Milestones. Today I passed my board exam for pediatric neurology or neuropediatrics, as we call it in Germany. Even though I am usually not big on celebrating occasions like this, I wanted to use this blog post to reflect upon a journey that led me to three different continents and started eleven years ago in the foothills of Appalachia.
Beginnings. When I was an exchange student at the University of Kentucky in 2002, we had an outreach clinic in Appalachia during my child neurology rotation. This is where I saw my first patient with Batten disease, a neurodegenerative disorder of childhood also known as Neuronal Ceroid Lipofuscinosis Type 3 (NCL3). Neurodegenerative diseases in children immediately make you ask three questions: (1) “What is happening there?” (2) “Why don’t I understand it?” and (3) “Is there anything that can be done?” By asking these questions, you’re already on your path to becoming a child neurologist. Personally, I have always had in interest in neuroscience and previous to an exchange year in the U.S., I had done my medical doctoral thesis on molecular neuroscience, investigating gap junctions in the Central Nervous System. But I wavered in my decision which specialty to choose. Neurology, Psychiatry, Neurosurgery? I eventually made a firm decision to become a child neurologist after an intense and impressive four weeks of child neurology in Kentucky, realizing that pediatric neurology is a unique intersection of neurology, pediatrics, and genetics. It also requires an understanding of how the growing brain works.
The growing brain. One patient who I will always remember from my Kentucky rotation is a small child with transverse myelitis, an inflammation of the spinal cord. This inflammation left the child temporarily paralyzed, but he recovered quickly. I vividly remember checking the Babinksi reflex on a daily basis, completely amazed that the child improved on a daily basis. Now, in 2014, a bit further in my career, I am aware how uncommon both things are, transverse myelitis in a toddler and the rapid recovery. Nevertheless, it left with me with a deep impression about the growing brain and the differences between adult neurology and pediatric neurology. There is plasticity in the growing brain usually not seen in adults. On a more global scale, there are differences between children and adults when it comes to neurology. Let me give you a drastic example. I usually tend to ask my students on the ward a seemingly simple question: “If a child has a perinatal stroke involving the Medial Cerebral Artery (MCA), what is the clinical presentation?” Nearly all of our students reply that the neonate will present with an acute hemiplegia, a palsy of one side of the body. While this is the correct answer for MCA strokes in adults, nothing could be further from the truth in neonates. The corticospinal tract, responsible for the movement of my fingers while typing these lines, is not yet myelinated in neonates. The majority of the motor activity seen in neonates is subcortical, not involving the cortex. This is why neonates do not present with hemiplegia. In contrast, one of the most common presentations of neonatal stroke is seizures, a feature rarely seen in adult stroke. To put it differently: Child neurologists are not little adult neurologists (Ashwal 1995).
Why did it take so long? As a reader of our blog, you might be surprised that I am only finishing my training now. To give you the honest answer: I could have been a bit quicker, but I was pretty much in time. Also, training schedules are as different between countries as possibly imaginable. Training in pediatric neurology in Germany takes a bit of time. You complete a full pediatric training of five years and add another three years of child neurology. In addition, in an academic setting, you are expected to develop a research career alongside of your residency. There is not MD PhD in Germany; you are expected to come out of your training as both a clinician and researcher. This gives you ample opportunity to develop your own focus. I was fortunate enough to combine a two year fellowship at the Epilepsy Research Centre, Melbourne, the premier center for epilepsy genetics in the world, with a neurology-focused pediatric training at the Department of Neuropediatrics in Kiel, one of the cradles of pediatric epileptology in Germany. In addition, I had the unique opportunity to be appointed as one of few clinical junior professors in Germany in 2011, a unique career track to provide academic independence to young clinicians already during their training. Looking back at the last eleven years, I would like to use this opportunity to thank my mentors for all their support.
Palaces. Preparing for a board exam is somehow a luxury. You suddenly have the right to retreat and study. And this is an occasion that you don’t often get in a busy research and clinic schedule. I took several days off for studying, trying to review many of the topics that I had encountered over the years that had escaped my long-term memory. And I realized that over the years, I had gotten quite good at cramming. Joshua Foer, a science journalist who became US memory champion within 12 months of training, talks about the art of memorization in his book Moonwalking with Einstein. There are basically three aspects to memorization: location, association, and repetition. During my studies at the University of Heidelberg and while preparing for the United States Medical Licensing Exams, I became a multiple-choice question junkie, enjoying the almost athletic aspect of going through hundreds of questions per day. Now I understand that I was heavily focusing on repetition, trying to beat the Ebbinghaus forgetting curve. As the more advanced exams are usually oral exams, there is no database of multiple choice questions. That’s when I tried to enjoy learning by building memory palaces. The art of memorization is credited to Simonides of Ceos, who is one of the fathers of the method of loci. This learning method basically suggests that you connect places in the real world with items that you would like to memorize, creating memory palaces. For example, if you place imaginary items alongside your way to your room, you can always mentally walk this path and remember the imaginary items placed there. This is how I first remembered Hebrew letters. The letter shin (ש), for example, is a broken wine glass on Katie’s desktop. For the hot phase of my exam, I build various memory palaces. Given the limited time available, I sometimes had to resort to extreme measures. A princess in a paper bag standing on the counter of our local coffee shop (symbolizing Spastic Ataxia Charlesvoix-Saguenay, one of the mimickers of Cerebral Palsy in children), an exploding ice cream parlor (symbolizing intranuclear ophthalmoplegia), and the tower of the Marienkirche in Bad Segeberg. As the latter involved some last minute cramming, it suffered the most, symbolizing the stereotypies of autism spectrum disorder, the early stages of brain development and SIX3, the gene for holoprosencephaly type 2 (HPE2). Anyway, as you can tell, I enjoyed being a student again for a few days, and I would like to thank my wife Katie and my parents for giving me the time to study.
Child neurology of the future. As I indicated earlier, one of the recurrent question of child neurology is, “Isn’t there anything that I can do?” Pediatric neurology today is still a relatively descriptive field. We can name and diagnose many diseases, but there is not specific treatment for a large number of conditions. I have two basic comments regarding this. First, not being able to treat causally does not mean that nothing can be done. In many degenerative diseases in childhood, there are intense and promising concepts, even though the causal mechanism itself cannot be altered. The modern multi-modal treatment of Duchenne Muscular Dystrophy is a good example. Also, in our patients with epileptic encephalopathies, we are now able to offer a broad range of therapeutic strategies ranging from new antiepileptic drugs for seizure control to physical therapy, even though we cannot affect the underlying mechanism. Secondly, pediatric neurology is probably one of the fields of medicine that will undergo a drastic change in the future. Specialties such as orthopedic surgery, oncology and even adult neurology have changed from purely descriptive and diagnostic fields to interventional fields of medicine, be it through surgical techniques, chemotherapy or novel methods for treatment of acute stroke. I predict a similar change in the field of pediatric neurology in the next 20 years. In addition, pediatric neurology is tightly linked to research. For most pediatric neurologists, there is not a single clinic day when you don’t encounter a patient who makes you ask questions relevant to research. In many cases, this question is: “Why is this child affected by this disease?” In many cases, you won’t find a reason. This is when you think genetics. We are advancing step by step in understanding the genetic basis of pediatric neurological disorders, and hopefully we will be able to translate this understanding into novel and targeted therapies in the near future.