Since it was Tuesday, it was once again time for an educational lecture – today, Jack would be delivering the third in a series of epilepsy talks, this one covering the subject of status epilepticus. This is a very serious condition in which a convulsive seizure lasts for greater than five minutes or one has multiple seizures without returning to normal in between. The distinction between status epilepticus, or SE for short, and a brief convulsive seizure is that the former can not only cause permanent injury to the brain but can also be life threatening with a significant mortality the longer the event continues. Therefore, the recognition and emergent treatment of SE are essential to preventing death or brain injury from occurring in this patient population. The causes of SE can merely be an underlying diagnosis of epilepsy with non-compliance on their anti-seizure medications (ASMs), though this can also occur even in the setting of therapeutic levels of an ASM. The other causes of SE, such as metabolic (i.e. hyponatremia) or structural (i.e. stroke or tumor) always need to be screened for as the treatment in addition to stopping the SE acutely, includes any necessary management of an underlying cause.
In the US, patients with SE will often need to be intubated, either because of the status itself and the inability to protect their airway, or the treatment of the SE which includes benzodiazepines that can impair respiratory drive and airway protection. Unfortunately, here at FAME and in much of Africa, there are very few, if any, ventilators available for use once a patient is intubated (there are two ventilators here at FAME that are used in our surgical theaters), and therefore, one must be very cautious when using medications that will ultimately lead down that path.
Jack presented an excellent and incredibly comprehensive treatment pathway for FAME that can be followed by all our clinicians when patients present in SE. There was a great deal of discussion prior to developing the pathway as well as during the meeting regarding the issue of the initial benzodiazepine to administer as it has been a long-standing practice at FAME not to administer midazolam, a very rapid onset benzo, without having the nurse anesthetist present in the event the patient could require some respiratory support. Our proposal, though, was to administer the midazolam intramuscularly rather than intravenously, a route that would be essentially equally effective and would not pose those same risks. Thankfully, everyone came together and there was ultimately a consensus by all parties involved and the protocol with the IM administration of midazolam was given a formal blessing. Helpful to the discussion was the fact that there were numerous studies demonstrating the far greater risk of injury from continuing to seize than there was for the potential of respiratory suppression. Having a status epilepticus treatment pathway available was another one of the goals that Cat, Julian, and our team had for this visit, and it was now complete.
Natalie, who had arrived in Tanzania last evening via Zurich and overnighted at the KIA Lodge adjacent to the airport, would be arriving to FAME this morning and was a very welcome member of our neuro team. Natalie had been here three years ago as one of our peds neuro residents and would now be returning as a fully trained stroke attending working at both Penn and CHOP. She would be adding a significant amount of expertise in not only the world of cerebrovascular disease, a very prevalent problem here in Sub-Saharan Africa due to untreated hypertension, but she would also serve as one of our pediatric neurologists, a very important position considering pediatrics represents about a third of the patients we see here at FAME and on our mobile clinics. The fact that previous residents who had accompanied me to FAME as part of our global neurology program, such as Cat, Natalie, and soon LJ, have elected to return as faculty, despite the significant financial and work related issues they have to overcome to get here, is a clear indication of the positive impact this experience has on our trainees. Each and every person who has worked here with me over the years, well over 100 of them, has not only made a huge difference in the lives of those less fortunate, but will also continue to do so over their careers.
One of the patients that was seen today was a woman who had originally presented several months prior with right arm sensory and motor changes and was subsequently found to have a large mass in the left parietal region that easily explained her symptoms and deficits. She had undergone an MRI as well and had been told by the neurosurgeon at KCMC that she should have the mass excised and sought a second opinion from us as to what our recommendations would be. She had already been placed on steroids for the edema that was present which was certainly something we would have suggested, but as far as what to recommend for the primary treatment of the mass was something just a bit out of our wheelhouse. I did offer to have the MRI loaded into our system so that Dr. Alex, our neuroradiologist back home who reads all our scans officially, could review it and make sure he agreed with the report we had from NSK where she had had the study. Unfortunately, he agreed with their read that the mass represented a high-grade glioma, a diagnosis that has a very poor prognosis whether she was living in Tanzania or in the US.
Clearly the best case of the day, and quite possibly of the month, for the reason that it demonstrated the full effect of the work we’ve been doing here at FAME not only over the last weeks, but for the last 15 years, was a young man who presented to FAME after being found down and having dislocated his shoulder. But it was not just any old dislocation, it was a very specific type, a posterior dislocation, that occurs most often in patients who have a generalized convulsion and dislocate their shoulder in the process of seizing. As morning report was ending as we were out of time due to the number of questions after Dr. Jack’s amazing talk, Dr. Dahaye (Regina) simply stated that she had a patient who had been brought in and had a posterior dislocation of the shoulder, fully aware of the significance of the event, and asked if we could see the patient. The neuro team was quickly on the case, and what was found, was that this 24-year-old young man, in addition to having fallen and lost consciousness causing his presentation (which was unwitnessed), also had about a year history of brief episodes of altered sensorium, without convulsion, as well as jerks of his arms. These jerks were most suggestive of myoclonus, a phenomenon very commonly seen in patients with a specific type of epilepsy – juvenile myoclonic epilepsy, or JME – that typically presents in adolescence, though can occur later in some patients.
With this history, we were concerned enough that the patient had underlying epilepsy, and probably a primary generalized epilepsy, that we planned on starting him on an anti-seizure medication to prevent further events. The commonly used medications for this type of epilepsy and that we have here are valproic acid (Depakote), levetiracetam (Keppra), and lamotrigine (Lamictal). Carbamazepine (Tegretol), perhaps the most commonly and cheapest used medication here other than phenobarbital, is a medication that is typically avoided in patients with this type of epilepsy as it can cause worsening of their seizures. We elected to put him on valproic acid as it would be the most accessible medication at the local duka la dawas (pharmacies) if he ever needed to refill the medication elsewhere and was an excellent choice for the type of epilepsy we felt he had clinically. It was also the least expensive of the three alternatives.
Though it wasn’t likely to change what our recommendations were going to be today (i.e. we were going to start him on an anti-seizure medication regardless), we also discussed obtaining an EEG as that could help us in the long-term management, such as deciding if and when we would contemplate tapering his medication in the future. We texted Jacob to see if he was available to do an EEG before the patient was discharged home, and, thankfully, he was. Even more so, he was thrilled to help out with the case. After applying the Brain Capture cap, he gelled the electrodes and checked the impedances which were all excellent. Jacob completed and uploaded the EEG to the cloud, though Cat and Julian were still in the air en route to Philadelphia, and despite the fact that we were communicating with Cat on the flight, her internet wasn’t anywhere close to what was needed for her to have read the study mid-flight. We explained to the patient that we would have the results of the EEG later in the day, but that we would start him on his medication prior to being discharged as we weren’t depending on the EEG results anyways.
Cat and Julian’s flight was set to arrive at around 9:00 am in Philadelphia, or 4:00 pm our time in Karatu. Within several hours of her landing, we received word from Cat in our WhatsApp group that she had looked at the EEG, and that it had indeed demonstrated a single well-defined generalized discharge consistent with a primary generalized epilepsy, and very likely JME. In less than 12 hours, we had gone from a FAME clinician being appropriately suspicious of a posterior dislocation of the shoulder in an otherwise generic episode of loss of consciousness and concerned about epilepsy, to having the patient evaluated by neurology with additional concerns for a specific type of epilepsy, deciding to treat the patient with a specific medication for that epilepsy, and finally confirming the diagnosis with an abnormal EEG that was consistent with a primary generalized epilepsy. Kudos to Dr. Dahaye for her wonderful clinical acumen that brought the patient to our attention, kudos to the neuro team for seeing the patient and being suspicious, kudos to Jacob for his diligence in obtaining a wonderful EEG, and finally, kudos to Cat and Julian for setting up the whole process that gave us the ability to treat this patient in such an unparalleled manner that would never have been otherwise possible.