Friday, August 4, 2017

Autoimmune encephalitis and Lyme


Many patients suffering with Lyme disease also suffer with autoimmune encephalitis. Perhaps this is widely understood. A better understanding of the phenomena may be diagnostically and clinically important. 
The terms Lyme encephalopathy, Lyme encephalitis and neuroborreliosis have been used interchangeably.  Typical symptoms include “brain fog,” memory issues, poor focus and concentration, confusion, disorientation and other forms of cognitive dysfunction or impairment. 

Lyme patients with predominant autoimmune issues have strange experiences which they may find difficult to put into words.  Complaints I hear, include:  strange head pressure (not headache); “brain on fire,” derealization (feel like on a different planet); sensory overload, brain shuts down; altered visual perceptions; hallucinogenic-like perceptions, experience; severe, strange depression, treatment resistant and many others. These patients may experience a unique world of misery which is otherwise inexplicable and indescribable.  

SPECT and PET brain scans are usually abnormal. 

Lab tests may help.  Anti-GAD-65 antibodies are frequently present.  Anti-dopamine receptor antibodies may be present, currently I get this information through the Cunningham Panel (Moleculera), other labs may offer a less expensive test.  There are a dozen or so known autoantibodies known in patients with autoimmune encephalitis but commercial tests are not available.  Anti NMDA receptor antibodies may be checked but I have not seen a positive in Lyme patients. 

With antibiotic therapy there may be a dramatic response: they get much worse.  This is even more the case with therapy directed at Babesia and Bartonella. One recent patient tolerates Rocephin but shuts down (becomes miserable) with the smallest doses of Mepron, Bactrim, Rifampin and Flagyl/Tindamax. 
This is not a Herxheimer reaction.  This is the most crucial point of this post.  Continued therapy will not lead to improvement.  A Herxheimer reaction is caused by a mini-cytokine-storm.  It is normal, although exaggerated response to killing germs. This other reaction is caused by exacerbation of an autoimmune response: the production of self-directed antibodies. This is very troublesome and difficult to manage.  

Treatments may include:  IVIG, Plasmapheresis, immunosuppressant drugs and steroids.  Make sure you get the diagnosis right.  

A short trial of high-dose steroids may be therapeutic and diagnostic.  IVIG, dosed 1.5-2 gm/kg may the most practical therapy for the greatest number of patients.  Getting insurance coverage is the main barrier. The primary immunosuppressive therapy is Rituximab.

Antibiotics are required as a mainstay of therapy once the autoimmune process is controlled. 
One patient is fairly well managed with IVIG, antibiotics and Rituximab.  The dogma that Lyme patients should never receive steroids or immunosuppressive agents is not always true.  

Tertiary care specialists will need to be enlisted in many cases.  The disease is very complex and multifaceted.   Something to keep in mind when patients do not respond to standard therapy as expected. 

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