ten did not have regular further pre–treatment blood tests. Informed consent was documented in only 5 patients. Out of 14 patients who had documented improvement after the first IVIG infusion, and received further infusions, four had no documented clinical deterioration prior to subsequent infusions. Only five out of the 18 patients on regular treatment had documentation on consideration of reduction in treatment dose and frequency. Intravenous Hydrocortisone and Chlorpheneramine were given as prophylaxis for allergic reactions in 17 patients before the infusion with no documented prior side effects. No patient information sheets or signed consent forms were used. The cost of IVIG was just under £0.5 million during the audit year. Outcomes We presented these results locally and designed a protocol that includes written consent forms and patient information sheets (1). We agreed on annual consultant review dates (considering dose and frequency reduction, need for continuing IVIG use or alternative treatments) and monitoring of blood tests. We also discussed the judicious use of pre–treatment intravenous Chlorpheneramine and Hydrocortisone and consideration of oral treatment due to cost benefits where appropriate. We aim to implement the authorised protocol and close the audit cycle by repeating this in 2014.

We report a case of a twenty three year old female presenting with status epilepticus due to a rare mutation in a nuclear gene causing mitochondrial dysfunction. She had previously been investigated for an inherited ataxia without a definitive diagnosis being made. She presented as an emergency with focal seizures that rapidly evolved into intractable status epilepticus. Despite prolonged, aggressive treatment in Intensive Care Unit, she remained encephalopathic with persisting seizures. Her younger sister who also has milder symptoms with ataxia visited our hospital on the day of acute admission and revealed some useful information that led us investigate and confirm the diagnosis in our patient. Her younger sister who was a medical student told us that she had been investigated elsewhere and was confirmed to have an autosomal recessive mutation in a nuclear gene named POLG. We tested and confirmed the same POLG mutation in our patient. POLG is a nuclear gene located on long arm of chromosome 15 that encodes the catalytic subunit of mitochondrial DNA polymerase gamma. The defects in this enzyme are associated with disorders of mitochondrial DNA maintenance. POLG mutation has several phenotypes with onset between early childhood to late adulthood. Typical clinical features include ataxia, neuropathy, epilepsy, ophthalmoplegia, myopathy and in some cases liver failure. Liver failure has been associated with treatment with Sodium valproate and this should be avoided in these patients. Prognosis is poor in the presence of epilepsy with mortality rates to be reported to be nearly 80%. After extensive discussions with the family, local ethics team, and medical director, our patient was palliated in the local palliative care hospital where she died peacefully. A clinical intuition is needed for referring these patients to genetic testing and the diagnosis is crucial to inform us the prognosis. We discuss her presentation, management and our patient9s phenotype Mitochondrial Spino–Cerebellar Ataxia and Epilepsy (MSCAE).