On the road. For this week, the Channelopathist will be a travel blog. I am on my way to Israel where we will be busy recruiting and phenotyping epilepsy families for the EuroEPINOMICS project for the next seven days. This trip abroad gives me the opportunity to do something that I have been thinking about for quite some time: reading “Epilepsy and Related Disorders” by William G. Lennox, one of the pioneers of epilepsy genetics. I will try to put some thoughts on Lennox into words this week while spending my time down here in Israel.
Light reading, but not a light book. There has been one problem taking the Lennox book on the road on earlier occasions. It is simply too heavy. I actually wondered whether there is some lead hidden in the book covers somewhere, but at least the airport scanners didn’t seem to pick up anything unusual. My copy was printed in 1960 and weighs more than my laptop. Therefore, I had to limit myself to only taking Volume 1 with me.
Symphony of neurons. I know very little about William G. Lennox, but at least three things become clear when reading parts of his book. First of all, Lennox was a very meticulous data collector. His records span reports on more than a thousand epilepsy patients with many separate case reports. Secondly, I find Lennox’s data visualization impressive. This was before the PowerPoint era, but Lennox has graphs, histograms etc. that really drive the point home that he wanted to make. I actually wonder how researchers back then did this. Did they draw this all themselves? I can only imagine how much time this must have taken to work with real paper spreadsheets and draw your histograms yourself. Third, Lennox likes to write. He states that “the writing is meant to be informal and lightened with whatever of history and humor is at hand”. He often uses poetical description as in his description of inherent rhythms: “A poet might say, that in the beginning, when God taught the stars to sing together, He also composed the symphony of the neurons”. As a reader, you can really feel that writing was sheer fun for him. In 2012, he would probably write blogs.
Lennox on Febrile Seizures. For some reason, I got stuck with the chapter on Febrile Seizures during my flight to Israel. Lennox looks back on a cohort of ~400 children with Febrile Seizures, even though the distinction between pure Febrile Seizures and seizures with fever as the first symptom of a lesional epilepsy wasn’t really fully clear to him yet. I think that the field of Febrile Seizures is a good starting point on how to appreciate what we have learned in the last 50 years. While Lennox was still trying to show that there is no anemia or hyponatremia during Febrile Seizures, the entire phenomenon and a possible mechanism was still very obscure to him. He provides the reader with at least seven possible explanations on how fever and seizures might be connected with little hard data to prove or disprove either of them, as he willingly acknowledges. Interestingly, he had access to disease phenotypes that don’t really exist anymore today. For example, he pointed out that patients with tertiary malaria rarely have seizures during the later fever episodes and that Febrile Seizures preferentially occur with upper respiratory infections. As for the mechanism, he wonders whether fever might make the cell membranes more permeable to endotoxins, which, in turn, results in seizures. Clearly, his book was written way before the ion channel era. Interestingly, Lennox also provides two case reports on patients with multiple febrile seizures, alternating hemiclonic seizures and cognitive decline after the first year of life, a description that would fit with Dravet Syndrome. In summary, the chapter on FS highlights the difficulties of distinguishing Febrile Seizures from other conditions resulting in epilepsy with fever. Useful concepts for this have really only been developed in the last two decades with the recognition of SCN1A mutations in Dravet Syndrome and the formalization of the GEFS+ spectrum.
Old New Land. The Old New Land is a reference to a utopian novel by Theodor Herzl, one of the founders of Zionism. When I looked at my bookshelf yesterday, I realized that I had accumulated quite a collection of books on Israel during the last nine months and the following selection might help you as an introduction to the everything Israel: get yourself pumped with chutzpah and can-do mentality with Dan Senor’s and Saul Singer’s START-UP NATION. Then delve deep into history with Simon Sebag Montefiore’s JERUSALEM – THE BIOGRAPHY. After this, learn about the Middle Eastern conflict through Peter Beinart’s THE CRISIS OF ZIONISM. Finally, go back to the very start and read Herzl’s 1902 utopian novel THE OLD NEW LAND, describing his dream of a cosmopolitan, egalitarian and tolerant new society. The Hebrew title for his book has had some impact and provided a name for a growing Jaffa suburb. It is called Tel Aviv.