A Tale of Complicated Enteric Fever: Common Disease With Unusual Complications

To the Editors: Enteric fever, caused by Salmonella typhi, is a highly prevalent infectious disease in the Indian subcontinent. Children usually present with high-grade fever and gastrointestinal symptoms, and it can affect any organ. Neurological complications are relatively uncommon among children which include delirium, encephalopathy, meningismus, meningitis, seizures, psychosis, raised intracranial pressure, cerebellar ataxia, chorea, and deafness.1,2 Landry-Guillain-Barre syndrome (LGBS) is an autoimmune disorder, that results from B-cell and T-cell activation and production of antibodies directed against peripheral nerves. Several infectious agents (Campylobacter jejuni, cytomegalovirus and Epstein-Barr virus) trigger antibody production. LGBS is an uncommonly reported complication of enteric fever in children. The LGBS associated with enteric fever usually presents with an active infection, which could be due to different sets of antibodies involved in the pathogenesis.3 The management of LGBS includes intravenous immunoglobulin (IVIg) and/or plasmapheresis in addition to supportive care. The neurological recovery in LGBS usually occurs over 4 weeks to 6 months. However, LGBS associated with enteric fever improves rapidly with intravenous antibiotics with or without IVIG. Hemophagocytic lymphohistiocytosis (HLH) can also rarely occurs as a complication of enteric fever in children4 and its management includes intravenous antibiotics and/or IVIG/steroids. We report a 9-year-old girl who presented with 10 days of high-grade fever and loose stools; an episode of seizure on day 5 followed by lethargy; progressive ascending weakness involving all 4 limbs, drooping of eyelids, difficulty in speaking for 5 days and urinary retention for 1 day. There was no history of trauma, recent dog bite or vaccination. Examination revealed a febrile child with stable hemodynamics, pallor, icterus, hepatomegaly and mild splenomegaly. On neurological examination, she was conscious, oriented to time, place, and person; had partial ptosis, bulbar weakness (low volume speech, swallowing difficulty), flaccid quadriparesis (Medical Research Council Scale sum score of 10/60), areflexia, single breath count of 20, intact gag reflex and normal sensory system. She was started on intravenous ceftriaxone and nasogastric feeds and shifted to the Pediatric intensive care unit for mechanical ventilation and neurological monitoring. Investigations revealed anemia, leukocytosis, thrombocytopenia and transaminitis. Her renal function and serum electrolytes were normal. Nerve conduction study revealed decreased motor compound muscle action potential and normal sensory findings suggestive of acute inflammatory demyelinating polyradiculoneuropathy. MRI spine revealed thickening and enhancement of cauda equina nerve roots. MRI brain was normal. In view of persistent fever spikes and hepatosplenomegaly, the workup was performed for HLH which revealed persistent thrombocytopenia, worsening leucopenia, hypertriglyceridemia, hyperferritinemia and hypofibrinogenemia. The Widal test showed a 4-fold increase in antibody titers (1:320 on day 3 and 1:2560 on day 14) and blood culture grew Salmonella typhi sensitive to ceftriaxone. Enteric fever with LGBS and secondary HLH/hyperferritinemic sepsis were considered. The specific treatment included IVIG (2 g/kg over 2 days). There was improvement in neuromuscular weakness (Medical Research Council score 40/60) and laboratory parameters over the next 5 days, became afebrile after 10 days, and received antibiotics for 14 days. She was discharged with a normal neurological status on day 16 and was doing well at follow-up after 14 days.