This is a follow up to my previous blog titled What are the dreadful autoimmune disorders that plague neurology. Autoimmune neurology is a rapidly evolving field; blink and you will miss important developments. So what’s evolving in autoimmune neurology? Below are my top 4.


1. Insignificance of isolated VGKC positivity

By The original uploader was Iantresman at English Wikipedia - Transferred from en.wikipedia to Commons., CC BY 2.5,
By The original uploader was Iantresman at English Wikipedia – Transferred from en.wikipedia to Commons., CC BY 2.5,

Anti VGKC antibody encephalitis is caused by two different antibodies called LGI1 and Caspr2. The immunology laboratory would however only test for these two if the ‘generic’ VGKC test is positive. Neurologists are understandably left scratching their heads when both tests turn out to be negative. Not any more, going by a report in Neurology titled The relevance of VGKC positivity in the absence of LGI1 and Caspr2 antibodies. The judgment is out: a positive VGCK antibody test is not significant if both LGI1 and Caspr2 are negative. What a relief.

2. IgG4-mediated autoimmune disorders

By Swharden - Own work, CC BY-SA 3.0,
By SwhardenOwn work, CC BY-SA 3.0,

This is a fairly new group of autoimmune disorders consisting of at least 13 different types. They are bad news because they cause many neurological disorders and also ravage other organs. I have previously discussed IgG4 peripheral neuropathy in my post titled What’s looming at the frontline of peripheral neuropathy. The other neurological diseases associated with IgG4 include, surprisingly, myasthenia gravis (MG), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and neuromyotonia. Less familiar IgG4 disorders are encephalopathyhypertrophic pachymeningitis and  sleep disorders with antibody to Iglon5. Trust the researchers to keep the clinicians ever on their toes.

3. GRIN-1 NMDA receptor encephalitis

DNA strand. Mehmet Pinarci on Flikr.
DNA strand. Mehmet Pinarci on Flikr.

Many acquired neurological disorders have a way of dragging genetics into their fold. Such is the case it seems with anti NMDA receptor encephalitis. This is the case with the GRIN-1 gene which codes for an NMDA receptor subunit. Mutations in this gene results in visual impairmentintellectual disability, and eye movement disorders. This is reported in Neurology by Josep Dalmau and colleagues in a paper titled Delineating the GRIN1 phenotypic spectrum. It is appropriate that the authors call this the genetic sibling of NMDA receptor encephalitis.

4. ECT for anti-NMDA receptor encephalitis 

Medcraft B-24 MarkII ECT. Niall Williams on Flikr.
Medcraft B-24 MarkII ECT. Niall Williams on Flikr.

The typical treatment of autoimmune encephalitis revolves around steroids, intravenous immunoglobulins (IVIg), and plasma exchange. Neurologists, when pushed to the wall, may use heavy duty agents such as Rituximab and Cyclophosphamide. Because anti-NMDA receptor encephalitis may be associated with ovarian teratomas, neurologists may make the difficult trip across the border to consult their gynaecology colleagues. I thought these were all the treatment options for anti NMDA receptor encephalitis until I read this case report, again in Neurology, which reported an excellent response to Electroconvulsive therapy in anti-NMDA receptor encephalitis. A no-brainer then if you see neurologists exchanging pleasantries with psychiatrists: they are the ECT experts. It is just a case report for now, but well-worth thinking about when all else fails.



You may check out The Anti NMDA Receptor Encephalitis Foundation which is raising awareness of autoimmune encephalitis.

And here is a recent practical and comprehensive review of anti NMDA encephalitis by Eric Lancaster in the Journal of Clinical Neurology

And indulge me to make another shameless pitch here for neurochecklists which, after all, covers   autoimmune neurology comprehensively!

13 thoughts on “What’s evolving at the cutting-edge of autoimmune neurology?

  1. As always Dr. Ibrahim, this is utterly fascinating and helpful! The links you supply with your posts are very well selected and it’s a pleasure to learn from you, as you take care to keep the viewer involved with that gift you have for science, medicine and a good dollop of ‘average Joe’…keep them coming!


  2. Can you give me some background on Neurosarcoidosis? I was diagnosed with this in 2012 after 5+ years of multiple theories. I became sick in 2006-2008 (?) and underwent MANY treatments and surgeries. My body was TOTALLY PARALYZED because my brain was not functioning. The good news is that I don’t remember most of this. The GREAT news is that I am now WALKING with NO ASSISTANCE and my brain is working again. I would like to know the cause and how this disease progresses.


    1. Thanks Paula. Congrats on an excellent recovery. Neurosarcoidosis is covered in neurochecklists with links to relevant articles. But your neurologist is best placed to advise on your particular circumstance.


  3. Many thanks for this update
    Very interesting particularly the VGKC Ab issue
    Though this makes one of my papers completely useless “i guess”!
    But also leaves out many unanswered question about the diagnosis of these cases (children with clinical phenotype of autoimmune encephalitis, positive VGKC Abs , but negative LGI1 or CASPR2. Not to forget that LGI1 &CASPR2 Abs are almost unheard of in pediatric patients.


  4. Our daughter was diagnosed with Hashimoto’s Encephalitis (another name for Autoimmune Encephalitis with unknown antbody but has a “marker” of autoimmunity with high Thyroidperoxidase) in 2015. She was treated with IV steroids, IVIG, Cellcept with partial response. Rituximab put her into remission and ECT resolved tenacious issues of catatonia, rage, Tourette’s type symptoms. She is now 85% back to baseline and in some respects better than ever. ECT made a huge difference.


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