20 striking examples of the curious and the unusual in neurology

Neurology is an extraordinary specialty. This is because it deals with the most complex organ, the brain. Neurologists therefore come up against very odd symptoms, and diagnose the most unusual syndromes. Below are 20 curious neurochecklists which reflect this peculiar feature of neurology. AEROPLANE HEADACHE BATHING HEADACHE BURNING MOUTH SYNDROME CRYING EPILEPSY EXPLODING HEAD SYNDROME FUGUE IMPAIRED FACIAL RECOGNITION OF EMOTIONS MIRROR […]

via 20 striking examples of the curious and the unusual in neurology — Neurochecklists Updates

Migraine and the challenge of white matter lesions in the brain

Neurologists often refer their patients with headache for a brain MRI scan. Quite often the reason for this is to reassure their patients who are worried about a sinister cause for their headache…and the anxiety provoking culprit is usually a brain tumour. The headache is often a migraine which has recently changed in character, or which is defying conventional treatment.

The neurologist is often ambivalent when requesting such scans. On the one hand, she expects the scan to be normal. On the other hand, she can not be certain there is indeed no sinister cause for the headaches. Another thing also bothers the neurologist, beyond the chance of detecting a brain tumour. And this is the ‘risk’ that the brain scan detects ‘incidental’ findings called white matter lesions (WML). Alas, these reassurograms frequently pick up these less sinister, but nevertheless unexplained, findings.

By Xavier Gigandet et. al. - Gigandet X, Hagmann P, Kurant M, Cammoun L, Meuli R, et al. (2008) Estimating the Confidence Level of White Matter Connections Obtained with MRI Tractography. PLoS ONE 3(12): e4006. doi:10.1371/journal.pone.0004006, CC BY 2.5, Link
By Xavier Gigandet et. al. – Gigandet X, Hagmann P, Kurant M, Cammoun L, Meuli R, et al. (2008) Estimating the Confidence Level of White Matter Connections Obtained with MRI Tractography. PLoS ONE 3(12): e4006. doi:10.1371/journal.pone.0004006, CC BY 2.5, Link

White matter lesions are often just age-related, ‘wear and tear’ changes, and they are more common in people with vascular risk factors such as hypertension, smoking and raised cholesterol levels. Neurologists generally believe migraine is also a risk factor for white matter lesions. And there are several studies to support this belief.

An example is a paper by the headache gurus Marcelo Bigal and Richard Lipton, published in the journal Cephalalgia, titled migraine as a risk factor for deep brain lesions and cardiovascular disease. Another is a paper by Kruit and colleagues in the Journal of the American Medical Association (JAMA) titled migraine as a risk factor for subclinical brain lesions. If you are still not convinced, try this article in the Archives of Neurology by Swartz and colleagues, with the unequivocal title-migraine is associated with magnetic resonance imaging white matter abnormalities.

MIGRAINE. aka TMan on Flikr. https://www.flickr.com/photos/rundwolf/331545021
MIGRAINE. aka TMan on Flikr. https://www.flickr.com/photos/rundwolf/331545021

With this strong evidence, neurologists are able to convince themselves there is nothing to these MRI high signal changes in their patients with migraine. No ‘chicken and egg’ philosophical equivocation is entertained. The scans are sometimes discussed at neuroradiology meetings where everybody murmurs ‘migraine white matter lesions’. All doubt dispelled, the neurologist reassures the patient, and hurriedly closes the chapter.

https://pixabay.com/en/book-closed-bookmark-close-section-2159519/

It is therefore with a strong jolt that neurologists read a recent article in the prestigious journal, Brain, greatly upsetting this cosy neurological consensus. In the paper titled migraine with aura and risk of silent brain infarcts and white matter hyperintensities, the authors found no association between migraine and brain white matter lesions. Shocking!

Shocked girl. Bixendro on Flikr. https://www.flickr.com/photos/bixentro/6360922151
Shocked girl. Bixendro on Flikr. https://www.flickr.com/photos/bixentro/6360922151

The authors studied female twin pairs aged between 30–60 years. The twins were  identified through the population-based Danish Twin Registry. The authors compared the MRI scans of the subjects with and without migraine, and found no difference in the frequency of white matter changes between the two groups. They proudly, and disconcertingly, declare that ‘we found no evidence of an association between silent brain infarcts, white matter hyperintensities, and migraine with aura‘.

Migraine with aura. Joana Roja on Flikr. https://www.flickr.com/photos/cats_mom/2761999058
Migraine with aura. Joana Roja on Flikr. https://www.flickr.com/photos/cats_mom/2761999058

Oh dear-what do neurologists tell their patients now? I shudder to think!

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The remarkable disorders of the 12 cranial nerves…and their checklists

Nothing depicts the strange mix of beauty and complexity of the brain as the 12 pairs of cranial nerves. These nerves take off from the three sections of the brainstem and have intriguing names such as the olfactory, the trigeminal, the vestibulochochlear, and the hypoglossal. These magnificent twelve turn and twist, loop and meander, as they find their way to their varied […]

via The remarkable disorders of the 12 cranial nerves…and their checklists — Neurochecklists Updates

What are the drugs in development for the treatment of epilepsy?

Progress report on new antiepileptic drugs: a summary of the Thirteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XIII) Bialer M, Johannessen SI, Levy RH, Perucca E, Tomson T, White HS Epilepsia 2017; doi: 10.1111/epi.13634 (Epub ahead of print) Abstract The Thirteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XIII) took […]

via What are the drugs in development for the treatment of epilepsy? — Neurochecklists Updates

Why is CLIPPERS breaking its shackles to the pons?

CLIPPERS is unusual enough you would think. Nothing to do with barbing and shearing I assure you. CLIPPERS stands for Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. The striking feature of CLIPPERS is inflammation in the pons, the chunky middle part of the brainstem. This distinguishes it from other neurological inflammatory disorders such as multiple sclerosis (MS) and neuromyelitis optica (NMO).

By Images are generated by Life Science Databases(LSDB). - from Anatomography[1] website maintained by Life Science Databases(LSDB).You can get this image through URL below. 次のアドレスからこのファイルで使用している画像を取得できますURL., CC BY-SA 2.1 jp, Link
By Images are generated by Life Science Databases(LSDB). – from Anatomography[1] website maintained by Life Science Databases(LSDB).You can get this image through URL below. 次のアドレスからこのファイルで使用している画像を取得できますURL., CC BY-SA 2.1 jp, Link

CLIPPERS has however now broken loose from its shackles to the pons, and is spreading down into the spinal cord. Sacrilege you might say.

By BruceBlaus - Own work, CC BY 3.0, Link
By BruceBlausOwn work, CC BY 3.0, Link

This disruptive and subvertive action was reported in two prestigious neurology journals. The first paper in the journal, Neurology, is titled CLIPPERS with diffuse white matter and longitudinally extensive spinal cord involvement. The second is reported in the journal, Brain, as CLIPPERS with lesions distributed predominantly in spinal cord.

"What's in a name?" Jack Dorsey on Flikr. https://www.flickr.com/photos/jackdorsey/170257936
“What’s in a name?” Jack Dorsey on Flikr. https://www.flickr.com/photos/jackdorsey/170257936

What is it about neurological inflammatory disorders that makes them so rebellious? Why do they defy convention and disregard their defining features. I discussed a similar phenomenon in my previous blog post titled Why is neuromyelitis optica (NMO) endlessly surprising neurology? NMO refused to play by the rules and was punished by having it’s named changed to NMOSD. Perhaps it’s time for CLIPPERS to suffer the same fate….starting with a shorter acronym perhaps?

 

Check out more on CLIPPERS in Neurochecklists

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