Polyradiculoneuritis with Myelitis: A Rare Differential Diagnosis of Guillain-Barré Syndrome

Abstract

Aim

To describe the symptoms, signs, findings and prognosis in children suffering simultaneously from polyradiculoneuritis and myelitis.

Methods

Retrospective review of eight patients aged 2 to 13 years out of 210 patients with polyradiculoneuritis reported from 70 hospitals. Diagnostic criteria for polyradiculoneuritis were: flaccid paresis with loss of tendon reflexes, increased CSF protein and slowing of nerve conduction velocity. Criteria for myelitis were: severe and persistent bladder dysfunction, a sharply defined sensory level and/or evolving spastic paresis, with or without myelitic changes in spinal MRI.

Results

In the disease's earliest stage it was difficult to differentiate polyradiculoneuritis with myelitis from classical GBS. However, onset was often unusually rapid compared to GBS. Five patients developed a sensory level and seven suffered from severe bladder dysfunction. Four of the six children studied showed focal myelitic changes in MRI. All seven children with sufficient follow-up remained with residual paresis and significant long-term motor deficits.

Conclusion

Due to its severe long-term prognosis, polyradiculoneuritis with myelitis must be differentiated from classical GBS. In the disease's early stage, the detection of a sensory level, severe bladder dysfunction and an unusually rapid onset can be helpful. The effect of high-dose corticosteroids is not yet clear. After the acute phase, most children require extended rehabilitation.

Atypical GBS - Variant GBS - Polyradiculoneuritis - Myelitis - Childhood

References

• 1 Alam S M, Kyriakides T, Lawden M. et al . Methylpredisone in multiple sclerosis: a comparison of oral with intravenous therapy at equivalent high dose.  J Neurol Neurosurg Psych. 1993;  56 1219-1220
• 2 Asbury A K, Cornblath D R. Assessment of current diagnostic criteria for GBS.  Ann Neurol. 1990;  27 (Suppl) 21-24
• 3 Chopra A, Rana P V, Narayanaswamy A S, Bajpai C P, Madan V S. Neurological complications following acute viral conjunctivitis: a new profile.  Tropical & Geographical Medicine. 1986;  38 197-202
• 4 De Gans J, Portegies P, Tiessens G, Troost D, Danner S A, Lange J M. Therapy for cytomegalovirus polyradiculomyelitis in patients with AIDS: treatment with ganciclovir.  AIDS. 1990;  4 421-425
• 5 Feasby T E, Hahn A F, Koopman W J, Lee D H. Central lesions in chronic inflammatory demyelinating polyneuropathy: an MRI study.  Neurology. 1990;  40 476-478
• 6 Jensen E L, Christensen B I. Acute myelo-meningo-radiculitis caused by the spirochaete Borrelia burgdorferi. .  Ugeskrift for Laeger. 1992;  154 3512-3513
• 7 Knebusch M, Straßburg H M. Acute transverse myelitis in childhood: nine cases and review of the literature.  Dev Med Child Neurol. 1998;  40 631-639
• 8 Korinthenberg R. Chronic inflammatory demyelinating polyradiculoneuropathy in children and their response to treatment.  Neuropediatrics. 1999;  30 190-196
• 9 Korinthenberg R, Schulte Mönting J. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study.  Arch Dis Child. 1996;  74 281-287
• 10 Lahat E, Pillar G, Ravid S, Barzilai A, Etzioni A, Shahar E. Rapid recovery from transverse myelopathy in children treated with methylprednisolone.  Pediatr Neurol. 1998;  19 279-282
• 11 Ormerod I E, Waddy H M, Kermode A G, Murray N M, Thomas P K. Involvement of the central nervous system in chronic demyelating polyneuropathy: a clinical, electrophysiological and magnetic resonance imaging study.  J Neurol Neurosurg Psychiatry. 1990;  53 789-793
• 12 Plasma Exchange/Sandoglobulin Guillain-Barré syndrome trial group . Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome.  Lancet. 1997;  349 225-230
• 13 Whiteman M L, Dandapani B K, Shebert R T, Post M L. MRI of AIDS-related polyradiculomyelitis.  J Comp Assis Tomogr. 1994;  18 7-11
• 14 Willis J, Van den Bergh P. Cerebral involvement in children with acute and relapsing inflammatory polyneuropathy.  J Child Neurol. 1988;  3 200-204
• 15 Wilmshurst J M, Walker M C, Pohl K R. Rapid onset transverse myelitis in adolescence: implications for pathogenesis and prognosis.  Arch Dis Child. 1999;  80 137-142