Neuropediatrics 2006; 37 - CS2_5_5
DOI: 10.1055/s-2006-945555

TREATMENT OF PERIPHERAL NEUROPATHIES

M Ryan 1
  • 1Children's Hospital at Westmead, University of Sydney, Westmead, NSW, Australia

Inherited Neuropathies: Advances in this field in recent years have been mainly in the area of genetic diagnosis. Interest in treatment of the inherited neuropathies has lagged behind the remarkable expansion in our knowledge of their genetic basis. A number of agents have recently been suggested as having a potential role in amelioration or reversal of axonal dysfunction or demyelination in the inherited neuropathies. Attempts to demonstrate efficacy of these agents will be hampered by the absence of detailed natural history data and established outcome measures for clinical trials in the paediatric neuropathies.

Immune Neuropathies: Guillain-Barré syndrome (GBS) is by far the most common acquired inflammatory neuropathy of childhood. There are an increasing number of clinical and neurophysiologic subtypes of GBS, which vary in underlying pathophysiology and responsiveness to immunosuppressive therapies. Supportive treatment remains the mainstay of treatment of paediatric Guillain-Barré syndrome. Improved mortality rates in childhood GBS are attributable to improved management of respiratory failure and other complications of this disorder. Pain, autonomic manifestations and systemic complications of immobility may be under-recognised in paediatric GBS and can cause significant morbidity.

Plasma exchange (PE) and intravenous immunoglobulin (IVIG) are of similar efficacy in treatment of most forms of GBS. Treatment with IVIG hastens recovery from GBS, although an improvement in ultimate outcome has not been demonstrated. The optimal dose regimen for IVIG administration is still not clear.

Chronic inflammatory demyelinating neuropathy is uncommon in childhood. Accepted first-line treatments of paediatric CIDP include IVIG and prednisone. Refractory cases may respond to newer immunosuppressive therapies such as mycophenolate mofetil and Remicade.