Guillain–Barre syndrome: a rare complication of leptospirosis and scrub typhus co-infection

Case report

A 20-year-old male presented with a chief complaint of fever and yellowish discoloration of eyes and skin for five days. Three days following admission, he developed motor weakness, initially involving both lower limbs which progressed to involve both upper limbs, within 24 h. Jaundice was present along with right hypochondrial tenderness. The neurological examination revealed hypotonia in all limbs, power of 3/5 in lower limbs and 4/5 in upper limbs with absent deep tendon reflexes. The plantar reflexes were flexor. Sensory, bowel and bladder functions were normal. On investigation, he had deranged liver and kidney functions. Antibodies to Orienta tsutsugamushi (scrub typhus-IgM) were detected by ELISA, and to Leptospira were detected using a microscopic agglutination test. Both infections were confirmed by polymerase chain reaction test to rule out any cross-reactivity. Other causes of acute febrile illness and jaundice were ruled out through relevant investigations. Abdominal ultrasound showed mild hepatomegaly. A magnetic resonance imaging (MRI) scan of the spine was normal. Cerebral spinal fluid (CSF) analysis showed 30 cells/µL and protein of 51 mg/dL. Electro-diagnostic studies were suggestive of acute inflammatory demyelinating polyradiculo-neuropathy, a type of GBS. Brighton’s criteria were fulfilled. Our patient was managed with doxycycline, cephalosporin and supportive measures. Intravenous immunoglobulins (IVIG) were not administered as symptomatic improvement was seen on the fifth day of admission. After 10 days, our patient could be discharged without any neurological deficit, having normal liver and renal parameters.

Discussion

GBS is an immune-mediated acute inflammatory polyneuropathy associated with antecedent infection. Its worldwide incidence is estimated as 0.8–1.9 cases per 100,000 per year. ¹ Both viral and bacterial infections are commonly implicated. Protozoal infections and non-infectious causes such as sarcoidosis, drugs, immunisation and surgical procedures have also been implicated. The pathophysiology of GBS following an infection is thought to be the result of antibodies mounting an immunological response, causing axonal inflammation leading to demyelination. An alternative explanation suggests nerve ischaemia by immunological mediators. ² Case reports of GBS in mixed infections have been a rarity. A mixed infection with malaria and scrub typhus has been reported by Rahul et al. ³

The clinical picture is dominated by rapidly progressive symmetric flaccid paralysis with diminished or absent deep tendon reflexes. Rarely, cranial nerve or sensory involvement may be the presenting complaint. GBS is a potentially life-threatening condition with 20–30% of patients developing respiratory failure requiring ventilatory support. Up to 60% develop autonomic disturbances. Despite optimal care, the mortality rate is almost 5%. ²

In an appropriate clinical context, an elevation in CSF protein (present in 50–66% of cases in the first week and > 75% of cases in the third week) without an elevation in white blood cells provides supporting evidence for the diagnosis. However, the CSF may be normal in first week of disease. Nerve conduction studies help in the diagnosis and to categorise GBS into various types.^4,5

Our patient, who lived in an area known to have sporadic cases of leptospirosis and scrub typhus, had no exposure to rat urine or contacts with mites, but developed a clinical picture of GBS one week following symptom onset and was eventually diagnosed to have concurrent leptospirosis and scrub typhus. In the absence of any other preceding infections or non-infectious cause, this concurrent infection was considered to be the trigger for development of GBS. It is difficult to be certain which infection was responsible for GBS in our patient, though the presence of concurrent infection may lead to an enhanced immune mediated inflammatory response.