Case of the Month

June 2016

“Just a sore glans in the throat”…

Albie de Frey WORLDWIDE TRAVEL MEDICAL CONSULTANTS and the School of Public Health, Faculty of Health Sciences, University of the Witwatersrand

A travel health advisor to a large South African construction company that has an operation in Zambia, receives an email report on the 2nd February from the risk manager regarding a 60-year old white male South African mine worker who was admitted to a mine hospital in the Copper belt in Zambia on 1 February 2016. He states that the patient was complaining of “…a swollen tongue and the glans [sic] in his throat…” and “…difficulty in speaking and eating”. The patient had a raised blood pressure on admission and was placed on treatment for hypertension.

The e-mail continues: “The doctor told him that hehas a nerve spasm in his neck / throat area that causes this condition and he was given medication to combat this. This morning his bp [sic] was 160/80. He feel much better and is also not talking in a slurred way”

The case is reported as a “Medical Incident”, not an emergency and a written medical report is promised, “…as soon as I receive it…”

Question 1: What is the most critical question to be answered first?

Answer to Q1

A lot more medical information is needed to make a decision regarding the further management of the case but the most critical question is whether this is an immediately life-threatening situation and whether the local level of care is appropriate.
Medical infrastructure and standards of care in Zambia are constrained, more so in the current financial situation in the commodities market.
Does the patient present with symptoms that may point to a life-threatening condition?

Question 2: With the limited medical information at hand, what is included in the differential diagnosis?
Answer to Q2

The first set of symptoms noted is a sore throat and swollen cervical glan(d)s. This could be due to:
  • the common cold
  • a sinus infection
  • post-nasal drip
  • acid reflux
  • streptococcal pharyngitis
  • tonsillitis
  • peri-tonsillar abscess
  • diphtheria
It is unclear at this point but would be useful to know whether he is pyrexial or not.

He has difficulty speaking and eating - this could be due to some of the above but one also has to consider
  • tetanus
  • viral encephalitis
  • bacterial meningitis
  • West African trypanosomiasis
  • Botulism
  • in the context of the Copper belt but less likely in this case, rabies.
Brain stem infarction looks plausible in the setting of hypertension but the noted blood pressure is not particularly high and his speech had improved overnight. The stated doctor’s explanation of a “…nerve spasm in the neck” fought off with “medication” does not add much towards a definitive answer other than posing the question whether his symptoms may be attributed to the side-effects of some as yet unknown medication, e.g. metoclopramide or a phenothiazine?
The question remains whether the patient has a life-threatening condition for which he should be medevaced or whether he has a minor upper airway infection for which he is unnecessarily hospitalized.

Question 3: In the absence of more clinical information forthcoming, what other source of medical information would be useful?
Answer to Q3

As part of comprehensive corporate travel health risk management, all expatriates should have a pre-deployment medical examination, travel health education and destination and risk exposure appropriate immunisations.

His medical records reveal that he is a 60-year old electrician from the Kwa-Zulu Natal south coast, first seen for pre-deployment screening in 2014.

He is aknown well controlled hypertensive on Coversyl® (perindopril) and simvastatin for hypercholestrolaemia. Stopped smoking several years ago.

He had a right total knee replacement at the end of 2015.

Previous postings include Angola and Mali several years previously.

He is up to date with vaccines against yellow fever, tetanus, pertussis, diphtheria, polio, hepatitis A and B, measles, mumps, rubella, meningococcal disease (ACYW) and typhoid. He received a single pre-exposure dose of rabies vaccine.

He had been advised to avoid mosquito bites and had been prescribed doxycycline as malaria prophylaxis which he had stopped taking…

On the afternoon of 2 February the risk manager manages to pry from the ward sister that the patient is being treated with “Amlodenk” for hypertension, prednisolone 40mg and a multivitamin… The doctor in charge will only complete a medical report the next day - or on the day of discharge.

The risk manager decides thatthe patient is being treated for hypertension and “an allergy that makes his tongue swell up from time to time” (Hence the prednisolone). The patient is not getting worse but also not improving.

Question 4: What would you further management plan be?
Answer to Q4

One needs to take a calculated risk - the patient clearly has something wrong but there is no attempt to make a definite diagnosis.

It does not appear to be an obvious infectious disease - still no report of a documented fever and the patient has not been placed on an antibiotic. Malaria has neither been mentioned nor clearly excluded. “Just an allergy or sore throat” does not warrant continued hospitalisation or time off work.

The patient is insured but his condition does not obviously call for the dispatch of an air ambulance.

The patient is taken from hospital and flies home on a commercial airline flight. He goes from the airport to the clinic where he presents apyrexial, GCS 15/15, pupils equal and reacting to light. Blood pressure 180/100 mm Hg, pulse 54 beats per minute, sinus rhythm. There is no evidence of jaundice, anaemia or cyanosis but he has a slightly flushed appearance.

The medical officer reports motor and sensory nerve fallout in the hypoglossal, facial and vagus nerves. Weakness of the tongue, left worse than right. Absent gag reflex, slight dysarthria and some dysphagia with drooling.

There is no neurological loss in the upper or lower limbs.

Question 5: It is decided to refer him to a physician. What is the provisional diagnosis and what should be done to confirm the diagnosis?
Answer to Q5

The patient is referred as a bulbar infarct / incident. Known risk factors are hypertension and hypercholestrolaemia.

The specialist notes his blood pressure as 180/100, pulse 80/min, SR. All pulses present and equal. Apyrexial with a respiratory rate of 12 per minute.

He has dysarthria and nasal speech. Normal cardiorespiratory system. No carotid bruit. Cognitive function intact, cranial nerves normal, no glossal or palatial paralysis. Normal peripheral motor and sensory function, reflexes all normal.

An MRI scan demonstrates small old ischaemic infarcts in the left internal capsule, no evidence of recent infarction. Chest X-ray is essentially normal, cardiac echography shows evidence of long standing hypertension with diastolic dysfunction. No mural thrombi demonstrated.

His full blood count is normal with an ESR of 3 mm in the first hour. The CRP is 2mg/L, normal renal and liver function. The total cholesterol is 5,1 mmol/L and LDL cholesterol 3,1 mmol/L. Blood glucose is 6,4 mmol/L.

The physician concludes that he had a small transient ischaemic attack involving the bulbar region and that he can be discharged on his previous chronic medication but with much better compliance. According to her he can return home for the weekend to the KZN south coast and return to Zambia the following week.

Question 6: Is this diagnosis and proposed managementdecision correct? If not, why not?
Answer to 6

The diagnosis seems reasonable taking all the known facts into consideration. However, it must be borne in mind that it would appear at the time of discharge that the patient did suffer a transient ischaemic attack of the brain stem and has evidence of a likely previous internal capsule infraction. As he would not be returning home close to the treating medical team and would eventually return to Zambia with limited medical care on hand, the team decided to keep him in hospital in Johannesburg overnight whilst his employer arranged a flight to Durban for the next day.

Thetravel health advisors insisted on seeing the patient the next week prior to his departure to Zambia to avail themselves of the patient’s actual condition at the time of return.

When seen by the physician in hospital the next morning he had no neurological fall out whatsoever and was discharged.

Two hours later the ward staff informed the physician that the patient had difficulty speaking and swallowing.

The patient was referred to a neurologist who confirmed the diagnosis of myasthenia gravis with a blood acetylcholine receptor antibody test and pyridostigmine challenge.

Myasthenia gravis is an autoimmune disease where antibodies produced by the body’s own immune system, interfere with the receptors for acetylcholine at the neuromuscular junction, and this prevents muscle contraction from occurring. It is characterised by varying degrees of skeletal muscle weakness, and although any voluntary muscle may be affected, those that control eye and eyelid movement, facial expression, and swallowing are most frequently affected. The onset of the disorder may be sudden and symptoms often are not immediately recognised as myasthenia gravis. Weakness of the eye muscles may be the first sign in some, but in others, difficulty in swallowing and slurred speech may be the first signs. A myasthenic crisis occurs when the muscles that control breathing are most affected and weaken to the point that respiration is inadequate, creating a medical emergency that requires a respirator for assisted ventilation.

A CT scan chest did not show any evidence of a thymoma and there wereno other signs or symptoms of other neoplastic disease.

The patient was discharged home on a maintenance dose of pyridostgmine in addition to his other chronic medication.

In lieu of the risk of a myasthenia crisis and the concomitant risk of sudden onset respiratory arrest in a region with limited medical expertise and infrastructure he was declared unfit to return to work in Zambia.
Learning Points
  • “Is the patient sick, or not sick?” is a critical question in everyday medical practice. More so if the patient is located several hundreds of kilometres, phone calls and e-mails away.
  • Expatriate mining and construction workers are general speaking a tough resilient bunch that do not easily lie down – if they say something is wrong, something is wrong.
  • Pre-deployment screening with good record-keeping and easy access to the records in the event of a medical emergency in the host country is of vital importance - it assists in medical management decision making regarding pre-existing illness, chronic medication, vaccine preventable disease risk, chemoprophylaxis and travel health insurance options.
  • Inter-colleagial communication and cooperation is extremely important across borders as well as across wards and consulting rooms - sharing information and discussing signs and symptoms that do and don’t fit is in the patient’s best interest.
  • Not every disease in an expatriate / traveller in the tropics is an infectious disease.
  • Uncommon and unusual diseases may present in the traveller just as it does at home.
  • Expatriates with newly diagnosed chronic illness or recuperating from an acute illness / surgery should never be redeployed in haste - err on the side of caution. It is easy to review an apparently septic surgical incision or adjust medication if the patient resides around the corner - not so if they are a thousand or more kilometres away.
  • A new diagnosis may have serious consequences for the patients employability abroad, may even lead to job loss and needs to be carefully considered.

1. http://who.int/ith/other_health_risks/infectious_diseases/en/

2. Country disease profile - zambia in shoreland travax,

3. Myasthenia Gravis. Author: Aashit K Shah, MD, FAAN, FANA; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE

4. Myasthenia gravis fact sheet. national institute of neurological disorders and stroke.


6. http://www.uptodate.com/contents/myasthenic-crisis

7. http://www.healthmap.org/site/diseasedaily/article/yellow-fever-deaths-reach-250-angola-42616

Thank you to my colleague, Dr Sheryl Kay and Dr Julia Botha, specialist physician, Wilgeheuwel Hospital for taking care of this patient and for sharing clinical information with me.

Thank you to Dr Garth Brink and Ms Lee Baker for reviewing the content and providing valuable additions.

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