Primary angiitis of the CNS: unusual presentations of a rare and dangerous disorder

Primary angiitis of the central nervous system (PACNS) is inflammation of the blood vessels of the central nervous system (stating the obvious you might say). It differs from other forms of angiitis or vasculitis, such as lupus and giant cell arteritis (GCA), which respect no boundaries. PACNS is as dangerous a neurological disorder as they come, and just as rare. It requires aggressive, and paradoxically equally life-threatening, immunosuppressive treatment. Between the devil and deep blue sea-that’s exactly where the neurologist managing a patient with PACNS will be found.

BRAINADE! the Brain Grenade. Emilio Garcia on Flikr. https://www.flickr.com/photos/lapolab/11929014084
BRAINADE! the Brain Grenade. Emilio Garcia on Flikr. https://www.flickr.com/photos/lapolab/11929014084

The clinical features of PACNS are unfortunately very non-specific and include headaches, seizures, stroke, and cognitive changes. This makes PACNS is a challenge to diagnose. Even when suspected, PACNS may evade detection even by the special scan of the blood vessels called angiography. More frequently, the only certain way of confirming this disease in life is with a brain biopsy. Did I say ‘certain’? I take that back. Alas, even brain biopsy is not guaranteed to make the diagnosis of PACNS. A high degree of confidence and teeth-gritting is therefore an absolute requirement in any neurologist unfortunate enough to come face-to-face with this menace.

By The original uploader was Glitzy queen00 at English Wikipedia - Transferred from en.wikipedia to Commons., CC BY-SA 3.0, Link
By The original uploader was Glitzy queen00 at English Wikipedia – Transferred from en.wikipedia to Commons., CC BY-SA 3.0, Link

To make complicated matters even worse for the unwary neurologist, there are now reports suggesting that PACNS presents in even rarer and atypical ways. For the neurological Sherlocks and Poirots, here are 2 unusual presentations of PACNS.

Isolated spinal cord involvement

Spinal Cord 2. Green Flames 09 on Flikr. https://www.flickr.com/photos/greenflames09/116396742
Spinal Cord 2. Green Flames 09 on Flikr. https://www.flickr.com/photos/greenflames09/116396742

This is a case report from the Journal of Neurology of a 44-year old woman who presented with PACNS but with purely spinal cord involvement and completely sparing the brain. The diagnosis in this case was only confirmed with a spinal cord biopsy. The authors reviewed the literature and only found 8 previous reports of PACNS beginning in the spinal cord, and half of these progressed to involve the brain. 

Unilateral cerebral presentation

Keep Left. Howard Lake on Flikr. https://www.flickr.com/photos/howardlake/4440588147
Keep Left. Howard Lake on Flikr. https://www.flickr.com/photos/howardlake/4440588147

Most cases of PACNS evenly involve both sides of the brain. This report, again from Journal of Neurology, bucks this trend with the report of a 55-year old man who had PACNS which only involved the left side of his brain. This unilateral hemispheric PACNS is a reminder that an entity called focal PACNS exists.

https://pixabay.com/en/light-bulb-brain-absorbed-light-1599359/
https://pixabay.com/en/light-bulb-brain-absorbed-light-1599359/

Do you have any sightings of unusual cases of PACNS? Please drop a comment

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The art of spinning catchy titles

How often is one turned off by a paper with a very convoluted or poorly worded title. One example I came across is The dangerousness of persons with the Othello syndrome. There are many other examples out there.

Fair use, https://en.wikipedia.org/w/index.php?curid=1629138
Fair use, https://en.wikipedia.org/w/index.php?curid=1629138

 

The focus here is however on articles with titles that not only reflect the topic, but play wonderfully with the words. This paper from Neurology is a classical example: Normal pressure hydrocephalus: how often does the diagnosis hold water?

 

By Vimont, Engelmann /Scan by NLM - National Library of Medicine (Call No. BF V765t 1835 OV2), Public Domain, https://commons.wikimedia.org/w/index.php?curid=3750904
By Vimont, Engelmann /Scan by NLM – National Library of Medicine (Call No. BF V765t 1835 OV2), Public Domain, https://commons.wikimedia.org/w/index.php?curid=3750904

 

What about this catchy title on absence epilepsy from Epilepsy Currents– The current state of absence epilepsy: can we have your attention?

 

What about this, alluding to the energy production role of mitochondria, from the Journal of Internal Medicine:  Batteries not included: diagnosis and management of mitochondrial disease. Surely alluding to the energy-generating function of mitochondria.

Vintage Arizona State University mitochondrial model. Gregory Han on Flikr. https://www.flickr.com/photos/typefiend/6819392279
Vintage Arizona State University mitochondrial model. Gregory Han on Flikr. https://www.flickr.com/photos/typefiend/6819392279

And this one is from Acta Neuropathologica Communications titled The prion hypothesis in Parkinson’s disease: Braak to the future. This is a reference to the Braak hypothesis which describes the spread of Parkinson’s disease pathology across the brain over time. Could prion diseases be responsible for Parkinson’s disease? For a clue, see my blog post, What are the links between prion diseases and parkinsonian disorders.

By Visanji, Naomi P., Patricia L. Brooks, Lili-Naz Hazrati, and Anthony E. Lang. - Visanji, Naomi P., Patricia L. Brooks, Lili-Naz Hazrati, and Anthony E. Lang. “The Prion Hypothesis in Parkinson’s Disease: Braak to the Future.” Acta Neuropathologica Communications 1, no. 1 (May 8, 2013): 2. doi:10.1186/2051-5960-1-2. http://www.actaneurocomms.org/content/1/1/2., CC BY 2.5, Link
By Visanji, Naomi P., Patricia L. Brooks, Lili-Naz Hazrati, and Anthony E. Lang. – Visanji, Naomi P., Patricia L. Brooks, Lili-Naz Hazrati, and Anthony E. Lang. “The Prion Hypothesis in Parkinson’s Disease: Braak to the Future.” Acta Neuropathologica Communications 1, no. 1 (May 8, 2013): 2. doi:10.1186/2051-5960-1-2. http://www.actaneurocomms.org/content/1/1/2., CC BY 2.5, Link
And from the journal Neurology again comes Blowing the whistle on sports concussions: will the risk of dementia change the game?  This, of course, is to do with the increasing recognition that repeated head injuries in athletes result in chronic traumatic encephalopathy. But is the sporting listening? You may wish to revisit my previous blog post on this, Will Smith and chronic traumatic encephalopathy.
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And from Brain comes this commentary titled Seizure prediction: making mileage on the long and winding road. It is not yet open access, and the synopsis doesn’t let the cat out of the bag. It is difficult therefore to establish what links the catchy title to the text. But it is still a work of art.
landscape-690588_1920

And finally, from Muscle and Nerve, comes Small fiber neuropathy: getting bigger! This is a review article highlighting the growing problem of a disorder with a self-deprecating name. Time to take notice!

By Dan Bennett - Flickr: marmaduke, CC BY 2.0, Link
By Dan BennettFlickr: marmaduke, CC BY 2.0, Link

Perhaps you have a few examples of your own to share.

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