Recurrent ADEM - my experience

Good evening to all. This is my first post here, I hope it can be of some use or, perhaps, help to someone.

I had a first ADEM episode in february 2009, when I was 42 years old, after a (presumed) flu episode. The symptoms were light dizzyness, head ache (localized in the "top" of the head - for me a significant warning, at this point), weakness and the progressive loss of visual capacity. The main symptons, anyway, were tiredness, general weakness and the progressive loss of sight.
It was the loss of vision that brought, after a series of inconclusive visits to various ophtalmologists while my sight was fading, that at the end brought me to a neurologist visit in an hospital; this happened whan my right eye was blind, and, finally, the inflammation of the optical nerve arrived to the end of it, allowing the ophtalmologist to see the inflammation of the optic nerves, and start the action. The MRI showed a complex set of lesions, mostly in the white matter and brain stem. Seen from a non expert, my brain had two "islands" the shape and dimensions of apple slices, surrounded by a constellation of a dozen smaller lesions (about 1cm) like a cloud in my brain. All of them, when they injected the contrast, sparkled like a Christmas tree.

The short term therapy was a set of three intravenous infusions of solu-medrol (methylprednisolone) 1000mg each, followed by four weeks of prednisone (as anti inflammation + immunosuppressant drug) that tamed the ADEM (in that moment the doctors were not sure if it was Multiple Sclerosis or ADEM). The sight was gone in the right eye, damaged in the left one (still functional, but somehow blurred).

The cure for the optic nerve damage was made of eight retrobulbar cortisone injections, that were extremely effective (if dangerous). I want to remark that this is not the "standard" protocol for optic neuritis, at best you can have one or two retrobulbars. My good luck was that a friend, and former high school fellow, is an ophtalmologist surgeon, so, when I told him the story and he saw the situation he did the series of injections (1day+2days of pause in series), and this, to tell it all, started a recovery of the sight, brought back at about 100% in the "good" (left) eye, and 80% in the "blinded" right eye.

After the cortison therapy, i folowed a "recovery" therapy given by the same friend ophtalmolgist, that was based upon series of 9 intramuscular injections of citicoline (1000 mg vials) - notice that this drug is available in Italy without prescription, and approved in Europe for the treatment of some brain damages.

Each series of 9 was followed by intervals of some days - 4 to 7 - and repeated again and again. After about eight months of this treatment, I had recovered 100% both eyes sight.

Part 2 - Recurrency and some comments.

Now, ADEM is a rare disease, it is very rare in adults, and it is typically monophasic. In my case it is not. And it has not evolved into Multiple Sclerosis.

In late july 2013 I was hit, when at work, by excruciating pain at the back, legs and arms muscles, and headache. I drove back home and went to bed. I was out of order and worsening for about a week, during which I experienced, amplified, the initial symptoms of the first ADEM episode, and many others. In first I was lethargic, with extreme fatigue and drowsiness, prolonged sleep, continuous dizzyness, light fever, lack of coordination in legs and arms. I asked for medical assistance to my family doctor, that immediiately ordered a series of blood analyses, and begun to follow me with attention (and very haigh professional capabilty, I want to remark). After about a week a new set of symptoms came out, that were, again optic neuritis (progressive weakening of sight in both eyes, this time was the left more heavily attacked), worsening of weakness, vertigo, headache at the top of the head (this, together to the eyes damage, rang the bell), paresthesia to legs and arms and some mood and behavioural disturbances (had to make an effort to not explode, but never lost self control. perhaps it was simply rage for this event in a string of personal events). At this point I did three things.

1) I talked with my friend ophtalmologist, that performed a control and prepared the syringe.

2) consult with him and my doctor. She, from symptoms, blood analyses, and previous event, diagnosed a recurrency of ADEM

3) i decided to not go to hospital, and, instead, to be cured by them. I had a MRI that showed a picture much worse than the previous one, with the old scars in the brain again active, and a wide set of new ones (included one in the spinal chord). They accepted to cure me, i had one retrobulbar for the optical neuritis (it was enough) and a heavy corticosteroids therapy. 7 intravenous infusion of solumedrol 1000mg (this time they worked, perhaps was needed a massive dose) followed by seven weeks of prednisone 100mg each day, and 3 weeks of tapering.

All went good, but in all honesty I consider myself lucky.

The actual situation is much better than then, if still not 100% OK. Sight with some disturbances (essentially a sort of flash blindeness when I stay too much in the open without sunglasses, or am exposed to internal lights too much strong), occasional short epsodes of light dizzyness, some reduction in stamina, tingling sensation to feet and left hand, and light residual handshake. Still under citicolyne 9+5.

After the therapy I went to a good hospital neurologic centre in Rome, I had rachicentesis (spinal puncture) that showed nothing, as the blood analysis for other autoimmune diseases, and now I have a confirmed diagnosis of recurrent ADEM. There is not prevention therapy, so, essentially, I can (or must) hope that it will not come back in the future.

Actually, I can work (I am an engineer), drive a car, take care of myself, and live a normal life. Well, I think i'll never ride my bike again due to the risk of dizzyness, and my Karate is not as powerful as it used to be :-) , but for the rest I can tell that I made it, thanks to my doctors, that have my forever gratitude.
(And thanks to my mother and my father. Even if i have lost them, i still feel that they kept an hand on me)

Final comments.
1) I DON'T recommend to have a therapy outside an hospital. I did at my own risk because i was convinced that my family doctor and friend (of which I have the highest esteem) and my friend ophtalmologist would have done a better job than a standard hospital protocol (that it was), and because for me and them was not the first time. And, in any case, should i had not any quick improvement, they would had brought me in hospital regardless of my will.
Whatever the case, the one against ADEM is my war, so, the choice on how to fight it and with what allies is mine, as the responsibility and the implied risks.

2) The recurrency of ADEM was for me, and possibly is, much more violent than the first episode. Recurrent ADEM is extremely rare, and it hits the same spots of the brain attacked before, AND new ones. It can quickly evolve into vasculitis, that can become a lethal condition. I came very close to it (shown in MRI). This thing is ferocious, and the second time it was fast. Much faster and aggressive than first time. If not for the experience of the first episode, neither the diagnosis nor the therapy would have been so quick and effective.
3) The recovery is relatively quick in the first three months, slow but still possible for about another year. Citicoline, vitamins B12 and D give a real help, at least in my experience.

4) My D vitamine level before the recurrency was nearly 0, well below the normal. This can have something to do with ADEM, but there is no positive proof.

5) AFAIK, the only therapy are corticosteroids (IV+oral) or, as alternate therapies, plasmapheresis, or IV immunoglobulines. In my case, the corthicosteroids were enough, but I took a really massive dose. luckily - or thanks God, if I am allowed - my body tolerates very high doses of cortisone.
In my humble opinion, to tame ADEM is needeed a really massive dosage, the highest that one can tolerate.

6) For optics neuritis, again in my personal and non professional experience, and taken into account the (mostly negative) experiences of other people that suffered the same condition (all of them with Multiple sclerosis), the only effective therapy is based upon retrobulbar injections of corticosteroids.
A warning: retrobulbar injections are dangerous, the eyeball and the optic nerve can be damaged during the injection (and one risks unrecoverable blindness) and are very painful. Very. And it's not easy to stay immobile during the shoot, nor it's easy to find an ophtalmologist that is competent enough to make it properly, or available to take the risk. Let alone to take it more than once or twice. But it makes the difference between recovery and permanent sight damage.

I hope that this experience of mine can be of some little help. If someone wants to ask, feel free, I'll try to reply at my best, but please take into account that I am not a doctor.

And my apologies for syntax or grammar errors. English is not my mother tongue.
Best regards and my best wishes to all.