When Shakespeare meets neurology: Hamlet, Ophelia and autoimmune encephalitis

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.

By Gentaur – Gentaur, Public Domain, Link

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.

By Benjamin WestOwn work, Public Domain, Link

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.

By V from Coventry, UK – Hamlet, CC BY 2.0, Link

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 130 interesting and unusual manifestations of HSP

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 […]

via The 130 interesting and unusual manifestations of HSP — Neurochecklists Updates

Who are on the line-up of the WESAN Exeter Neurology course in 2017?

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. 

Exeter Cathedral timelapse. Joe Dunckley on Flikr. https://www.flickr.com/photos/steinsky/894007623

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.

 

The Rougement Hotel Exeter. Robert Cutts on Flikr. https://www.flickr.com/photos/panr/5542700403

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

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The Neurology of Amnesia

Adam Zeman, Professor of Cognitive Neurology at Exeter 

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Advances in the Diagnosis of Neurogenetic Disorders

Julia Rankin, Consultant Clinical Geneticist at Exeter 

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Emerging Treatable Autoimmune Disorders of the CNS

Sarosh Irani, Associate Professor of Neurology at Oxford 

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Movement Disorders: A Video Presentation

Kailash Bhatia, Professor of Clinical Neurology at Queen Square 

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Cold Injury in the Royal Navy

Paul Hughes, Neurologist at Haywards Heath 

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Hepatitis E and the Nervous System

Harry Dalton, Consultant Hepatologist at Truro

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The mTORopathies-What the Neurologist Needs to Know

Finbarr O’Callaghan, Reader in Paediatric Neuroscience at Great Ormond Street Hospital  

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Refractory Epilepsy: Hope is not a Strategy

Hannah Cock, Professor of Neurology at St George’s Hospital 

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Looking Back on a Career in Neurology – Some Things I Would Probably Have Done Differently

Nick Fletcher, Consultant Neurologist (rtd) at the Walton Centre 

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Ganglioside Antibodies and the Landscape of Immune Neuropathies

Hugh Willison, Professor of Neurology at Glasgow 

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Metabolic Muscle Diseases

Jon Walters, Consultant Neurologist at Swansea 

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MS: Emerging Treatments and Treatment of Progressive Disease

Claire Rice, Consultant Neurologist at Bristol  

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You may register for WESAN Exeter now

Follow WESAN on twitter: @wesanexeter

By FranzfotoOwn work, CC BY-SA 3.0, Link