ABSTRACT
Amyloid-ß transmission has been described in patients both with and without iatrogenic Creutzfeldt-Jakob disease; however, there is little information regarding the clinical impact of this acquired amyloid-ß pathology during life. Here, for the first time, we describe in detail the clinical and neuroimaging findings in 3 patients with early onset symptomatic amyloid-ß cerebral amyloid angiopathy following childhood exposure to cadaveric dura (by neurosurgical grafting in 2 patients and tumor embolization in a third). Our observations provide further in vivo evidence that cerebral amyloid angiopathy might be caused by transmission of amyloid-ß seeds (prions) present in cadaveric dura and have diagnostic relevance for younger patients presenting with suspected cerebral amyloid angiopathy. Ann Neurol 2019; 1-7 ANN NEUROL 2019;85:284-290.
Subject(s)
Amyloid beta-Peptides/metabolism , Cerebral Amyloid Angiopathy/diagnostic imaging , Dura Mater/transplantation , Adult , Age of Onset , Cadaver , Cancer Survivors , Cerebral Amyloid Angiopathy/metabolism , Cerebral Amyloid Angiopathy/pathology , Cerebral Amyloid Angiopathy/physiopathology , Craniotomy , Dura Mater/metabolism , Embolization, Therapeutic , Female , Hemangioma, Cavernous, Central Nervous System/therapy , Humans , Iatrogenic Disease , Magnetic Resonance Imaging , Male , Middle Aged , Papilloma, Choroid Plexus/surgery , Parotid Neoplasms/therapy , Skull Fractures/surgeryABSTRACT
PURPOSE: Anonymised, routinely-collected healthcare data is increasingly being used for epilepsy research. We validated algorithms using general practitioner (GP) primary healthcare records to identify people with epilepsy from anonymised healthcare data within the Secure Anonymised Information Linkage (SAIL) databank in Wales, UK. METHOD: A reference population of 150 people with definite epilepsy and 150 people without epilepsy was ascertained from hospital records and linked to records contained within SAIL (containing GP records for 2.4 million people). We used three different algorithms, using combinations of GP epilepsy diagnosis and anti-epileptic drug (AED) prescription codes, to identify the reference population. RESULTS: Combining diagnosis and AED prescription codes had a sensitivity of 84% (95% ci 77-90) and specificity of 98% (95-100) in identifying people with epilepsy; diagnosis codes alone had a sensitivity of 86% (80-91) and a specificity of 97% (92-99); and AED prescription codes alone achieved a sensitivity of 92% (70-83) and a specificity of 73% (65-80). Using AED codes only was more accurate in children achieving a sensitivity of 88% (75-95) and specificity of 98% (88-100). CONCLUSION: GP epilepsy diagnosis and AED prescription codes can be confidently used to identify people with epilepsy using anonymised healthcare records in Wales, UK.
Subject(s)
Data Collection/methods , Epilepsy/diagnosis , Epilepsy/epidemiology , Adult , Algorithms , Anticonvulsants/therapeutic use , Child , Electronic Health Records/statistics & numerical data , Epilepsy/drug therapy , Female , Humans , Male , Reproducibility of Results , Wales/epidemiologyABSTRACT
Choreoathetoid movements are quite common in cerebral palsy (CP). This is the first report of a patient with choreoathetoid CP who was successfully treated with carbamazepine. Therefore, clinicians should try carbamazepine for involuntary movements in CP patients before pursuing other procudures.
