The A–Z of limb girdle muscular dystrophy (LGMD)

The muscular dystrophies are important inherited disorders of muscle. They are characterised by their sheer diversity, their multifaceted presentations, and their complicated diagnostic pathways. Some muscular dystrophies are relatively easy to recognise and diagnose. These include Duchenne’s muscular dystrophy (DMD), facio-scapulo-humeral muscular dystrophy (FSHD), myotonic dystrophy, and Emery Dreifuss muscular dystrophy (EDMD). In contrast, limb girdle muscular […]

via The A – Z of limb girdle muscular dystrophy (LGMD) — Neurochecklists Updates

The emerging links between depression and MND

At first, it seemed like a single drop, but it is quickly turning into a trickle. The first inkling was a study of >1,700 people with motor neurone disease (MND) which was published in the journal Neurology titled Depression in amyotrophic lateral sclerosis. The authors found that depression is a very frequent diagnosis shortly before people are diagnosed with MND.

Von Vincent van Gogh - The Yorck Project: 10.000 Meisterwerke der Malerei. DVD-ROM, 2002. ISBN 3936122202. Distributed by DIRECTMEDIA Publishing GmbH., Gemeinfrei, Link
Von Vincent van Gogh – The Yorck Project: 10.000 Meisterwerke der Malerei. DVD-ROM, 2002. ISBN 3936122202. Distributed by DIRECTMEDIA Publishing GmbH., Gemeinfrei, Link

Surely a coincidence, I thought. A rogue finding, or even an understandable response to illness. My excuses were however debunked by another paper published soon after in the Annals of Neurology. Titled Psychiatric disorders prior to amyotrophic lateral sclerosis, the study found that depression may precede the diagnosis of MND by more than 5 years. The authors also report a high frequency of other psychiatric conditions preceding the diagnosis of MND, such as anxiety and psychosis

Depression. Nils Werner on Flikr. https://www.flickr.com/photos/130721398@N06/25363062843
Depression. Nils Werner on Flikr. https://www.flickr.com/photos/130721398@N06/25363062843

 

And just off the press is this report from Nature Communications titled Genetic correlation between amyotrophic lateral sclerosis and schizophrenia. What do we make of this? Is this just the tip of the iceberg? Surely more studies are needed before any firm conclusions. Perhaps this may lead to some early biomarker that enables neurologists to stop the process of progression to full blown MND. Perhaps.

https://pixabay.com/en/sky-clouds-rays-of-sunshine-414199/
https://pixabay.com/en/sky-clouds-rays-of-sunshine-414199/

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The 43 spinocerebellar ataxias: the complete checklists

Of all the disorders neurologists have to deal with, few have as many subtypes as spinocerebellar ataxia (SCA). Most neurologist are familiar with the more common SCAs-types 1,2,3,6, and 7. When pushed, SCA17 will pop into the mind…just because of its similarity to Huntington’s disease (HD). At the last count, there are 43 different forms of […]

via The 43 spinocerebellar ataxias: the complete checklists — Neurochecklists Updates

More activity on the fringes of gluten neurology

I have dabbled into gluten neurology before with my post gluten neurology-persisting and growing? 

https://pixabay.com/en/wheat-field-wheat-cereals-grain-640960/
https://pixabay.com/en/wheat-field-wheat-cereals-grain-640960/

Prophetic it seems, as I am here forced to revisit the topic because  I came across a few recent interesting reports on the neurology of gluten.

Gluten psychosis

By Vincent van Gogh - bgEuwDxel93-Pg at Google Cultural Institute, zoom level maximum, Public Domain, Link
By Vincent van GoghbgEuwDxel93-Pg at Google Cultural Institute, zoom level maximum, Public Domain, Link

Take this case report from Nutrients titled gluten psychosis: confirmation of a new clinical entity. The article comes with some good references that suggest it will do no harm to check anti-gliadin antibodies in people with unexplained psychosis. I do wonder how one case report would confirm an entity such as gluten psychosis, but there you are.

Gluten-induced visual impairment

By OpenStax College - Anatomy & Physiology, Connexions Web site. http://cnx.org/content/col11496/1.6/, Jun 19, 2013., CC BY 3.0, Link
By OpenStax College – Anatomy & Physiology, Connexions Web site. http://cnx.org/content/col11496/1.6/, Jun 19, 2013., CC BY 3.0, Link

The second item is another case report published in Journal of Neurology titled severe, persistent visual impairment associated with occipital calcification and coeliac disease. The subject of the case report has long-standing coeliac disease and visual impairment. Her brain MRI scan showed calcifications in the visual area, evidence the authors claim, of celiac disease causing brain calcifications …..and thereby causing the patients visual loss. Is it just a case of correlation rather than causation? But there you are.

Gluten-induced motor neurone disease (MND)

Multiphoton microscopy of mouse motor neurons. ZEISS microscopy on Flikr. https://www.flickr.com/photos/zeissmicro/12174353194
Multiphoton microscopy of mouse motor neurons. ZEISS microscopy on Flikr. https://www.flickr.com/photos/zeissmicro/12174353194

The third report however pushes credulity to the limits. It is a review in Brain Blogger titled celiac disease and amyotrophic lateral sclerosis-is there a link? To its credit, the piece is heavily referenced; one such reference is from the American Journal of Neuroradiology  titled White Matter Lesions Suggestive of Amyotrophic Lateral Sclerosis Attributed to Celiac Disease. The thought is hard to bear, but there you are.

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By Photograph by Pdeitiker - Transferred from en.wikipedia to Commons., Public Domain, Link
By Photograph by Pdeitiker – Transferred from en.wikipedia to Commons., Public Domain, Link

What are your thoughts on the neurology of gluten? Please leave a comment

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The 29 proven anti-epilepsy drugs…and their practical checklists

Epilepsy is a big problem for Neurology. It is a common, and often life-long, disease. Epilepsy is like the mythical hydra in its diverse manifestations, and in its duplicitous evasion of treatment. The pillars of epilepsy treatment are the anti-epileptic drugs (AEDs). It is however a very tricky business to wield these very powerful tools. For example, one epilepsy medication […]

via The 29 proven anti-epilepsy drugs…and their practical checklists — Neurochecklists Updates