013 Spinal cord biopsy in neuromyelitis optica: a case report

We report a case of longitudinal extensive myelopathy (LEM) in a previously-well 31-year old male electrician who presented with a 2-month history of progressive bilateral weakness of the lower limbs, urinary difficulty and constipation. There were flexor spasms at the hips, ankle clonus, and Hoffman’s, but no cranial nerve, signs. Pain sensation was reduced below the nipples. MRI images showed expansion of the spinal cord from C3 to T2, with extensive T2 abnormalities, irregular post-contrast enhancement and cystic changes, suggesting a radiological diagnosis of pilocytic astrocytoma. Laminectomy at C3/C4 revealed no tumour. Biopsies were taken where an enhancing lesion was seen on the MRI study. Histopathology revealed gliotic changes affecting white matter tracts, with foci of chronic inflammation containing scattered CD3+ T, and rare CD20+ B, lymphocytes. There was no evidence of malignancy. HIV, syphilis, and HTLV-I serologic studies were negative. The patient was treated with prednisolone and azathioprine and was ambulant unassisted 18 months later. Serologic studies for anti-AQP4 IgG autoantibodies were unavailable at the time of presentation, but were subsequently found to be positive in the patient’s serum. While LEM is frequently seen in neuromyelitis optica, very few cases of the spinal cord pathology have been published.