I remember driving my car many years ago, listening to one of my favorite radio shows, Car Talk - Click and Clack The Tappit Brothers. At the close of their show on this particular day, they ended with the usual clever banter. But for me, this day would be anything but funny.
One brother said to the other, he had been searching the internet and had finally come across a diagnosis that explained his brother’s silly behavior! He said, “You have Gelastic Seizures!” And they both broke out laughing. I was stunned! I pulled my car to the side of the road and listened as the jokes ensued. “That’s it!” “You are right!” “What a great thing to have!” My heart sank and I began to sob. It was not at all funny – it was heartbreaking.
Gelastic and Dacrystic seizures are extremely rare and often very difficult to recognize. Many families have reported the first (mis)diagnosis they were given was colic, acid reflux (GERD), and even just a “fussy baby”. Some have even questioned about why they would bring such a “Happy Baby” in for an exam? Often, it isn’t until the first complex partial, or other seizure type develops, that parents can reflect back and say that they thought they just had a child that was “quirky”. Adult patients have said, “I didn’t mind the gelastics early on, they actually felt comforting.” With such a wide variety of ways gelastics and dacrystic seizures can present – it is no wonder these seizures are often missed in the early days.
With an occurrence of 1 in 200,000 - most neurologists will never see a patient with these types of seizures. However, the importance of educating our ourselves and the medical professionals that may indeed have an HH patient walk through their door - is critical. Correct diagnosis, in the minimum amount of time, followed by appropriate treatment is always the goal.
Here are some of the facts according to the medical literature:
- Gelastic seizures are the most specific symptom associated with HH.
- They are usually brief, often just a few seconds in duration, but can go much longer. In rare situations, an event described as Status Gelasticus can happen. This condition is characterized as “a prolonged cluster of gelastic seizures (that is, for more than 20-30 minutes, a duration similar to that of status epilepticus), but without necessarily involving loss of awareness between seizures.” (1)
- They can be very frequent, with as many as 100 seizures or more a day, or less frequent, with a few a week/month.
- For many, there is no feeling of mirth or genuine happiness, more of a feeling of loss of control. However for others, there may be a general feeling of contentment and satisfaction. Dacrysic seizures typically look like crying and present as a feeling of sadness or frustration.
- Gelastic seizures usually do not respond to medication
- Interictal and ictal EEG features associated with HH and gelastic seizures often show no change from the on-going background, which is itself often normal (2)
Clinical research over the last 20 years has led to better diagnostics and treatments for individuals with Hypothalamic Hamartoma. There is still much to learn about how HH and both gelastic and dacrystic seizures affects each individual.
Let us know which professionals you are working with in your state so that others may benefit from your experience!
As for the wonderful Tappit Brothers, hopefully they will continue to do what they do best – diagnose cars!
- Long-term outcome after emergency resection of hypothalamic Hamartomas for status gelasticus. Sandipan pati, M.d.,1 Reena G RaStoGi, M.d.,2 adib a. abla, M.d.,3 HaRold l. Rekate, M.d.,3 and Yu-tze nG, M.d.2 . Neurosurgical Focus/Vol 30/Feb 2011.