Tuesday, December 1, 2015

Answer to Prayer: Teratoma


Beautiful Finland

© 2012 Am Ang Zhang

The following is extracted from The Cockroach Catcher: Chapter 29 The Power of Prayers.

  Some time in early February of 1978 I was called to do a Home Visit on a thirteen year old girl by Dr Pinkerton, a paediatric consultant. Dr Pinkerton had been the local Paed for years and was generally well regarded. She had, in my short time as consultant, referred a couple of cases, most notably that of a Tourette syndrome and a boy with non-stoppable hiccups. Both cases put me in her A-list and I gathered that not many were on that list. Needless to say I realised too that her cases were never straightforward or simple.  Those she would have dealt with herself. The girl had upper arm stiffness on the left side and Dr Pinkerton could not find much else wrong with her, and so it crossed her mind that perhaps there was something psychiatrically wrong.  The girl was also carrying out some strange rituals around the house and Dr Pinkerton did wonder about psychosis or even catatonia.
...............


 The x-ray came back. The tell tale tooth was there and yes – a Teratoma[5], the distinctive type of tumour that can include teeth, hair, sometimes, even a jaw and tongue.  I guessed just a split second before the results came back. How annoying.
         Working diagnosis: Teratoma with possible toxic psychosis.
         Emergency operation was arranged. Yes, she would be fine a little while after the operation, I reassured the parents.
         The paediatric junior arrived and took some history and did a quick physical before she was prepared for the theatre. This petite doctor with a very babyish face told me that on her first day in her last job she had to do an emergency tracheotomy. This time she had been on call for the last three nights and the battery in her old Mini could not cope with the heavy frost so she had to wait for AA before coming. She was most apologetic for not having got in earlier.
         She asked if I had seen many toxic psychosis cases and I asked if she had come across any in her psychiatric placement. As with all good psychiatrists answering a question with another is in our blood and here it worked well.
         Neither of us knew what was to hit us next.
         At 2 A.M. I had a call from her.
         “Your patient – I mean our patient could not be aroused after the operation. Yes they removed the teratoma, complete and intact. It is bigger than any specimen I have seen but she could not be aroused.  Any ideas?”
         “Call the paediatrician on call in the regional paediatric unit and I will be in.”
         What happened?  I asked myself as I drove to the hospital.
         What had we done? This was fast becoming a nightmare situation.
         What was I going to say to the parents?

         Something else was going on here, and I was not happy because I did not know what it was. I was supposed to know and I generally did. After all I was the consultant now.........


Read the whole chapter: Chapter 29  The Power of Prayers


In medicine, truly new discoveries are uncommon and with the emergence of guidelines and protocols it has become even more difficult to make new discoveries. It has taken over 30 years before I could understand what happened to my Teratoma patient. Luckily for her, the treatment she received would have been in line with what we know now of the condition.

Hospital Medicine indeed has its important place and most important of all in the discovery of new conditions and establishing diagnostic and treatment programmes.

It is perhaps timely to remind the next generation of Bright Young Things that become doctors to remember that psychiatric symptoms presented by a patient may indeed be the presentation of a neurological condition.

This is more so for bizarre combinations of psychiatric and other symptoms. It was in the last five years or so that much progress has been made on what is now called Anti-NMDA Receptor Encephalitis.

Who knows, one day medical scientists might be able to decipher the most difficult of psychiatric conditions: Schizophrenia. Bright Young Psychiatrist might have noticed that Clozapine, one of the most effective drugs for schizophrenia has a marked effect on the immune system.

In the mean time Pennsylvania might have something they could be proud of: the discovery of this new neurological condition.


WoodlochPennsylvania ©2012 Am Ang Zhang

For now, my patient’s parents’ prayer has been answered. 

Chapter 29  The Power of Prayers


Anti-NMDA Receptor Encephalitis


NEW ORLEANS — A mysterious, difficult-to-diagnose, and potentially deadly disease that was only recently discovered can be controlled most effectively if treatment is started within the first month that symptoms occur, according to a new report by researchers from the Perelman School of Medicine at the University of Pennsylvania. The researchers analyzed 565 cases of this recently discovered paraneoplastic condition, called Anti-NMDA Receptor Encephalitis, and determined that if initial treatments fail, second-line therapy significantly improves outcomes compared with repeating treatments or no additional treatments (76 percent versus 55 percent). The research is being presented at the American Academy of Neurology's 64th Annual Meeting in New Orleans.

565 cases! Not so rare!

The condition occurs most frequently in women (81 percent of cases), and predominately in younger people (36 percent of cases occurring in people under 18 years of age, the average age is 19). Symptoms range from psychiatric symptoms, memory issues, speech disorders, seizures, involuntary movements, to decreased levels of consciousness and breathing. Within the first month, movement disorders were more frequent in children, while memory problems and decreased breathing predominated in adults.

My patient was under 18 and presented with catatonia symptoms. She later lose consciousness and was ventilated.

"Our study establishes the first treatment guidelines for NMDA-receptor encephalitis, based on data from a large group of patients, experience using different types of treatment, and extensive long-term follow-up," said lead author Maarten TitulaerMD, PhD, clinical research fellow in Neuro-oncology and Immunology in the Perelman School of Medicine at the University of Pennsylvania. "In addition, the study provides an important update on the spectrum of symptoms, frequency of tumor association, and the need of prolonged rehabilitation in which multidisciplinary teams including neurologists, pediatricians, psychiatrists, behavioral rehabilitation, and others, should be involved."

The disease was first characterized by Penn's Josep Dalmau, MD, PhD, adjunct professor of Neurology, and David R. Lynch, MD, PhD, associate professor of Neurology and Pediatrics, in Annals of Neurology in 2007. One year later, the same investigators in collaboration with Rita Balice-Gordon, PhD, professor of Neuroscience, characterized the main syndrome and provided preliminary evidence that the antibodies have a pathogenic effect on the NR1 subunit of the NMDA receptor in the Lancet Neurology in December 2008. The disease can be diagnosed using a test developed at the University of Pennsylvania and currently available worldwide. With appropriate treatment, almost 80 percent of patients improve well and, with a recovery process that may take many months and years, can fully recover.

Teratoma: finally!

In earlier reports, 59 percent of patients had tumors, most commonly ovarian teratoma, but in the latest update, 54 percent of women over 12 years had tumors, and only six percent of girls under 12 years old had ovarian teratomas. In addition, relapses were noted in 13 percent of patients, 78 percent of the relapses occurred in patients without teratomas.
As Anti-NMDA Receptor Encephalitis, the most common and best characterized antibody-mediated encephalitis, becomes better understood, quicker diagnosis and early treatment can improve outcomes for this severe disease.
The study was presented in a plenary session on Wednesday, April 25, 2012 ET at 9:35 AM at the American Academy of Neurology's annual meeting.
[PL01.001] Clinical Features, Treatment, and Outcome of 500 Patients with Anti-NMDA Receptor Encephalitis

Of 100 patients with anti-NMDA-receptor encephalitis, a disorder that associates with antibodies against the NR1 subunit of the receptor, many were initially seen by psychiatrists or admitted to psychiatric centres but subsequently developed seizures, decline of consciousness, and complex symptoms requiring multidisciplinary care. While poorly responsive or in a catatonic-like state, 93 patients developed hypoventilation, autonomic imbalance, or abnormal movements, all overlapping in 52 patients. 59% of patients had a tumour, most commonly ovarian teratoma. Despite the severity of the disorder, 75 patients recovered and 25 had severe deficits or died.

Related paper:



Post Script:
“Ten years later mother came to see my secretary and left a photo. It was a photo of her daughter and her new baby. She had been working at the local bank since she left school, met a very nice man and now she had a baby. Mother thought I might remember them and perhaps I would be pleased with the outcome.

I was very pleased for them too but I would hate for anyone to put faith or god to such a test too often.”

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