Management approach including pembrolizumab for fingolimod-associated progressive multifocal leukoencephalopathy in a patient with relapsing-remitting multiple sclerosis.

A 62-year-old man with relapsing-remitting multiple sclerosis developed progressive multifocal leukencephalopathy (PML) after 6 years on fingolimod. The fingolimod was immediately discontinued and preexisting mirtazepine increased. Three weeks later, with brain magnetic resonance imaging (MRI) appearances worsening and cerebrospinal fluid (CSF) JC virus (JCV) titres increasing, maraviroc was introduced. At 6 weeks, subtle punctate contrast enhancement raised the possibility of immune reconstitution inflammatory syndrome (IRIS), followed by a single focal-to-generalised tonic clonic seizure and a further deterioration in clinical disability. Mefloquine was commenced alongside three doses of pembrolizumab administered a month apart. Serial CSF examinations and several imaging modalities including spectroscopy and fused FDG-PET-MRI (18F-fluoro-deoxy-glucose-positron emission tomography-magnetic resonance imaging) were used to help distinguish between PML, PML-IRIS and rebound MS activity and guide optimal management at each stage. A handful of small, enhancing ovoid lesions developed between the first two doses of pembrolizumab, probably representative of a mild rebound phenomenon. A sustained improvement became obvious thereafter with CSF JCV-DNA undetectable 16 weeks following fingolimod withdrawal. To our knowledge, this is the first case of combined therapy and use of pembrolizumab in a fingolimod-associated PML.

Sporadic CJD in association with HIV.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal neurodegenerative disorder. We report an unusual case of pathologically confirmed sporadic CJD developing in a HIV-positive patient but presenting with clinical and radiological features suggestive of variant CJD. CASE PRESENTATION: A 63-year-old man with chronic stable HIV developed progressive difficulties with decision-making, obsessive compulsive disorder and visual hallucinations over 3 months. CSF examination detected a weakly positive 14-3-3 protein, elevated S-100 protein, and siginificantly elevated total-Tau protein. Brain MRI revealed bilateral abnormal signal within the posterolateral thalami compatible with pulvinar sign. Further investigations revealed a negative tonsillar biospy and positive blood test consistent with variant CJD. However, prion protein genotyping detected MV heterozygosity at codon 129 and post-mortem histopathological examination was consistent with sporadic CJD. CONCLUSION: Although MRI findings were suggestive of variant CJD, the short residence in the UK and MV heterozygosity are aytpical, and the histopathological examination was consistent with sporadic CJD. With only two cases of HIV and sporadic CJD reported so far, the association of CJD with HIV remains unclear.

Varicella vasculopathy presenting with thunderclap headache.

Our report serves to highlight Varicella vasculopathy as a rarity not to be overlooked in the differential diagnosis of subarachnoid haemorrhage.

Use of Hydrocoil in small aneurysms: procedural safety, treatment efficacy and factors predicting complete occlusion.

Coil technology has been directed to reduce recurrence rates and we have seen the introduction of trials comparing the efficacy of surface modified versus bare platinum coils (BPC). This article reports on one treatment strategy in the treatment of small aneurysms by the placement of Hydrocoil across the neck of the aneurysm. Procedural safety, treatment efficacy and factors which predict complete occlusion are evaluated. We retrospectively identified a subgroup of small aneurysms treated over a four-year period. Analysis comparing aneurysms treated with Hydrocoil and BPC versus Hydrocoil alone was undertaken. Eighty-five aneurysms were coiled; 62% with Hydrocoil alone, 38% in combination with BPC. At six-month follow-up, overall 50% were completely occluded, 39.5% had a neck remnant and 10.5% had a residual aneurysm. Complete occlusion was identified in 39% in the Hydrocoil and BPC group compared to 56% in the Hydrocoil alone group. In 56/76 (74%) cases analysed, Hydrocoil loop successfully bridged the neck of the aneurysm in which 38/76 (68%) of these were completely occluded at six-month follow-up. Thirteen procedure-related complications occurred. Aneurysms treated with Hydrocoil alone resulted in fewer recurrences compared with a combination of Hydrocoil and BPC. These data suggest that the technique of positioning Hydrocoil at the neck of the aneurysm increases the probability of complete occlusion and is therefore a strong predictor of aneurysm occlusion. In our experience, this technique did not demonstrate an increased risk of intra-procedural rupture or thrombo-embolic complications compared to conventional embolization with BPC.

Decreased consciousness: bilateral thalamic infarction and its relation to the artery of Percheron.

This case series highlights two patients seen in the same stroke centre presenting with unusual symptoms. They were later diagnosed with bilateral thalamic infarcts, probably related to an unusual anatomical variant. The difficulties in establishing the diagnoses due to their relative rarity and complexity could have impacted on patient outcomes.

Is cardiac computed tomography a reliable alternative to percutaneous coronary angiography for patients awaiting valve surgery?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether a CT angiogram could replace routine percutaneous coronary angiography for excluding coronary arterial disease for patients undergoing a non coronary cardiac procedure. Using the reported search 595 papers were identified. Eleven papers represented the best evidence on the subject and the author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study comments and weaknesses were tabulated. We conclude that angiography with 64-slice multi-detector CT scanner provides reliable non-invasive imaging to exclude significant coronary artery stenoses prior to valve surgery. The negative predictive value of a normal CT scan is around 97%, thus providing a good alternative to conventional angiography in lower atherosclerotic risk patients. The ability of CT angiography to assess the reduction in luminal diameter is reduced in the presence of calcium deposits, and is also reduced in vessels under 1.5 mm. Further disadvantages include an inability to perform scans in patients with arrhythmias or atrial fibrillation, and a five times increased radiation dose compared to conventional angiography.