A fifty-five-year-old man with hypertension and who used regular medications for anxiety came to the Emergency Room in Malmö, Sweden, complaining of dyspnea in 2009. He had arrived to Sweden by plane from Sri Lanka five days prior, where he resides a large part of the year. Upon exiting Sri Lanka, he was put into custody for almost 6 weeks under unhygienic conditions.
Already in Sri Lanka, but aggravated upon the return to Sweden, the patient experienced throat pain and shortness of breath. At the emergency room the patient presented with severe shortness of breath and fever of 39 °C. Throat inspection revealed swelling and greyish membranes. The patient deteriorated quickly with hypoxia and hypercapnia. He was intubated and put under ventilator support. Bronchoscopy showed greyish membranous plaques covering the larger part of the bronchus and partly occluding the left major bronchus. Serial X-rays showed progressive atelectasis of the left lung (Fig. 1). The membranes could mechanically be removed from its underlying layer and repeated bronchoscopies with lavage were performed.
Culture specimens were sent from larynx and bronchoscopy specimens as well as from a 1 cm2 skin ulceration. Due to suspicion of diphtheria, Loeffler’s tellurite media was used for culture. On day four from admission, the results from cultures showed growth of toxin producing C. diphtheriae, subsequently typed to non-gravis, both from the ulcer and from the respiratory tract. Serologies for Human Immunodeficiency Virus (HIV), hepatitis B and C and syphilis returned negative, as well as urine antigen tests for Streptococcus pneumoniae and Legionella pneumophila. The diphtheria strain was susceptible to both cefotaxime and erythromycin, which the patient was receiving since admission. It was in this situation judged too late for administration of DAT in relation to possible side effects and the duration of symptoms, and the patient was never administered this treatment. In the following weeks (day 5–14), the patient showed signs of improvement in infection control with decreasing C-reactive protein (CRP) and was afebrile, however the left lung remained deflated. The patient was under ventilator support for one month through tracheostomy performed on day seven. The prolonged time in ventilator was due to inability to recruit the left lung, despite repeated bronchoscopy and cleaning of greyish debris from the airways. A pleural catheter was also placed in the left inter pleural space with clearance of about one liter of transudative fluid. Bacterial cultures from the pleural fluid were negative.
Between day 14 and 16 the patient had changes in the electrocardiogram with T-wave inversions and short periods of ventricular tachycardia as well as elevated cardiac enzymes that resolved spontaneously (Fig. 2). An echocardiogram was performed with no significant pathology. Additionally, a passing increase in serum creatinine was noted during this period. No clear reasons for these adverse organ effects were identified and were judged to be Diphtheria toxin related. On day 19 the patient deteriorated with fever and increased purulent secretions from the airways. This was considered due to Ventilator Associated Pneumonia (VAP) with Methicillin Resistant Staphylococcus aureus (MRSA) which was successfully treated with intravenous vancomycin for two weeks. In the fourth week, the patient improved and could gradually be weaned off of the ventilator. The tracheostomy tube was removed on day 46 from admission and the patient was mobilized in the following week with physical therapy and was prepared to be discharged to a center for rehabilitation.
On day 55, however, the patient developed a gradual onset of neurological symptoms. First he got increasingly weaker voice and shortly thereafter weakness of the extremities, and increasing difficulties to breathe. From day 58, the patient quickly deteriorated with a complete paraplegia and respiratory failure ensued, requiring reintubation. Repeated neurographic examinations showed severe polyneuropathy with mixed demyelination and axonal loss. The pattern was not deemed to be consistent with Guillain-Barré, nor Critical Illness and was judged to be due to toxin effects of diphtheria toxin thus no neuroimaging was considered necessary. Furthermore, lumbar puncture showed no significant pathology.
On day 56, surveillance Bronchiole Alveolar Lavage (BAL)-culture was sent for extensive testing and culture due to respiratory deterioration. This subsequently showed growth of Mycobacterium tuberculosis (MTB) fully sensitive to rifampicin, isoniazide, pyrazinamide and ethambutol. MTB-PCR was negative. Bronchoscopy specimens for MTB culture and PCR had previously been sent for investigation on day two from admission, but was negative at that time. The patient was started on combination therapy for pulmonary tuberculosis (TB) on day 70.
The patient gradually regained motor function from day 80 and the patient could once again be weaned off of the ventilator. The sensory functions likewise gradually returned from the center to the periphery (Fig. 3). After an extended stay for mobilization, the patient was discharged to a rehabilitation clinic on day 91 from admission. On follow up after three and six months, the patient continued to improve in motor and sensory functions, The patient was followed for three years reporting only minor polyneuropathic symptoms in his feet, but had resumed his daily activities with no motor impairments.