We may be a small Island nation but we’re an immensely diverse one too, with over 135 nationalities represented. However, with a population of just touching 80,000, rare diseases are indeed rare. That means that variants of rare diseases are sufficiently rare that identifying them requires a true expert with the benefit of many years working with larger populations.
In this case, Dr Azad Esack, Consultant Neurologist at Doctors Hospital, not only diagnosed a rare case of Guillain Barre Syndrome, but managed to identify it as an extremely rare variant. It’s all the more remarkable when you consider that those of greatest risk are commonly over the age of 50 and yet this was during a pregnancy.
In March 2023, a pregnant woman in her 3rd trimester began experiencing strange physical health symptoms that caused her and her family some concern. After consulting her Obstetrician, she was referred to an Ophthalmologist. Apart from the exception of no eye movement, an examination from the ophthalmologist returned normal. They needed another opinion. The symptoms she was experiencing were associated with multiple cranial nerve palsies. So, she was referred to DH’s Neurologist and Neurophysiologist, Dr. Azad Esack, with the following symptoms:
- Difficulty with speech
- Difficulty swallowing
- Double vision
- Weakness in arms
These are symptoms that can have many etiologies, including many that are far more common than Guillain Barre Syndrome. Her examination with Dr Esack, and subsequent investigations, established:
- No eye movements
- Marked bilateral weakness of the face
- Drooling/excess saliva
- Neck weakness
- Abnormal speech/difficulties with articulation
- Strength in arms significantly reduced (particularly in upper arms)
Additionally, she had reasonably good leg strength, though she experienced slight unsteadiness when walking.
The results were conclusive. Though the brain scan returned normal, extensive nerve testing gave significant clues to the diagnosis. Of note, when considering rare conditions is that they often present themselves with symptoms in common with more common conditions, without question reinforcing the importance of assessment by an experienced professional. Dr. Esack’s diagnosis was precise, and immunological testing verified his conclusion. Our patient had an unusual variant of Guillain Barre Syndrome (GBS) called Pharyngeal Cervical Brachial (PCB).
In 2010, Dr Esack wrote a paper on GBS, having diagnosed 250+ cases in the UK and Caribbean. With a 1 in 100,000 chance of developing GBS, most commonly in the over 50 age group, in a population of 44 million persons living in the Caribbean, GBS clearly occurs, but this variant, PCB, is, especially in a pregnant woman extremely rare and potentially a once in a career lifetime occurrence. In fact, this is only the second reported case in the English literature.
Following a definitive diagnosis, our patient at DH could receive appropriate treatment for this illness, in this case infusions of Intravenous Immunoglobulins (IVIG) over a number of days. This treatment was started within four hours of the patient presenting to DH. In addition, and specifically because we had both the mother and the baby to consider, the patient’s vital signs, including her respiratory parameters, were monitored very closely while in our care, and we additionally monitored her baby’s health too. Safety and thoroughness are both paramount concerns in this situation.
By our patient’s third day of treatment, we noticed her speech, swallowing, and upper limb weakness had improved. She was discharged six days after admission.
Our patient had this to say about her condition at this sensitive time for her, and her stay at Doctors Hospital “The beginning was rough as I tried to process what was happening. I felt frustrated, but they [Doctors Hospital team] helped bring it down to my level of understanding, and after that, everything went smoothly.”
Besides Dr Esack, our patient was cared for by a team of skilled nurses, pharmacy staff, imaging, and lab professionals and we’re proud to be able to offer a multidisciplinary team approach consisting of such highly qualified health professionals.
Since then, our patient has undergone follow-ups in our Outpatient Clinic and continues to show steady improvement. Her strength in her limbs, speech, and swallowing have all returned to normal. But still, healing takes time. When last seen, her eye movements had almost normalized, but she was still left with mild bilateral facial weakness. “But this is improving,” shares Dr. Esack.
GBS patient recovery can extend from a few weeks to several months and so it is also important to have access to a team you are familiar with and trust as you go through the process of recovery.
There are health lessons for all in this case too. Although many illnesses turn out to be common and mild, it isn’t always the case.
If you find yourself developing an acute (sudden onset) difficulty with speech, swallowing, or mobility (mainly if this has occurred after an infection [viral or bacterial]), seek medical attention sooner rather than later.
A message from our mom-to-be, “Everybody knows their body best. If you feel like you’re not your normal self. Just go see a professional. You never know. You could actually save yourself.”
PPS The patient delivered a normal healthy baby boy and both mother and baby are doing well
Doctors Hospital offers comprehensive services on-site at our hospital in George Town, at the top of Walkers Road, and we’re privileged to have state-of-the-art MRI or CT Imaging that’s the best in the Caribbean. Our pharmacy is dedicated to serving patients staying within our walls or quickly grabbing their prescriptions with an average 15-minute wait time but more importantly, as a hospital-located pharmacy, has the expertise to source and manage drugs that are unusual too.