A rare neurological complication of typhoid fever: Guillain-Barre' syndrome.

Guillain-Barre' syndrome is a rare complication of typhoid fever, and only a few such cases have been reported in the pediatric age group. We report a young boy with blood culture proven typhoid fever that developed this very rare neurological complication quite early in the course of the disease. Following treatment with intravenous antibiotics and intravenous immunoglobulin, he improved.

Introduction

Salmonella group of organisms causes typhoid fever. It has a high prevalence in tropical countries. The typical symptoms may not be seen in all patients and the disease may have unusual manifestations.[1] The incidence of neurological manifestations in typhoid fever varies widely. We recently had a case of typhoid fever that developed Guillain-Barre’ syndrome (GBS) quite early in the course of the disease. This case report is to highlight a rare neurological complication of typhoid fever that pediatricians need to be aware of.

Case Report

An 18-month-old male child was admitted in our institution with a history of fever for 4 days, progressively increasing weakness of all four limbs and difficulty in respiration for 1 day. The child was appropriately immunized for age and was developmentally normal. On general examination, he was pale, febrile with heart rate of 110 beats/min, blood pressure of 78/56 mmHg and respiratory rate of 36/min with paradoxical type of respiration. Both lung fields were clear on auscultation. On abdominal examination, liver was found to be palpable 5 cm below costal margin and spleen 2 cm below the costal margin. The cardiovascular examination was normal. On neurological examination, he was conscious, oriented with anxious looks. Gag reflex was intact, and all other cranial nerves were normal. Motor examination revealed flaccid quadriplegia. Power in both upper, as well as lower limbs, was found to be 0/5. Sensory system was intact. There was no bladder or bowel dysfunction.

Laboratory investigations on admission showed hemoglobin of 10.3 g/dL, total leukocyte count of 3520/mm3 with differential count of polymorphs 50%, lymphocytes 43%, monocytes 5% and eosinophils 2%. Platelet count was 1.5 lakhs/mm3. Peripheral smear showed microcytic hypo chromic anemia with no malaria parasite. Liver and kidney function tests were normal. Serum electrolytes were normal with potassium being 5.1 meq/L. Blood gas analysis showed pH- 7.48 and the peripheral saturation of 97% on room air. Chest X-ray was normal. Cerebrospinal fluid analysis showed albumin-cytological dissociation, 10 cells, all lymphocytes, sugar-89 mg/dl against blood sugar of 108 mg/dl, protein-171 mg/dl.

The provisional diagnosis of GBS was kept and child was transferred to the pediatric intensive care unit. Electrophysiological study showed pure motor axonal neuropathy. He was given intravenous immunoglobulin (0.4 g/kg/day for 5 days) and other supportive care. Blood culture report received on 3rd day of admission, showed nalidixic acid resistant Salmonella typhi, sensitive to ceftriaxone. Serum was sent for Widal test as well at this point which came out to be positive (“O” as well as ‘H’ titers being > 1/160). The child was put on injectable ceftriaxone and became afebrile by day 10 of illness. The child did not require ventilator support and was discharged on day 14 with the final diagnosis of GBS secondary to typhoid fever.

At discharge, he was able to sit with support and the diaphragmatic paralysis recovered; the power in all limbs was 3/5. Follow-up at 4 weeks showed power of +3/5 in all the limbs and was able to sit without support.

Discussion

Typhoid fever is a common infectious disease in developing countries. Neurological complications are not uncommon. In children, known and reported neurological complications are encephalopathy, meningism, spastic paralysis-cerebral origin, convulsions, meningitis, parkinsonian syndrome, sensory motor neuropathy, cerebellar involvement, and schizophrenic psychosis.[12] However, GBS is not common.

Guillain-Barre syndrome is an immune-mediated polyneuropathy that has often been associated with a variety of infectious agents such as bacteria, and virus.[23] There are, however, very few reports of GBS associated with typhoid fever in pediatric age group.[456] Datta et al. also reported a case of typhoid fever in a 10-year-old girl, who developed GBS subsequently as a complication of typhoid fever.[7] The present case report differs from the previous reports in age of presentation (presented at the age of 1.5 years), early development of GBS following typhoid fever (by day 4 of illness) and male sex (other cases were females) [Table 1].

Table 1

Case reports of GBS secondary to typhoid fever in children

This case report attempts to highlight the fact that typhoid fever can be associated with unusual neurological complication like GBS quite early in the course of the illness; also, prognosis in such cases seems to be relatively good. Typhoid fever should be kept in mind in any child having fever and features of polyneuropathy, since early definitive treatment may play a vital role in recovery.