Treatable, If Identified
VGKC Autoimmune Encephalitis
By A. G. Moore 8/20/2013
I am returning in this post to a topic addressed on July 22, 2013. I come back to the subject because I’ve learned more since then and what I’ve learned might help someone, somewhere. And I’m alerting the lupus community because this syndrome,–autoimmune VGKC, or limbic, encephalitis–occurs most often in families where at least one person (a first-degree relative) has an autoimmune disease.
About four months ago, in early April, a member of my family became ill. At the time we didn’t know how serious this illness would be. All we knew was that its origins were not understood and that it was getting worse every day. First the happy ending : recovery is now in progress and the prognosis is good.
An amazing aspect to this story is: once doctors recognized what was going on, an effective treatment was available. A sobering aspect to the story is this: there was a good chance that doctors would never know what was going on. This disorder has been described in the literature only over the last ten years and new information about it is coming out all the time.
Although it is believed that VGKC (voltage-gated potassium channel) autoimmune encephalitis is very rare, no one knows for sure how often this disorder occurs–because of misdiagnosis. Which is why I’m writing this post; even if a doctor isn’t able to form a cohesive clinical picture from the syndrome’s disparate symptoms, maybe a family member will notice some of the hallmark signs.
The disease has classic neurological symptoms. These include disorientation, amnesia, seizures, movment disorder and dementia–although all of these may not appear in the same person, nor may they occur in any predictable order. One thing that seems to be common is rapid onset. In this scenario, doctors first suspect an environmental antagonist, such as a virus, bacteria, parasite or fungus. When all of these usual suspects are discounted, less likely causes will be investigated.
In the case of VGKC autoimmune encephalitis, a hallmark symptom is low sodium, sometimes referred to as SIADH. SIADH, in conjunction with classic neurological symptoms and a family history of autoimmune disease, may suggest the possiblity of autoimmne encephalitis (see: Autoimmune Encephalitis–New Awareness, Challenging Questions)
Some of the tools used to arrive at a firm diagnosis are: EEG and MRI studies; tests for specific autoantibodies in serum; and a spinal tap to look, once again, for autoantibodies. If the autoantibodies are present, the doctor can be confident in the diagnosis and the outcome of treatment is likely to be good. Even in the absence of autoantibodies, however, good outcomes have been realized after initiation of immune suppressive therapy. One important thing to remember is that delayed treatment may compromise the outcome.
Research on autoimmune encephalitis is so new that doctors are still looking for answers. Therefore, I obviously cannot, as a non-scientist, offer more than the most cursory introduction to the subject. What I can do though, is alert the community to the fact that this disease exists and that doctors may not detect it. I can also describe, in the broadest terms, symptoms that are characteristic of the disorder. Most importantly, I can repeat what I have stated so many times before: go to the smartest doctors, doctors who are conversant with the latest research. In the case of my relative, the change in fortune came with a change of doctor. The right person saw something, performed the appropriate tests and began an effective treatment plan.
My story has a happy ending; I hope the information in this blog helps to bring the same ending to someone who is confronted with a similar issue.
Two excellent article on this subject: