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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | NMO (Neuromyelitis Optica) | NMO | 4A0 Case 4 History
Case 4 History ---- The patient was a 10-year-old female with a 1-year history of intermittent pain when she turned her neck and persistent rightward head tilt. More recently she developed progressive right hand weakness with paresthesias and increasing difficulty with ambulation secondary to progressive leg weakness. Physical examination was significant for profound right hand weakness and sensory impairment, and bilateral lower extremity weakness and proprioceptive impairment. Magnetic resonance imaging showed widening of the cervical spine with increased intramedullary T2 signal and contrast enhancement. The abnormal T2 signal extended from the craniocervical junction to T5, and the abnormal enhancement extended from the craniocervical junction to T3. No abnormalities were seen in the remainder of the brain. Corticosteroid therapy with dexamethasone was instituted, but the patient showed no improvement. Given the lack of steroid response and the urgent need for decompression, a C1 laminectomy and C2 through T3 laminotomy was performed. Intraoperative ultrasound confirmed the extent of the lesion, which upon exposure was found to be brownish gray and well-demarcated from the surrounding spinal cord, splaying the posterior columns laterally on each side. A distinct plane between the mass and the surrounding cord made gross total resection possible. The tissue presented shows an H&E-stained section of the spinal lesion. The authors provided additional information: "The lesion on histological examination had patchy loss of myelin on LFB stain, with apparent preservation of some axons, with GFAP-positive gemistocytic cells, macrophages that were positive for CD68, and mainly CD3 positive T cells, with a few CD20 positive B cells. The presenter and his colleagues entertained a diagnosis of inflammatory demyelinating pseudotumor, until they received a report from Mayo Clinic, that the anti-aquaporin-4 antibody was found in a specimen of the patient’s blood. The patient had been previously worked up for a “bad eye,” information that was not known at the time of surgery. This girl has had an aggressive course of the disease, and she had relapsed four times since surgery was performed in 2007." (This case was presented at the Diagnostic Slide Session, AANP meeting 2009, Case #6)
