[A case of optic neuropathy, recurrent transverse myelopathy and hypertrophic pachymeningitis associated with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA)].

Abstract

A 77-year-old woman developed recurrent transverse myelopathy of the thoracic cord about a year after acute retrobulbar optic neuropathy on the left. Neurological examination revealed paraplegia, total sensory loss down from the level of the sixth thoracic cord and sphincteric disturbances. Her clinical features resembled multiple sclerosis, but magnetic resonance imaging (MRI) of the brain and spinal cord revealed no evidence of demyelination or inflammation. MRI of the brain showed the tentorium cerebelli on the right of low signal in T1- and T2-weighted images with marked enhancement by gadolinium, indicating pachymeningitis. Characteristically, perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) together with antinuclear antibody was positive, whereas lupus anticoagulant and anti-cardiolipin antibody were negative. A high dose of corticosteroid including pulse therapy did not improve her symptoms. In the present case, the optic neuropathy, recurrent transverse myelopathy and hypertrophic pachymeningitis may have been caused by some common inflammatory processes related with p-ANCA.