Bulbar-onset ALS patients with cognitive impairments and neuronal loss in the anterior cingulate gyrus subsequently developed more profound neuropsychological dysfunction whereas both language and speech capabilities remained relatively preserved. Of note, the absence of bulbar signs did not predict an absence of cognitive decline.
Increased numbers of referrals would be consistent with an awareness raising effect of NICE/SCIE and NDS and a willingness among GPs to refer cases. But the falling proportion of dementia diagnoses suggests that these are 'worried well' individuals. There is no evidence for closure of the dementia 'diagnosis gap'.
Giant cell arteritis (GCA) is a rare form of large and medium vessel vasculitis affecting about 20 cases per 100,000 persons older than the age of 50 years. GCA results in inflammation and constriction of the temporal arteries, cranial arteries, the aorta, and its major branches. Patients often present with vague constitutional symptoms and fever of unknown origin. GCA is a medical emergency requiring prompt diagnosis and early treatment with glucocorticoids which is essential to avoid irreversible end organ damage such as loss of vision, stroke and aneurysm formation. We report a case of a 63-year-old male patient presenting to our healthcare facility with sudden loss of vision and an ischemic brain infarct to be finally diagnosed as a case of giant cell arteritis with positron emission tomography-computed tomography imaging used to evaluate the full extent of the involved vasculature. Diagnostic imaging with FDG positron emission tomography-computed tomography can play a crucial role in the diagnosis, evaluation of the full burden of the disease and follow up to the response of therapy.
The stroke treatment world wide has seen a giant leap in last few years as the science has moved from systemic infusion of clot busting drugs to highly selective intra arterial intervention. The intervention requires careful selection of subset of stroke secondary to large vessel occlusion in a timely fasshion. Since the publication of MR CLEAN ( Multicenter Randomised CLinical trial of Endovascular treatment for acute ischaemic stroke in the Netherlands) trial in 2014 and recent NICE approval to provide this service UK wide stroke services will face challanges to provide this in an equitable fashion. We at Walton Centre have been providing this service to the whole of Cheshire, Merseyside since 2013. Through this audit we would like to share our experience of stroke intervention in this stand alone neurosciences centre. This audit compared the standards against the European Stroke organisation guidelines for ischaemic stroke 2016. We measured the following key parameters along with clinical outcome.1) Does recanalisation influence immediate neurological recovery.2) Does bridging thrombectomy (IV thrombolysis +thrombectomy) differ from primary thrombectomy.3) Does performing the service out of hours influence the outcome
Intracranial dural arteriovenous fistula is a rare cause of reversible vascular dementia, through a presumed mechanism of intracerebral venous hypertension. The clinical presentations are variable with no easily recognisable syndrome. It is a treatable and reversible cause of cognitive decline, unexplained otherwise. Often the sole presentation could be progressive cognitive decline to suggest a neurodegenerative dementia however the careful clinical history may give the clue of increasing intracranial pressure. Careful radiological review will help to intervene early to cure this condition. We report a case of 55 year old man who presented with progressive symptoms of pulsatile tinnitus for two years, with features of encephalopathy for six month and subsequent focal neurological symptoms and seizure. He was initially diagnosed as dementia and was later treated as stroke before he was referred to our centre. MRI scan of brain revealed dilated veins along the cerebral convexities and the brain stem with progressive diffuse white matter oedema. Cerebral angiography revealed a dural A-V fistula around the right transverse/sigmoid sinus. His history of familial hemochromatosis raises the suspicion of hyperviscosity induced venous thrombus as the putative cause. This was treated with onyx embolisation. His symptoms and the radiological abnormalities resolved completely.
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