Neck stiffness in two children with Guillain-Barré syndrome after Campylobacter jejuni infection.

Abstract

Sirs: The association of meningismus with enteric infections caused by Salmonella or Shigella is well recognized, and we can find two reports of an association between meningismus and Campylobacter jejuni enteritis [4, 5]. We here describe two children with GuillainBarre syndrome (GBS) after C. jejuni infection, who had meningismus. Patient 1. Four days after a 3day bout of watery diarrhea, a 15year-old boy developed ascending muscle weakness, paresthesias, and urinary retention (day 1). On day 4, he was alert, and his cranial nerve function was intact. Generalized areflexia and tetraparesis of Medical Research Council (MRC) grades 3–4 were found in his arms and 0–1 in his legs. He complained of severe pain from the buttocks to the legs, but sensory disturbance was absent. Neck stiffness was moderate, and the Kernig sign was elicited. No autonomic disturbance was found. Serum had a high antiGM1 IgG antibody titer of 1:4000 (normal, less than 1:500). CSF analysis showed 2 cells/μl and 57 mg/dl protein on day 3, and 12 cells/μl with 153 mg/dl protein on day 16. C. jejuni was isolated from a stool specimen taken on admission. Neck stiffness had disappeared by day 8. From day 8, he was treated with intravenous immunoglobulin (IVIg: 0.4 g/kg/day for 5 consecutive days). A nerve conduction study on day 9 showed inexcitable motor responses. Left facial palsy and incomplete limitation of left eye movement respectively appeared on days 14 and 16, after which both gradually disappeared. Leg pains were resolved by day 19. He had MRC grades of 2–3 in the arms and 2–4 in the legs on day 44. Ten months after onset, he could walk independently and had MRC grades of 4–5 in the arms and legs. Patient 2. A 6-year-old boy developed vomiting with fever. Six days later, he had pain in the popliteal fossa (day 1), and fever reappeared. Because of limb weakness he could not walk the next day. On admission (day 3) he was alert, and cranial nerve function was intact. He had tetraparesis of MRC grades 1–2 in the arms and the legs. Deep tendon reflexes were absent in the lower limbs, but preserved in the upper ones. He complained of severe leg pains, especially at the patellar fossae, but there were no sensory disturbances. Neck stiffness was obvious. The Kernig and Brudzinski signs were elicited. Passive neck flexion led to worsening leg pain. The nerve conduction study showed a low compound muscle action potential (4.0 mV) with normal distal motor latency (3.4 ms) and normal motor nerve conduction velocity (60 m/s) in the median nerve. No motor responses were evoked in the peroneal nerves. Serum had a high anti-GM1 IgG antibody titer of 1:16000. CSF analysis showed 3 cells/μl and a normal protein level on day 3, and 8 cells/μl with elevated protein (123 mg/dl) on day 11. He had no history of diarrhea, but C. jejuni was isolated from a stool specimen taken on admission. From day 4, IVIg was initiated, after which his neck stiffness and severe leg pain decreased and respectively had disappeared by days 10 and 20. There was temporary urinary retention and bowel dysfunction from admission to the end of IVIg therapy. From day 149, he was again treated with IVIg because of residual neurological deficits. He always used a wheelchair and still needed assistance to walk on day 169. Ten months after onset, he was able to run although his hands remained weak. To our knowledge this is the first report of meningismus on patients with GBS from whom C. jejuni was actually isolated. McKhann and coworkers [2], however, had already described patients with GBS of children and young adults in northern China who occasionally showed the resistance to passive flexion of the neck. The resistance to neck flexion disappeared within days of onset. The patients with GBS in northern China frequently had serological evidence of recent C. jejuni infection [1]. In one series of childhood GBS, moreover, onethird of the cases had meningismus [3]. These give us warning that some patients with GBS, especially of children, after C. jejuni infection, or both, can have a stiff neck in the early phase of the illness. Because the combination of neck stiffness and preceding C. jejuni enteritis is expected to occur occasionally, a large prospective study is needed to determine the causal relationship between them.