Acta neurologica Taiwanica

Complicated acute motor axonal neuropathy with delayed acute respiratory distress syndrome and rapidly progressive glomerulonephritis: a case report.

PMID 23479243


Acute motor axonal neuropathy (AMAN), a variant of Guillain Barre syndrome (GBS), is frequently induced by the antecedent infection of some atypical pathogen, such as Campylobacter jejuni, Mycoplasma pneumonia and some virus. It is generally accepted that corticosteroids and immunosuppressants are not recommended in patients with GBS including AMAN. However, if systemic autoimmune reaction developed, the principle of management might be changed. We report a young man who rapidly developed acute motor axonal neuropathy. Although plasma exchange had been given, the violent immunological reaction was unable to be controlled, prolonged leukemoid reaction and high level of autoimmunological titers, including C-reactive protein (CRP), rheumatoid factor (Rf), and antineutrophil cytoplasmic autoantibody (ANCA) persisted. Consequently, two months later, this patient developed acute respiratory distress syndrome (ARDS) and type 3 of rapidly progressive glomerulonephritis (RPGN) with rapid decline of renal function until immunosuppressants were given. AMAN combined with the violent systemic autoimmune reaction strongly indicated an uneven disease course and implied that only standard plasmapheresis is not sufficient and corticosteroids with immunosuppressant should be added in early stage.