My 5 year old daughter case of Anti NMDA
Arani Marquez Montufar - Monday, June 20, 2011
Everything began end of March 2011 when she complained about a tingling on her right foot. After a week she began to lose her balance and her toes were moving. That Sunday we decided to bring her to emergency to our local children`s hospital. After doing some testing, they told us she was going to be admitted for a few days to have an MRI and some other tests. My heart sank, they said not to worry that probably it was Sydenham Chorea, a rare illness but treatable and it would go away in a few months. How wrong they were.
After many blood, MRI, EEG, Electrocardiogram, Strep tests and many others, they have found no traces of strep which is one of the confirmations for that disease, still they kept the diagnoses and she was discharged 5 days later with a dose of Penicillin daily, Valproic Acid for the movements.
That weekend was terrible. She seemed very sleepy during the day, but she could not sleep at all at night; actually this started at the hospital but at home it got worse. She could not walk any longer, the movements were awful, she was upset at all times and she had laughing over the top episodes, when the angry ones came, she was very confused and she was saying that me or her dad wanted to hurt her and to stop doing it, she bit us and she was very aggressive, kicking and hitting, she had some odd behaviours like obsessive, pulling down the bottom of her pants and up her socks over and over she did not want to show any skin on her legs.
Next morning we decided to go back to emergency, she was admitted again, they removed the Valproic Acid since they thought it was not helping and put her on 35mg of prednisone, and 2 ml of clonazepan, another neuro doctor came into the picture and put her on Haldol. She was already very agitated and had several angry, biting throwing things, kicking, pulling her hair out and hitting episodes throughout the day and night, she had not sleep at all already three weeks by then. After they started the Haldol, she had drooling, non-responsive, gone kind of spells, they seem like seizure to me without the movements, they would last from 5 to 30 seconds. They increased the Haldol and Clonazepan, they became worse and another neurologist came into the picture, she suspected another autoimmune disease, she wanted to send blood and serum to Calgary and ask for a spinal tap, another MRI and an EGG, she removed Haldol, the weird gone drooling spells disappeared, the movements got somehow better and she began to walk again, slowly she was eating less and less, until to me she look like anorexic, all her clothing was way to big on her.
At one point she began eating again more then ever, I guess Prednisone, she became restless, real anxious she just wanted to walk over and over, go to the hospital book library, bring a pile of books and come back for more, she would just repeat after us, the anger episodes seemed less and less until they were just a couple a day but long in duration.
Little by little she had less severe anger episodes, she would take something to bite instead of biting us, she started to show some kind of remorse if she bit us, at this point a month already at hospital, she was discharged with an IVIG treatment before leaving the hospital May 3 2011.
I gave birth the same night my second baby girl 3 weeks before time, guess due to the stress.
May 15 she had an appt. with her neurologist, and she booked her for 6 more IvIG treatments, results were back finally and she was confirmed she has Anti-NMDA Receptor Encephalitis, so it took about 6 weeks to be finaly diagnosed although she was treated since week second with prednisone and first IVIG 4th week, I do think it make a big difference on her case.
Little by little we would see improvements on Arianne, her movements were more distonic, than choreatic she would have lots of facial tics, and still wanted to bite but she was more in control of it, she started chewing gum instead, she was still repeating after us, she seemed most of times in her little world, and would talk like singing with long syllables and very nasal sound.
Day after day, she became more aware of things, she would respond a bit more and she started to interact a little bit, her tics and movements were less and less bad, she was screaming at one point for a few days, then after this she would answer to questions and to say a few words without repeating after us.
On the next two weeks she had friends over, she reacted amazing, she played, she was laughing and really happy, she was interacting more and more, and day by day she would show some improvement. At one point we could even see our little one almost back ton how she was before all of this for a few minutes.
As of today, June 20 she had another MRI for scanning her belly. No tumors were found as I expected. She has done 7 IVIG so far; the last two she got rashes on all her body.
Her mood changes and is over the top mostly when she is upset which is quiet often these past 3 days – hard to know when she was doing great if is due the meds or the illness.
So far no more improvement although she is back to school (jardin) 3 hours only, don’t want to over- tire her, still lots of movements at nighttime. Some nights are better than others, nightmares came back, although not as bad as before, language is not completely like it used to be, sometime she speaks like a girl lots younger than her and behaves like it as well, and sometimes she seems more mature than even before the illness, do not know what to make of it.
We hope she does not relapse, we have to wait for her doctor to come back since she is traveling next Monday to know what is next.
Praying every day for her and others.
Update June 27
Last IVIg treatment, 12pm now still at hospital, we hope she gets no reaction. Started to lower prednisone from 20mg, 5 mg every week till she is off, clonazepan from 1ml mornings and 2 at night starting tonight 1ml and 1ml. We will follow up in a couple of weeks.
She had a good week, we hope she will continue doing well.
Update July 21
Nearly a month after they are removing Steroids. Many things have returned that have had almost disappeared or were missing like facial tics, body movements are more pronounced now noticeable during the day, headaches, trouble sleeping some nights, pain in feet and stomach. I pray every day.
Tuesday, July 26 we have follow up appt. with neuro. They are now talking about a new drug called Cellcept for 12 months; it is a suppressor of the immune system, we do not like side effects, but it is to put things on a balance or the possibility of a relapse, we have an appointment with a rheumatologist till August 18 whom is supposed to tell us about the drug, I think personally is very far away, since the drug itself takes 2-3 months to have any effect, according to neuro dr. she has a window of 2 to 3 months and it has been six weeks already. We’ll see.
August 24 my dear Arianne is free of all medications except for clonazepam 1ml in the evening. We decided together with her neurologist and rheumatologist not to give her the Cellcept. They will ask Dr. Dalmau who was the the doctor who discovered the disease and treated more cases than any other doctor.
Now everything is fine except she still has movements at night and sometimes cannot sleep well, try to give melatonin to see if it helps her to sleep. In general during the day except when she gets angry a little easier especially when tired, it seems almost like she was before. I keep praying and I am grateful for my beautiful daughters.
Update: March 6 2012
We gave her the melatonin, it works wonders with her sleep. I did remove it a month ago, as she was about 6 months on it, she does not have problems falling asleep now.
In November 2011 we ran to emergency as we thought she was relapsing. Movements got worse, tics came back during the daytime and she had a very angry and agitated episode. They gave her a couple more treatments of IVig, reaction was good overall, but she got severe headaches and vomit after 24 hours for a couple of days, the second time I kept her as hydrated as possible before and after it seemed to diminish her headaches and nausea.
As of today she has been over 6 months on Cellcept, we have not see many improvements. If there have been any, they are very little, she still presents movements at nighttime, jumping muscles, her arms and legs fly sometimes or they jump, her feet flip, and her head moves a bit. She has some calm periods, at least she does not wake up almost ever, so that is an improvement, she has all kinds of tics during day time and the last we just receive her neuropsychological test results and she has excecutive function problems, which means she cannot organize, planify or comprehend many things, emotionally means sometimes she can’t relate to people, feel or care, this problems as the neuropsychologist put it explains a lot of her up and down behaviors.
We will see how she can be helped at school because she basiclly needs one on one help and probably a tutor later on. If it will be reversible, is too soon to know yet, we do hope and keep praying. My new task is to learn as much as I can of this function and to learn strategies and all I can to help her to develop new ways to cope with this.
Overall she is here, she plays, she sings, she is happy she can function at least for now at school for a first grader and basically all her other functions tests, including memory were average which it means she is like anyone else in most things and considering what she has gone through this is remarkable.
March 26 she will have another appointment with neurology at hospital to check her progress and to talk about our concerns, her blood was sent to Calgary another lab in Canada that now test for anti NMDAR a month and a half ago and came back clean, first time had high levels, we know it is no confirmation that it is gone, but we will not put her through a spinal tap unless necessary.
She will probably have to go through an MRI and ultrasounds in a few months since it would be a year since last ones to keep scanning for Teratoma tumours. I will keep updating on her condition, and recovery whenever we have new info.
I hope her story helps someone.
Update January 2014
Getting close to 3 years since onset.
She took Cellcept for 2 years, it was removed, September 2013, with no signs of relapse so far. She is off all meds and her learning at school improved wonders that she is considered now an average kid. I could notice she is not as agile as she used to be before she got ill and she still needs time to procces some information, she needs at least 9 hours of sleep, if not she becomes very upset as any normal kid her age with lack of sleep I guess. She becomes anxious with high emotions. Tics never went away. Her nourologist consider it might not have anything to do with the NMDA, she was diagnosed with Tourettes syndrome this past November 2013.
Overall she is doing fantastic.
Some stomach issues we hope nothing to do with all meds she took, she has been seen by a gastro specialist and they will perform an endoscopy of her upper and lower intestines soon. It will be interesting to see what they find inside, although nerve racking she has to be put to sleep. Prayers and Blessings to all.