Today was our first announced day and what we encountered outside the emergency ward (having been taken over this month to be our neurology clinic) was a bit of a mob scene, with patients everywhere waiting for Angel to get them registered. They would then proceed to have their vitals taken so we could begin to see them. Our first patient at morning report was a young gentleman, who had arrived during the night with new onset left hemiplegia following left neck pain. He was also found to be HIV positive – a new diagnosis. He would need to be seen in consultation by one of the residents.
We had three exam “rooms” for patient visits, and I told Angel that we could start by registering thirty patients for the day, as I knew we would inevitably see more than that. Later in the morning, the residents suggested setting up a fourth station to see patients waiting in the hallway adjacent to one of the other stations. So, it was with this arrangement that we began to plod away seeing patients for our first “announced” clinic day of the season. The number of patients we would see would quickly increase well beyond the thirty we had originally registered.
Ståle, a gentleman who runs a home in Mto wa Mbu and whom I have known for several years, came this morning with his car full of neurologically impaired children. Somehow, he has been tasked with caring for a number of young men with muscular dystrophy, most of whom we diagnosed and have treated for several years. He brings all of them on the same day packed into his Land Rover with their wheelchairs tied to the top.
Every resident who has met Ståle and the children he cares for has been changed for the better after seeing the dedication he has to these kids and the wonderful outlook these children have, despite knowing the condition they have and what the future holds for them. With this in mind, we saw all the kids, most of them with Duchenne’s muscular dystrophy, and continued on with our incredibly busy day.
One of his young boys, who had recently moved to Ståle’s home and had been assumed to have Duchenne’s muscular dystrophy, was clearly different from the other children. I saw him with Amisha, a neurology resident from Children’s Hospital of Philadelphia, and, unfortunately, we really had no history as the boy couldn’t give us any and Ståle had no family members to contact. The boy was unable to walk, but had no pseudohypertrophy of his calves and the majority of his atrophy was in his shoulders and arms. He also had some mild wasting of his temporalis muscles. Given this constellation of findings, it became clear to us that he had either fascio-scapulo-humeral or limb-girdle dystrophy, both of which have a tremendously better overall prognosis than Duchenne’s as they have a normal life span, albeit with significant disability. Still, it was a bit of good news we were able to relay.
Finally, in the midst of our incredibly busy clinic, I found that a patient had been brought in to see us on a stretcher and was promptly placed in the emergency room. Dr. Gabriel had mentioned to me previously that there was a patient he wanted us to see (and appropriately so), who had been in the hospital here in July with presumed encephalitis and wasn’t recovering. Unfortunately, after bringing the patient in, he promptly began with focal seizures that appeared to be epilepsia partialis continua – something that can often be difficult to treat. According to his family, the seizures had begun about a week prior and were essentially occurring on a regular basis. His original presentation was such that he was found unresponsive by his family at home and then had been brought into a local hospital where he remained unresponsive for about another 48 hours before being transferred to FAME. Here, it was clear that he had an encephalitis and underwent an LP and was placed on antibiotics and acyclovir. He eventually had a CT scan showing numerous large early hypodensities in the brain and more specifically, the bilateral temporal lobes, the most common location for injuries that occur in herpes encephalitis.
He had been treated with a 21-day course of oral acyclovir, but had never really woken up. He had also been placed on carbamazepine for his seizures, but at a relatively low dose considering the injuries to his brain and his propensity to have seizures for the rest of his life. As he lay on the emergency room gurney, unresponsive with continuous jerking of his left face, arm and leg, his family stood by patiently awaiting our input and whether we had anything at all to offer their 28-year-old family member. There was no issue with handling the seizure part of the equation, but having our very own infectious disease specialist here was certainly a blessing. We have no confirmatory tests here such as a PCR on the spinal fluid, so the diagnosis would be based on the clinical features that were quite suspicious for HSV encephalitis with treatment that had been delayed by at least several days. Herpes simplex encephalitis is something that must be treated immediately upon consideration of the diagnosis, as the virus rapidly multiplies and the damage it causes becomes more extensive. It is also irreversible once it occurs. The mortality rate for HSV encephalitis can be very high even when treated and the morbidity is great with chronic seizures and very common severe cognitive deficits.
We had to tell the family that he would not recover any of his function and would almost unquestionably remain unresponsive if he did not succumb to some complication. Yet, we could possibly improve his seizures by increasing his carbamazepine and, if that did not work, we could add phenobarbital later.
Our other patients were the typical mix of epilepsy, Parkinson’s disease, headaches, and back pain, to name but a few. Thankfully, we had no plans for the night as we had seen a total of 42 patients – the largest number we had seen in a single day.