Wednesday, May 31, 2017

Chapter 29: A favourite of readers!

As the BBC reported on the work on NMDA, this blog post was from Sep 1, 2013 and it was an answer to my case of Teratoma induced coma/psychosis.

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

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:

Chapter 29  The Power of Prayers             

Sunday, May 21, 2017

Dry Falls: Thinking Ouside The Box

Ideas without precedent are generally looked upon with disfavor
and men are shocked if their conceptions of an orderly world are challenged.
Bretz, J Harlen 1928.

At the start of the new school term after the summer of 1960, we had a new geography teacher. Now if the truth be told, up until that time Geography was not up on the list of subjects even the diligent students found exciting. Geography often competed with Civics for the bottom honour of the least favoured subject.

Not any longer. We now had a bright young teacher straight from the University of Hong Kong; and from the first lesson we were spell bound. Yes, she was elegant and stylish, but I really did not think those were the only reasons for our newly discovered enthusiasm. She was able to capture our attention and made what we thought were boring subjects riveting. For the first time what happened millions of years ago fascinated us. Glaciers became hot.

Looking back now, I realised our understanding of Planet Earth was then in its infancy. Harry Hess of Princeton University was just formulating the Sea-Floor Spreading theory that is now generally accepted in the geological community.

We were so inspired that the Meteorology Club membership increased dramatically. A guided visit to the Royal Observatory (Hong Kong) further cemented our new found interest.

On my recent vacation in Washington State, I was reminded of our inspiring geography teacher. No, not many of us became geologists or meteorologists; but our interest in such matters stayed with us.

In Washington State we saw a geological puzzle that took over 40 years to solve: the Dry Falls.

The Dry Falls, Washington State, U.S.A.© Am Ang Zhang 2007

The Dry Falls in northeast Washington was once the world’s largest waterfall, with water plunging 400 feet over a 3.5-mile-wide cataract. To put it into perspective, Victoria Falls, arguably the biggest and grandest fall in the world is only slightly over a mile wide and has a drop of 360 feet. Iguazu Falls is wider at 1.67 miles although nearly half a mile of it is now dry. Its drop ranges from 210 to 269 feet. Victoria Falls has therefore the largest curtain of water. Niagara, is small by comparison: ¾ mile wide and a drop of only 167 feet. However, it is the most easily accessible of the three falls.

The Dry Falls was the result of a series of events and catastrophes starting from 17 million years ago. For 6 million years, enormous lava flow after lava flow engulfed the area. This was followed by a warping of the plateau and an uplift of the mountainous region in the north. Then the Ice Age began about 1 million years ago, resulting in the formation of glaciers, subsequent flooding and formation of huge waterfalls. When the ice eventually receded, the network of rivers and streams in the plateau were left high and dry several hundred feet above the Columbia River. Today Dry Falls overlooks a desert oasis filled with lakes and abundant wildlife.

(Those interested in the full details can read the transcript of Mysteries of the Megaflood.)

The theory of the colossal flood was first proposed by geologist J. Harlen Bretz of the University of Chicago. In the summer of 1922, he became intrigued by the maze of huge streamless canyons, dry falls and other strange geological features in this area. As the area had few roads, he had to cover much of the 3000 square miles by foot. He worked painstakingly for the next seven summers, documenting what he saw, and came up with his hypothesis: a catastrophic flood greater than geologists had ever recognized on earth. It was a bold challenge to the prevailing principle of "uniformitarianism," which held that geological changes occur slowly, through steady processes. To other geologists, the idea of a sudden, colossal flood was unthinkable heresy. They emphatically declared his theory ‘wholly inadequate,’ ‘preposterous,’ and ‘incompetent.’ Though warned, Bretz proceeded to publish his conclusions.

In 1965, the International Geological Congress toured the Channeled Scablands (name invented by Bretz) and finally sent Bretz a telegram stating, ‘We are now all catastrophists.’

Satellite photographs taken in 1974 confirmed Bretz’s theory beyond doubt.

In 1979, the 96 year old Bretz was awarded the Penrose Medal of the Geological Society of America, that nation’s highest geologic honour.

Thursday, May 18, 2017

Lost City of Petra!

All photos  ©2017Am Ang Zhang
Carved directly into vibrant red, white, pink, and sandstone cliff faces, the prehistoric Jordanian city of Petra was "lost" to the Western world for hundreds of years.
Located amid rugged desert canyons and mountains in what is now the southwestern corner of the Hashemite Kingdom of Jordan, Petra was once a thriving trading center and the capital of the Nabataean empire between 400 B.C. and A.D. 106.           
National Geographic:

Monday, May 1, 2017

NHS: Best Health Care!

Friends moved to France after their retirement and lived in one of the wine growing districts.
 ©2008 Am Ang Zhang
They were extremely pleased with the Health Care they received from their doctor locally. After all, not long ago, French Health Care topped the WHO ranking.

Then our lady friend had some gynaecological condition. She consulted the local doctor who referred her to the regional hospital: a beautiful new hospital with the best in modern equipment. In no time, arrangement was made for her to be admitted and a key-hole procedure performed. The French government paid for 70% and the rest was covered by insurance they took out.

They were thrilled.

We did not see them for a while and then they came to visit us in one of our holiday places in a warm country.

They have moved back to England.

What happened?

Four months after the operation they were back visiting family in England. She was constipated and then developed severe abdominal pain. She was in London so went to A & E (ER) at one of the major teaching hospitals.

“I was seen by a young doctor, a lady doctor who took a detail history and examined me. I thought I was going to be given some laxative, pain killer and sent home.”

“No, she called her consultant and I was admitted straight away.”

To cut the long story short, she had acute abdomen due to gangrenous colon from the previous procedure.

She was saved but she has lost a section of her intestine.

They sold their place in the beautiful wine region and moved back to England.

The best health care in the world. 

Now we know.

Let us keep it that way.

NHS & Private Medicine: Best Health Care & Porsche

Do we judge how good a doctor is by the car he drives? I remember medical school friends preferred to seek advice from Ferrari driving surgeons than from Rover driving psychiatrists.

My friend was amazed that I gave up Private Health Care when my wife retired.

“I know you worked for the NHS but there is no guarantee, is there?”

Well, in life you do have to believe in something. The truth is simpler in that after five years from her retirement, the co-payment is 90%.

He worked for one of the major utility companies and had the top-notch coverage.

“The laser treatment for my cataract was amazing and the surgeon drives a Porsche 911.”

Porsche official Website

He was very happy with the results.

“He has to be good, he drives a Porsche.”

Then he started feeling dizzy and having some strange noise problems in one of his ears.

“I saw a wonderful ENT specialist within a week at the same private hospital whereas I would have to wait much longer in the NHS.”

What could one say! We are losing the funny game.

What does he drive?

A Carrera.

Another Porsche.

We are OK then.

Or are we.

He was not any better. And after eight months of fortnightly appointments, the Carrera doctor suggested a mastoidectomy.

Perhaps you should get a second opinion from an NHS consultant. Perhaps see a neurologist.

“I could not believe you said that, his two children are doctors. And he has private health care!” I was told off by my wife.

He took my advice though and he got an appointment within two weeks at one of the famous neurological units at a teaching hospital.

To cut the long story short, he has DAVF.

I asked my ENT colleague if it was difficult to diagnose DAVF.

“Not these days!”

He had a range of treatments and is now much better.

All in the NHS hospital.

“I don’t know what car he drives, but he is good. One of the procedures took 6 hours.”

Best health care.

I always knew: Porsche or otherwise.

Best Health Care: NHS GP & NHS Specialist

Does having a good hunch make you a good doctor or are we all so tick-box trained that we have lost that art. Why is it then that House MD is so popular when the story line is around the “hunch” of Doctor House?

Fortunately for my friend, her GP (family physician) has managed to keep that ability.

My friend was blessed with good health all her life.  She seldom sees her GP so just before last Christmas she turned up because she has been having this funny headache that the usual OTC pain killers would not shift.

She would not have gone to the doctor except the extended family was going on a skiing holiday.

She managed to get to the surgery before they close. The receptionist told her that the doctor was about to leave. She was about to get an appointment for after Christmas when her doctor came out and was surprised to see my friend.

I have always told my juniors to be on the look out for situations like this. Life is strange. Such last minute situations always seem to bring in surprises. One should always be on the look out for what patient reveal to you as a “perhaps it is not important”.

Also any patient that you have not seen for a long time deserves a thorough examination.

She was seen immediately.

So no quick prescription of a stronger pain killer and no “have a nice holiday” then.

She took a careful history and did a quick examination including a thorough neurological examination.


Then something strange happened. Looking back now, I did wonder if she had spent sometime at a Neurological Unit.

She asked my friend to count backwards from 100.

My friend could not manage at 67.

She was admitted to a regional neurological unit. A scan showed that she had a left parietal glioma. She still remembered being seen by the neurosurgeon after her scan at 11 at night:

“We are taking it out in the morning!”

The skiing was cancelled but what a story.

Best GP

Best Specialist