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Guillain-Barré syndrome (GBS) classically presents as ascending symmetric areflexic weakness with positive sensory symptoms. Recently, we managed a child presenting with unusual posture and hyperextension of the whole spine.
A 9 year boy presented with inability to hold books and write, and a limp. Over 12 hours he had developed tingling sensations and pain in the calf muscles; pain progressed to involve the neck and back by 24 hours and he was unable to flex his neck and extend his limbs. During the next two days weakness increased, especially of the lower limbs. By day 4, he had developed hyperextension of the cervical and thoracolumbar spine with flexed and adducted limbs. On day 6 when he presented to us, vital signs including blood pressure were normal and remained so during the hospital stay. He had painful restriction of passive extension at all joints; motor power and tone could not therefore be assessed. He had bilateral symmetrical weakness: shoulders (abductor, adductor, 2/5), elbows (flexor 3/5, extensor 2/5), wrists (dorsiflexor, palmarflexor, 2/5), finger flexors (2/5), hand grip (20–30%), hip flexors (2/5), knees (flexor, extensors, 2/5), ankle (0/5), toes (0/5). Deep tendon reflexes were absent except for the biceps, which also disappeared by day 12. Cremastric and abdominal reflexes were present; plantars were absent bilaterally. The spine was normal except for hyperextension of the cervical and thoracolumbar region. Respiratory muscles, higher mental functions, speech, cranial nerves, and bowel and bladder functions were normal. A plain radiograph of the spine showed mild thoracic lordosis. Cerebrospinal fluid examination on day 11 showed high protein (95 mg/dl). On day 12, spinal hyperextension and abnormal limb postures disappeared following improvement in pain as a result of analgesic therapy. Kernig’s and Brudzinski’s signs could be elicited. Lasegue’s sign1 and the straight leg raising test were also positive. Symmetrical hypotonia became obvious.
GBS was suspected in view of progressive bilateral symmetrical weakness, severe radiculopathy, and albumino-cytological dissociation.2 Nerve conduction studies, performed on day 23, showed reduced nerve conduction velocity in the motor nerves. Sensory nerves were normal. We could not determine F wave conduction velocity. The pain and tenderness subsided gradually. With regular physiotherapy the neck became soft, motor power improved, and he was able to sit unsupported by day 20. Four months later, neurological examination was normal. A stool culture for poliovirus was negative.
Children with GBS frequently have pain in the back and lower limbs, which is aggravated on the straight leg raising test in most of them.3 These pain syndromes are attributable to radiculitis,4 an early and predominant feature in GBS.5 Prominent radiculitis in this case might have led to severe pain in the back, causing generalised paravertebral muscle spasm. This resulted in the unusual posture of hyperextension of the spine.