Coining of a New Term, “Status Gelasticus”

To the Editor: We read with great excitement the title of the manuscript by Palmini et al. (1), which involved the term, statusgelasticus. However, we were somewhat surprised that the term was used as if it were accepted medical terminology. As far as we are aware, the term status gelasticus had never been used before our describing the emergency transcallosal resection of hypothalamic hamartoma (HH) in a 30-month-old boy for status gelasticus previously (2,3). In our article, we defined status gelasticus (regardless of impairment of awareness as in epilepsia partialis continua) as near-continuous gelastic seizure activity for a prolonged period (3). This was a reminder that status epilepticus is defined either as “a seizure that persists for a sufficient length of time or is repeated frequently enough that recovery between attacks does not occur” or seizures that persist for 20 to 30 min (4,5). In our article, we made the point that although status gelasticus clearly implies a form of status epilepticus consisting of a prolonged cluster of gelastic seizures, our patient (like the patient described by Palmini et al.) was not strictly in status epilepticus, as he did regain full awareness between seizures (if any impairment of awareness did occur during each gelastic seizure). We propose that the term be used for a patient with a prolonged cluster of gelastic seizures (i.e., for 20–30 min, a duration similar to that for status epilepticus). Their case also was of great interest because it raises many points and controversies about HHs. Although their patient, in retrospect, had been having gelastic seizures for “as long as he could remember,” these seizures had not been diagnosed preoperatively. Gelastic seizures are often not diagnosed for many years, and the diagnosis of HH delayed. If one is to believe that this patient truly had dual pathology and independent seizure foci, then he is indeed a unique case, and we shall evaluate all future HH patients with caution. However, if he is a relatively more typical case of HH with initial onset of gelastic seizures followed by evolution of other seizure types from a presumed secondarily generalized epileptogenesis, then he remains a unique case of secondary epileptogenesis but with partial onset (temporal lobe). Many more cases of unsuccessful focal neocortical resection for the treatment of symptomatic epilepsy in patients with HHs have been described (6) and seen by us than this unique successful case. Also of interest was the comment by the authors that in retrospect (the HH had not been diagnosed), they would have probably had more reservations with regard to proceeding with their successful surgery. We would likely have proceeded with HH resection in such a patient, even if the seizures had localized to the left temporal lobe by scalp video-EEG monitoring, as we have previously shown that video-EEG monitoring may be unnecessary and can actually be misleading in the localization of seizures in patients with HHs (7,8). The HH and epilepsy model continues to teach us many interesting lessons about subcortical epileptogenesis (9).