This 40 year old African-American woman presented with optic neuritis five years prior, with recovery to 20/20 after IV methylprednisolone (IVMP) (). Three months later, dysesthesias in 4 extremities, mild sensory ataxia, and urinary incontinence developed. Cervical-spine MRI revealed longitudinally-extensive transverse myelitis (LETM) and serum was positive for NMO-IgG antibodies (1:1920). Treatment included IVMP for this relapse. Azathioprine and daily prednisone were added. Azathioprine was adjusted to lymphocyte counts of 500–1000 K/mm3.5
She relapsed two months later, with five total episodes of transverse myelitis in the first year, each treated with IVMP. Azathioprine was discontinued and mycophenolate mofetil 1000 mg twice per day with daily prednisone was utilized for 18 months. She experienced four additional sensory and bladder relapses, unaccompanied by urinary tract infection. Motor testing was always normal over the first three years.
Figure Timeline of clinical events and treatments with Expanded Disability Status Scale. Relapses treated with IVMP are shown by red dots, relapses treated with PLEX are noted (red dots are IVMP, blue dots were non-relapse exam visits, green dot is PLEX without (more ...)
Due to continued disease activity, rituximab was administered. Beginning five months after rituximab, and with confirmed zero CD19+ cells, she experienced 4 additional relapses over five months, stabilizing after each. However, these culminated in persistent paraplegia.
Nine months following rituximab, B cells began to return (CD19 was 9, normal 79-545). Fourth-line treatment options were discussed due to concern for quadriplegia and respiratory compromise. Mitoxantrone was not chosen due to dose limitations and risks of cardiotoxicity and malignancy. 6,7
Cyclophosphamide was not used due to risk of infection among other side-effects. Alemtuzumab was selected to suppress mononuclear immune system cells and because it is well- tolerated with promising early results in relapsing MS. She received 5 days IV alemtuzumab at 12 mg/kg/d, with five days IVMP.
Six weeks following alemtuzumab, a relapse manifested by worsening dysesthesias culminated in apnea and intubation. MRI showed an expansile, contrast enhancing LETM. CD4 count was 30 cells/mm3 (normal 393-1607). She received PLEX and was extubated. Four months into alemtuzumab, aphasia, dysarthria, lethargy, and moderate right arm weakness developed. Brain MRI revealed three large, discreet regions of T2 signal abnormality with patchy enhancement, the largest being 33 cm3 (). Thoracic cord demonstrated an enhancing expansile T2 lesion. Brain biopsy, performed to exclude opportunistic infection and malignancy, confirmed a demyelinating process. CD4 count was 133, and CD19 was 115. Over the next 5 months, she had 2 confirmed relapses and received IVMP. Mycophenolate and low-dose prednisone were added, with no further clinical relapse after 8 follow-up. She currently resides in a nursing facility, transfers with a lift, and cannot feed herself.