Neuromyelitis optica phenotype associated with therapy-responsive acute peripheral neuropathy

A 51-year-old Hispanic woman with a history of Churg-Strauss syndrome (in remission for 20 years)–associated mesangial glomerulonephritis and end-stage renal disease with a recent renal transplant and previously cured cytomegalovirus (CMV) infection who was on a tapering dose of tacrolimus presented with sudden onset of lower extremity numbness. Symptoms progressed to complete paraplegia and sensory loss over 24 hours. The following day, ascending paresis extended to bilateral upper extremities, with high thoracic sensory level and complete blindness. Initial MRI revealed contrast enhancement of bilateral prechiasmatic optic nerve as well as T2 hyperintensities involving the central gray matter of the entire spinal cord with associated edema. CSF analysis documented neutrophilic pleocytosis (182 neutrophils per mm3) and increased total protein level (142 mg/dL) without oligoclonal banding. Bacterial, fungal, and mycobacterial cultures from the CSF were negative, as was PCR for CMV, Epstein-Barr virus, herpes simplex virus, human herpesvirus 6, and varicella-zoster virus. Serologic autoimmune assessment, including onconeuronal, anti-neutrophil cytoplasmic antibody, and other rheumatologic, Lyme, and aquaporin-4 (AQP4) antibody testing, was negative. Moreover, testing for sarcoidosis and HIV antibody was unrevealing. Nerve conduction studies (NCS) performed 2 weeks after symptom onset documented severe reduction in compound motor action potential amplitudes (worse in the ...
Source: Neurology Neuroimmunology and Neuroinflammation - Category: Neurology Authors: Tags: EMG, Devic's syndrome, Optic neuritis; see Neuro-ophthalmology/Optic Nerve, Transverse myelitis, Autoimmune diseases Clinical/Scientific Notes Source Type: research