My Broken Brain, Barcelona, Spain
To have an unknown autoimmune neurological disease is a bad thing for a neurologist. This is just what happened to me nine years ago, when I was 48 years old.
After a prodromic prolonged phase of chills, malaise and low grade fever I became the victim of a disorganized thinking, as in “The Wizard of Oz”, and began to be confused, agitated and experience paranoid delusions. First my own hospital carried out a full work-up, which included physical and complete neurological examination, spinal tap, CT head and body scan, brain MRI, paraneoplastic and autoimmune antibodies. Cerebrospinal fluid exam yielded only one abnormality, a slight pleocytosis. The rest was otherwise normal.
Due to agitation I was placed on a neuroleptic schedule with Haldol but things worsened, my colleagues needed escalating doses of drugs to control my behaviour and, finally, transferred me to another hospital with full inward psychiatric capabilities.
My agitation rapidly got worse. Due to my fluctuating level of consciousness, doctors began to point out to an organic cause of my symptoms, again repeating ancillary tests, adding a whole body PET scan, testicular examination, EEG, second spinal tap and a full virologic, bacteriologic and immunologic lab exam. I was examined by a miriad of internists, neurologists and psychiatrist. A neuropsychological exam showed difficulties in both naming (accordeon – piano folding, tent – plastic house) and attention in a fronto subcortical pattern.
Due to my state, and no response to neuroleptics, even electroconvulsive therapy was contemplated. Finally I gained partial control of the symptomatology and transferred to a long-term care psychiatric facility.
With neuroleptics, lithium, antidepressants and benzos I could just manage to return to work, as a neurohospitalist, a year after the onset of my disease. This was in January 2007 and as I became invoved with patients and the daily activity of the hospital ward, I began to regain useful eyesight, memory for diseases, pattern recognition and judgement on treatments for neurological inpatients and outpatients.
But inside me there was a great hole, the spectre of a waste land, a desert. Something very difficult to define, had gone forever. I was apparently normal, though sleepy and slow and with a ravage filling my inside.
Time is the best healer and I progressively felt better although still taking a lot of pills, as the final diagnosis given was that of bipolar or schizoaffective disorder (“otherwise not specified”).
When in the spring of 2009 Dr. Josep Dalmau came to Barcelona to give a speech on “Synaptic autoimmunity in the brain” and explained his research and clinical experience with NMDAr encephalitis, I realized inmediately what had been going on with me for three years ago (1). In fact I had actually written, on a piece of torn paper in those terrible days, what I believed my diagnosis to be: “limbic encephalitis due to ticks”, thinking of borrelia but pointing to the true origin of my illness. This observation was lost amidst mountains of disorganized papers I left at home before being admitted to the psychiatric ward.
In the next couple of years I continued living normaly and doing business as usual, with small doses of lithium, quetiapine and topiramate which finally I gave up on my own. Each day I thought, as I do now, of how serious my disease was, the big crisis, the delusions, the broken brain, the possibility of becoming dependent on others or even death. As life continued I got engaged and married to Elsa, a neurology resident from my department. Blanca, my fourth daughter, was born in March 2010.
A year after, together with the earthquake and tsunami in Japan, that caused the destruction of Fukushima nuclear plant, I got experienced, on a unforgettable night, a transmural inferior myocardial infarction and a burst of “fire in my brain”, as the journalist Susannah Cahalan has described her disease in a book published a few years ago.
Things were different this time. Primary angioplasty restored my flow through a big thrombus in the right coronary artery and two mililiters of my cerebrospinal fluid travelled from Barcelona to the Hospital of the University of Pennsylvania. In a few days I finally received the label of NMDAr encephalitis sufferer and began inmediately treatment with intravenous corticosteroids. The recovery was spectacular and in less than two months I was back working full time at my hospital, attending strokes, seizures, headaches and even diagnosing one difficult patient with my same disease.
I was thinking constantly on being the subject of a bizarre experiment of nature and neurology, having the honor to be the first and only person in the world being a neurologist and having had a neurological disease not yet described in my first bout of psychiatric symptoms in 2006.
Life has been placid again until recently, September 2014, when symptoms reappeared. I began to fabulate again, being completely insomniac, seeing cockroaches in my room and behaving aggressively.
Again prednisone, this time with rituximab, and here I am, writing this paper and also a book, to relate the strange case of Dr. Escudero, neurologist from Barcelona with two times recurrent NMDAr encephalitis. This presentation (male, adult, recurrent) is not the usual pattern in this disease in which 50% are young girls with ovarian teratoma and bizarre body movements and behaviour problems which, sometimes, call to mind or can be confused by those of the girl of the film “The exorcist.”
Looking for very rare disease, as Dr. House does in my usual practice as neurologist, I never could have guessed that this would happen to me an even less in the way it happened. My brain began to burn, it did so three times at least, and nobody knew, at the beginning, where the fire came from, or knows at this moment if it will burst again.
As Fitzgerald in ‘The Great Gatsby’ says: “So we beat on, boats against the current, borne back ceaselessly into the past.”