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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | NMO (Neuromyelitis Optica) | NMO Spectrum Disorder | 5A0A Case 5 History - 1
Case 5 History (AANP 2009 - 7) ---- This 65 year-old woman carried the diagnosis of relapsing-remitting multiple sclerosis and a peripheral neuropathy for the last 13 years of her life. She had a past medical history of transverse myelitis, neurogenic bladder with an indwelling catheter, constipation, leg spasms, hypothyroidism, hypertension, hypercholesterolemia, and gastroesophageal reflux disease. She had been treated with immunosuppressive/modifying agents that included Copaxone, Novantrone, Rebif, Tysabri, and prednisone. One of her cousins had multiple sclerosis. Two months prior to hospitalization, she presented to the Emergency Department for burning, tingling, freezing “pins and needles” sensation of both hands with hand numbness. Tysabri was discontinued. Two months following this episode, the woman presented to the hospital with acute onset of left hemiplegia, aphasia, and altered consciousness. Within 24 hours, she rapidly deteriorated, and required intubation and mechanical ventilatory support. MRI of the head revealed diffuse ventricular prominence with bilateral linear to nodular periventricular enhancement and abnormal FLAIR signal in the periventricular white matter and basal ganglia. CSF revealed normal glucose, protein of 135 mg/dl, 6 WBCs/uL (3 lymphocytes, 3 monocytes), no oligoclonal bands, and no infectious agents by cultures or PCR. CSF flow cytometry revealed T-cells with a normal CD4:CD8 ratio, normal antigen expression, and no B-cells. Serologic studies were negative for NMO antibodies at that time. The ESR was 58 mm/hr, ANA was 1:80, and activated C3 value was 1215 ng/ml. Biopsy of the right frontal cortex and caudate nucleus which showed “vacuolation and nonspecific changes”. Medical treatment included plasmapheresis and high dose steroids. The patient remained unresponsive and died during the hospitalization.
