Sunday, February 9, 2020

Anti-NMDAR or Autoimmune Encephalitis:$1 Million Investigation:

In the new world of AQPs, should CCGs be paying for doctors that did all kinds of investigations and still did not know what was wrong with you? What if your illness is so new that very few of the new breed of tick-box doctors know nothing about?

As we are now suppose to follow guidelines and protocols, where would new disease come in? Not that long ago, Legionnaires was declared by a psychiatrist as mass hysteria until he also caught it. Would we now be able to discover Helicobacter pylori without some maverick experimenting on himself!

Hospital Medicine has its place in the medical world and most Bright Young Things are after the Pride in world class medicine.

So, in my recent research on Anti-NMDA Receptor Encephalitis, I came across a book by Susannah Cahalan. In Hospital, her doctors did loads of tests and investigations but could not find out what was wrong with her. The cost mounts up and her insurance had to pay for these negative investigations. Luckily she worked for NY Post and her insurance covered most of the fees.

NPR:

A Young Reporter Chronicles Her 'Brain On Fire'

November 14, 201211:00 AM

In 2009, Susannah Cahalan was a healthy 24-year-old reporter for the New York Post, when she began to experience numbness, paranoia, sensitivity to light and erratic behavior. Grasping for an answer, Cahalan asked herself as it was happening, "Am I just bad at my job — is that why? Is the pressure of it getting to me? Is it a new relationship?"

But Cahalan only got worse — she began to experience seizures, hallucinations, increasingly psychotic behavior and even catatonia. Her symptoms frightened family members and baffled a series of doctors.

On some of the symptoms she exhibited at the hospital:

"I slurred my words. I drooled. I didn't have proper control over my swallowing ... I kept my arms out in unnatural poses. At one point, I was like the Bride of Frankenstein — I kept my arms out rigidly. I was slow. I could hardly walk, and when I did, I needed to be supported ... I started [acting] very psychotic. I believed that I could age people with my mind. If I looked at them, wrinkles would form, and if I looked away, they would suddenly, magically get younger. And I believed that my father had murdered my stepmother. I believed all these incredibly paranoid — a huge, extreme example of persecution complex. And then as the days went on, I stopped being as psychotic, and I started entering into a catatonic stage, which was characterized by just complete lack of emotion, inability to relate, or to read, or hardly to be able to speak."

$ 1 Million of negative Investigation:

As weeks ticked by and I moved inexplicably from violence to catatonia, $1 million worth of blood tests and brain scans revealed nothing. The exhausted doctors were ready to commit me to the psychiatric ward, in effect condemning me to a lifetime of institutions, or death.

Pride & World Class Medicine:

The head doctors started speaking of a mythical miracle man named Dr. Souhel Najjar. "He would find out what it is," they would say. They even nicknamed him "Dr. House."

My mom requested that we meet this Najjar in person. And after two spinal taps came back with high white blood cell counts -- an abnormal occurrence that signifies brain swelling -- the case was officially passed on to Najjar, a Syrian-born neurologist, neuro-pathologist and epileptologist at NYU Medical Center.

When he came to speak with us, my family was buoyed by his confidence.

He grasped my hands and said, "I'm going to find out what this is and fix it."

Then he handed me a pencil and a piece of paper.

"Draw a clock," he said.

I grasped the pencil and made a circle. Feeling a little confused and put on the spot, I drew in the clock face.

My mother and father gasped.

All the numbers were written on the right side of the clock face, and no numbers were on the left side.

NAJJAR now had five clues as to what was overtaking me: the seizures, the catatonia, the high blood pressure, the high white blood cell count in my spinal taps and the bizarre clock. It was preliminary proof that the right side of my brain (which controls the left side of the body) was inflamed. He believed it was some sort of autoimmune encephalitis, or the swelling of the brain caused by an attack by rogue antibodies.

He decided to send my blood and spinal fluid to a well-respected neuro-oncologist from the University of Pennsylvania named Dr. Josep Dalmau to test to see if rare antibodies were present.

But we had to wait two weeks for the results.

In the meantime, a brain biopsy would be necessary, Najjar said. They would need to cut out a piece of my temporal lobe.

After the surgery, the blood work and spinal fluid came back positive for rare antibodies called anti-N-methyl-D-aspartic acid receptor, or anti-NMDAR encephalitis. The name signifies that the receptors in the frontal lobe, responsible for cognitive reasoning, and the limbic system, or the emotional center of the brain, are under assault by the immune system.

My body was attacking my brain.

Penn's Dr. Dalmau had discovered these antibodies in 2007. Until then, people suffering from my madness were misdiagnosed, likely ending up in mental hospitals, if not dead. Experts aren't sure what causes it, though they believe it's genetic, not environmental.

Najjar estimates that nearly 90 percent of those suffering from autoimmune encephalitis go undiagnosed.

Cahalan's new memoir is called Brain On Fire-My Month of Madness