Friday, February 12, 2021

Bright Young Doctors & Rainbow: NMDA & Autoimmune Encephalitis

In Medicine, it is amazing what the Bright Young Doctors can offer. In my day and now!


Forty years ago, a patient of mine was unconscious for 23 days and it was mother's belief that it was through prayer that her daughter was saved. I did not know anything about NMDA or Autoimmune Encephalitis then nor did Queen Square.

The Bright Young Doctor in my days:

" A good doctor is one who is not afraid to ask for help but he must also know where to ask.
         “Get me Great Ormond Street.”
         “I already did.”
         She is going to be a good doctor.
         “Well, the Regional unit said that they had no beds so I thought I should ring up my classmate at GOS and she talked to her SR who said “send her in”.”
         Who needs consultants when juniors have that kind of network?  This girl will do well.
         “Everything has been set up. The ambulance will be here in about half an hour and if it is all right I would like to go with her.”
         “Yes, you do and thanks a lot.”   ............."                     full ChapterHERE


But perhaps God works through his people in his own way. Discoveries in Medicine should therefore enhance our faith rather than the other way round.

It took nearly 40 years for the real answer to her prayers to really emerge.

 ©2012 Am Ang Zhang


The Bright Young Doctor now:

I was staying at our resort in Boquete and was having dinner with three friends all of them with medical connections. One was in hospital administration and one a nurse. The husband of the nurse was a pharmacist. Somehow the conversation drifted into medical topics and knowing that I am a Child Psychiatrist the pharmacist started talking about his nephew who was nearly sent to a mental institution as he suffers from catatonia and doctors eventually diagnosed schizophrenia and put him on antipsychotics. Luckily the catatonic symptom probably saved him as some bright young thing just read the book Brain On Fire and gave him the Clock Test. That led to the NMDAR antibody testing that proved positive. He responded well to the treatment regime that has been developed and is off all antipsychotic medication.

My Teratoma patient was lucky as she belong to that group that improved without further treatment once the Teratoma was removed. She eventually had a baby.


The Power of Prayers & Teratoma: Brain & NMDA!


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

Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies

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:

Anti-NMDA Receptor Encephalitis: Diagnosis, Psychiatric Presentation, and Treatment


Chapter 29  The Power of Prayers

The following is extracted from The Cockroach Catcher: Chapter 29 The Power of Prayers.
According to old Chinese advice, it is wise never to discuss politics or religion even amongst best friends.           Religious belief can often blur judgement in the wisest of people. In psychiatry it is sometimes not easy. This is particularly true in cases of florid psychosis, which often presents with symptoms of hallucination, delusion and even vision.         I remember my early days of psychiatry in a mental hospital in Hong Kong. Yes, it was the days of 2000-bed hospitals. Yes, it was the days of Medical Superintendents who had supreme power and all doctors of whatever rank and experience were Mental Health Officers with special authority to sign papers for compulsory admissions. The forensic unit was contained within the same complex.         Those were the days when we encountered psychosis in the raw so to speak. All the colony’s really mad people were admitted to this one place set in the furthest corner of the colony. In our year seven of us decided without much discussion that we all wanted to go into psychiatry. That was over 10% and all had quite idealistic reasons. It was perhaps a bit of a disappointment to our parents that we did not pursue a more conventional specialty that might provide us with more status and financial reward. Then there was the fear of contamination that somehow one might become mad too. Recent day medical students are said to shy away from psychiatry for these same reasons.         Education seems to have little effect on superstition.                                     ......................................….read the full ChapterHERE



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

Brain on Fire      

                     

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