The probability of a disease following a diagnostic test is critically reliant on prior clinical probability
A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, Gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the Kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.
A patient is reported on with a subarachnoid haemorrhage (SAH) from an aneurysm of the posterior communicating artery, who initially presented with a sentinel bleed into an arachnoid cyst and normal magnetic resonance angiography (MRA) of the intracranial vasculature which led to a delay in diagnosis. Although this is a very rare presentation of a relatively common condition, it is important to recognise the importance of intracystic haemorrhage in such circumstances as well as the limitations of MRA, as a delay in diagnosis may have serious clinical consequences.
A 68year oldman isdescribed withan alien left hand,cortical myoclonus, bilateralparietal lobedysfunction andmemory impairment butpreserved language skills. Theclinical diagnosis was ofcorticobasaldegeneration but at necropsy, fouryears aftertheonsetofsymptoms, thepathology was ofAlzheimer's disease together withsome scattered chromatolytic paleneurons inthecerebral cortex. The alienhand signhas not previously beendescribed inAlzheimer's dementiaand isan illustration ofthe clinical heterogeneity thatmay occur in association withAlzheimerhistopathology.
A 68 year old man is described with an alien left hand, cortical myoclonus, bilateral parietal lobe dysfunction and memory impairment but preserved language skills. The clinical diagnosis was of corticobasal degeneration but at necropsy, four years after the onset of symptoms, the pathology was of Alzheimer's disease together with some scattered chromatolytic pale neurons in the cerebral cortex. The alien hand sign has not previously been described in Alzheimer's dementia and is an illustration of the clinical heterogeneity that may occur in association with Alzheimer histopathology.
Three patients with childhood onset symptomatic dystonia responded to levodopa. None fulfilled criteria for a diagnosis of "dopa responsive dystonia" (Segawa's disease). One may have had athetoid cerebral palsy for almost 25 years. All obtained dramatic and sustained benefit from levodopa therapy. A therapeutic trial of levodopa is advised in all patients in whom dystonia has developed in childhood or early adult life, regardless of suspected aetiology or duration of symptoms.
Journal Article Effects of neonatal administration of capsaicin on nociceptive thresholds in the mouse and rat Get access A G Hayes, A G Hayes Pharmacology Department, Glaxo Group Research Ltd., Greenford Road, Greenford, Middlesex, UB6 0HE Search for other works by this author on: Oxford Academic Google Scholar J W Scadding, J W Scadding MRC Cerebral Functions Group, Department of Anatomy, University College, London WC1 6BT, UK Search for other works by this author on: Oxford Academic Google Scholar M Skingle, M Skingle Pharmacology Department, Glaxo Group Research Ltd., Greenford Road, Greenford, Middlesex, UB6 0HE Search for other works by this author on: Oxford Academic Google Scholar M B Tyers M B Tyers Pharmacology Department, Glaxo Group Research Ltd., Greenford Road, Greenford, Middlesex, UB6 0HE Search for other works by this author on: Oxford Academic Google Scholar Journal of Pharmacy and Pharmacology, Volume 33, Issue 1, September 1981, Pages 183–185, https://doi.org/10.1111/j.2042-7158.1981.tb13748.x Published: 12 April 2011 Article history Received: 17 November 1980 Published: 12 April 2011