11/28/2025
For CASPR2-antibody autoimmune encephalitis that is not responding adequately to intravenous methylprednisolone, the most effective second-line therapies are intravenous immunoglobulin (IVIg) or plasma exchange (PLEX). However; plasmapherisis is painful, complicated and comes with high risk of infection. These treatments are used in addition to or in place of steroids for more intensive immunosuppression.
The presence of CASPR2 antibodies in the blood serum but not in the cerebrospinal fluid (CSF) indicates that the condition may primarily affect the peripheral nervous system, although central nervous system symptoms are still common. The treatment approach, however, remains largely the same as for other forms of CASPR2-associated disorders.
First-line therapies beyond methylprednisolone
After IV methylprednisolone, the next standard interventions are typically:
Intravenous Immunoglobulin (IVIg): This treatment involves administering a concentrated dose of healthy antibodies to help neutralize the patient's autoantibodies. IVIg is often used in combination with corticosteroids as a first-line treatment.
Plasma Exchange (PLEX): This procedure involves removing the patient's blood plasma, which contains the harmful antibodies, and replacing it with a substitute fluid. PLEX is an effective method for rapidly clearing autoantibodies from the circulation.
Second-line and refractory therapies
If symptoms persist or worsen despite first-line therapies (corticosteroids, IVIg, and/or PLEX), doctors may consider more aggressive second-line options, which is why the Dr & I are simply trying to go straight to Rituximab. If not then I'll end up having plasmapherisis done at the next flare up. As Morvan's absolutely disrupts every aspect of my life. Currently dealing with active autoimmune encephalitis. Autoimmune friend limbic is taking the day off for good measure. 😆 Yes if you read enough, you'll notice my sarcasm. 10/10 don't recommend being so rare you have to teach Drs lol As a female there is less than 5/6 of us documented with Morvan's since the first case in 1860. TOTAL 108 ever documented. Although it is fascinating to learn about, not going to hold ya. It's absolutely h*ll to live with.
Anyway- Pray I am able to begin Rituximab vs more flare ups. Id like to avoid plasmapherisis if possible, but am okay with getting it if need.
I love life, being a mom. An even better mom than ever, as I strive daily to learn from my lessons in life. It'll slowly work out. Just takes time and adjustment.
Hard when I want to scream I friggin told you so, to so many people, from professionals, to friends, to enemies and family. As I've always been open and honest about being sick, they thought for the longest time it had to be MS etc etc. I know everyone remembers.
Whelp... it altered who I was and how Id act, what id accept. In ways I would have never been or accepted. I just wish people would have taken the time to listen before now. Especially Medical Community, so that I could have began treatment long before now. Like, I should have never had treatments stop. Over 2 decades of missed treatments, created permanent damage. Which has created chronic severe pain amung other issues. All chronic. Still beyond blessed to be alive. My brain is healing and I do feel more like my self mentally & emotionally. Which is a beautiful feeling. It's been WAY to long since I've felt like myself. My youngest definitely gets ask the rewards & hard lessons learned that his siblings did not. That's how I know I'm a great mom. I learn from my mistakes. Ask my youngest how different I am, in SO MANY GOOD WAYS since beginning ANY treatment for this disorder. Imagine how much better that gets with the correct treatment!? I've lived with this for 2 decades, I'll take 3 more decades on planet earth, please. 😆 No really, I'll stick to passing at 112 after we find a cure, build a wing at ecmc for extensive research starting with Morvan's and Casp2/anticasp2 & all forms of encephalitis. I think as patient & medical team we should link up and really put some thought into this. Yall are a teaching hospital, what better place to find a needle in a haystack remission and/or cure remedy. We can expand from there, told yall, I never lied about my health or how it affects every aspect of my life. At this point pain & suffering × about 30 people's pain. My family never deserved the pain or trauma that a double- blind study caused us. Caused them. Hindsight 20/20 If I knew what I know now, I would never have agreed. As this disorder is life- threatening and life-debilitating. These type of double blind studies are literally playing rxssi@n roulette with the lives of the unsuspecting participants. is where it is at!
Rituximab: This medication is a monoclonal antibody that targets and depletes B-cells, which are the immune cells that produce antibodies. It is often used for patients who do not respond to initial therapies or for long-term immunosuppression to prevent relapse.
Cyclophosphamide: This potent immunosuppressant is another second-line option for more severe, refractory cases. It is an alkylating agent that targets rapidly dividing immune cells.
Additional considerations
Underlying malignancy: CASPR2 autoimmunity can sometimes be a "paraneoplastic syndrome," meaning it is triggered by an underlying tumor, most commonly a thymoma. A diagnostic workup for potential malignancies is an important part of the management plan, as treating the tumor is essential for a favorable outcome.
Symptomatic management: In addition to immunosuppression, other medications may be needed to manage specific symptoms, such as anti-seizure medications, sleep aids, and mood stabilizers.
https://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/diagnosis-and-management-of-autoimmune-encephalitis #:~:text=First%2Dline%20Therapies:,exchanges%20over%2010%2D14%20days
National Institutes of Health (NIH) | (.gov) https://share.google/78zig8x22pux7uVGl
Autoimmune Encephalitis Alliance https://share.google/cYDMBR1YoVAI2xcjY
Autoimmune Encephalitis Alliance https://share.google/edFw50GQU0Lf8w9ko
Neurology® Journals https://www.neurology.org/doi/10.1212/NXI.0000000000200041
ARUP Laboratories https://share.google/NBY8w5q9mZTIyheyH
National Institutes of Health (NIH) | (.gov) https://share.google/amy6syjMZ5xk1dfzC
https://www.thieme-connect.com/products/ejournals/html/10.1055/s-0045-1801860?device=mobile&innerWidth=384&offsetWidth=384
