Dystonia is perhaps the most complicated neurological disorder. For one, even the term dystonia is difficult to define. It combines fixed postures and abnormal movements, all in one. Terms like twisting and torsion come up regularly in the language of dystonia. Dystonia was therefore a challenge for neurochecklists. But building from the basic elements, neurochecklists […]
Neurology is a precarious specialty. Many neurological disorders are difficult to diagnose, and many neurological treatments are risky. Neurological error also leaves a high burden on patients, often resulting in death or permanent injury. It is therefore not surprising that the frequency and cost of neurological medical malpractice are high. Neurology has the […]
Neurology can’t seem to get away from autoimmune disorders of the central nervous system. This blog has visited this topic several times before such as with the posts titled What are the dreadful autoimmune disorders that plague neurology? and What’s evolving at the cutting-edge of autoimmune neurology? The attraction of autoimmune neurological diseases lies in part in the ever-expanding spectrum of the antibodies and the challenging symptoms and syndromes they produce.
The fairly well-recognised ‘conventional’ antibodies are those against VGKC (Caspr 2 and LGI1), NMDA, and AMPA. There is however an almost endless list of less familiar antibodies such as those against glycine, adenylate kinase 5, thyroid, GABA-A receptors, α-enolase, neurexin-3α, dipeptidyl-peptidase-like protein 6 (DPPX), and myelin oligodendrocyte glycoprotein (MOG). I am however fascinated by the group of disorders caused by antibodies to metabotropic receptors. The main antibody in this group targets the metabotropic glutamate receptor 5 (mGluR5). The clinical picture with this antibody is a form of encephalitis which may manifest with prosopagnosia (difficulty recognising faces), and with the curious Ophelia syndrome.
Yes, you read it correctly. Ophelia syndrome is named after Shakespeare’s unfortunate Danish maiden, and it was first described by Dr. Ian Carr whose daughter, at the age of 15, developed progressive loss of memory, depression, hallucinations, and bizarre behaviour. These symptoms aptly describe Ophelia’s deluded and obsessional attraction to the equally deluded and murderous Hamlet. Ophelia syndrome is almost always associated with Hodgkins lymphoma and affects young people.
Thankfully Ophelia syndrome is a relatively mild disease without the Shakespearean tragic ending because it has a good outcome if recognised and treated.
Why not explore all the autoimmune neurological disorders on neurochecklists.
The hereditary spastic paraplegias (HSP) are a diverse group of inherited disorders of the spinal cord. They may be transmitted dominantly or recessively, and through the X Chromosome. There are, at the last count, 79 different genetic forms of HSP. These are titled, rather confusingly, SPG1 to SPG79. Some forms of HSP occur more frequently than others. SPG4 […]
The West of England Seminars in Advanced Neurology (WESAN) is an annual course convened by Neurologists at the Royal Devon and Exeter Hospital. You may check out the previous programmes listed in the blog post tilted What has the Exeter Neurology Course accomplished in the last decade. You may also learn more about WESAN in the blog post titled A short history of WESAN.
The WESAN programme this year, as usual, is an exquisite balance of topics covering the spectrum of neurological specialisms. The agenda is a fine blend of new and old topics, delivered by experts at the cutting edge of their fields.
So what has WESAN lined up for 2017? Here are the topics and speakers
Functional Neurological Disorders-The Assessment as Treatment
Jon Stone, Professor of Functional Neurology at Edinburgh
The Neurology of Amnesia
Adam Zeman, Professor of Cognitive Neurology at Exeter
Advances in the Diagnosis of Neurogenetic Disorders
Julia Rankin, Consultant Clinical Geneticist at Exeter
Emerging Treatable Autoimmune Disorders of the CNS
Sarosh Irani, Associate Professor of Neurology at Oxford
Movement Disorders: A Video Presentation
Kailash Bhatia, Professor of Clinical Neurology at Queen Square
Cold Injury in the Royal Navy
Paul Hughes, Neurologist at Haywards Heath
Hepatitis E and the Nervous System
Harry Dalton, Consultant Hepatologist at Truro
The mTORopathies-What the Neurologist Needs to Know
Finbarr O’Callaghan, Reader in Paediatric Neuroscience at Great Ormond Street Hospital
Refractory Epilepsy: Hope is not a Strategy
Hannah Cock, Professor of Neurology at St George’s Hospital
Looking Back on a Career in Neurology – Some Things I Would Probably Have Done Differently
Nick Fletcher, Consultant Neurologist (rtd) at the Walton Centre
Ganglioside Antibodies and the Landscape of Immune Neuropathies
Hugh Willison, Professor of Neurology at Glasgow
Metabolic Muscle Diseases
Jon Walters, Consultant Neurologist at Swansea
MS: Emerging Treatments and Treatment of Progressive Disease
Claire Rice, Consultant Neurologist at Bristol
You may register for WESAN Exeter now
Follow WESAN on twitter: @wesanexeter
Vascular risk factors in INPH: a prospective case-control study (the INPH-CRasH study) Israelsson H, Carlberg B, Wikkelsö C, et al Neurology 2017; 88:577-585 Abstract OBJECTIVE: To assess the complete vascular risk factor (VRF) profile of idiopathic normal pressure hydrocephalus (INPH) using a large sample of representative patients with INPH and population-based controls to determine the […]