Combined Guillain-Barré Syndrome and Acute Disseminated Encephalomyelitis: a Pediatric Case Report

Background: Guillain-Barré syndrome is caused by a demyelination of the peripheral nervous system. The main clinical features of the syndrome are peripheral weakness, ascending paralysis, and absent of deep tendon reflexes. Acute disseminated encephalomyelitis (ADEM) is a demyelination of the central nervous system, and its main feature is encephalopathy. Although both entities are of autoimmune origin, reports of acute combined central and peripheral nervous system demyelination are rare in pediatric population.

Case: we present a case of a previously healthy eight years old boy who was admitted to the hospital with fever, acute headache, double vision, and encephalopathy. One week before his admission he had gastroenteritis. Physical examination revealed difficulty standing, weakness in his upper and lower limbs and absent of deep tendon reflexes in lower extremities. Workup showed normal CBC and normal CRP. Lumber puncture showed 33 white blood cells, glucose 70 mg/dl, and protein 56 mg/dl.

Brain MRI revealed numerous white matter lesions with high signal intensity in T2 involving the brain and cervical spinal cord, suggestive of ADEM. He was treated with high dose corticosteroids. Despite of the treatment, he showed no improvement in his peripheral weakness. Following another lumber puncture, a significant elevation in protein (173 mg/dl) was detected. Nerve conduction test revealed axonal injury. Spinal MRI scan revealed enhancement of the cauda equina nerve roots.

He was treated with immunoglobulins, with improvement in his peripheral weakness.

Physicians should be aware that, even though uncommon, the co-existence of both entities is possible, and adjust therapy accordingly.