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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | NMO (Neuromyelitis Optica) | NMO | 3A0 Case 3 History
Case 3 History ---- This was the 2nd admission for this 58 year old woman with a history of progressive quadriparesis. The patient was in good health until approximately November 1975 when she had acute onset of right hemiparesis and hemisensory deficit with occipital headache. She was hospitalized for 5 weeks with recovery to very slight residual right hemiparesis manifested by slight limp and decreased fine motor skills in the right upper extremity. The diagnosis at that time apparently was stroke. The patient did well until spring of 1976 when she began to have right anterior thigh cramps and dysesthesias with right hand stiffness. Her workup then included normal skull X-rays, electroencephalogram and brain CT. She was treated with Phenobarbital and Dilantin for possible seizure activity because of the intermittency of her complaints and the symptoms resolved. The patient was next admitted in September of 1977 for the acute onset of paraparesis. She was felt to have transverse myelitis and was worked up with a negative myelogram and negative laboratory studies (before the availabililty of the test for IgG-NMO autoantibody). She made a partial recovery but her followup was erratic. The patient was seen again with several months of paraplegia, inability to walk, and bladder incontinence. She showed no known heart, lung, renal, or GI disease. Her family history is negative for an inherited disease. She had a flaccid paraplegia with upper extremity paresis, right greater than left. Her sensory examination showed a T2 level to pin prick and marked proprioceptive loss that was greatest in her feet and right upper extremity, but was also diminished in her left upper extremity. Her deep tendon reflexes were absent in the lower extremities and also in the right upper extremity. Pertinent laboratory studies obtained showed that on fluoroscopy of her spine, there was residual Pantopaque from the myelogram in 1976 but there was no block or arachnoiditis present. She had lumbar punctures which on various occasions showed from 5 to 30 polys. Glucose was 40, protein was in the 30-40 range. CSF IgG/albumin ratio was 0.39 and she had a normal CSF/serum ratio of 0.44. On several determinations oligoclonal bands were questionably present. She had negative CSF cultures for fungi, acid fast bacilli, and bacteria. Other laboratory tests showed an ESR of 135. There was mild anemia with a Hb of 9.9, Hct. of 30.7. Her B12 and folate levels were normal. She had a collagen vascular workup which showed a negative ANA; a serum protein electrophoresis that showed decreased albumin, but no monoclonal gammopathy. In addition, her myeloneuropathy began to ascend and after the initial workup, she was treated with steroids and INH prophylaxis. Despite this treatment, her pin prick level ascended to C4 and on 3/l/78 she had an episode of respiratory difficulty with intercostal breathing, a pO2 of 33, and a pCO2 of 49. Shortly after that her BP decreased and an EKG showed idioventricular rhythm. She was treated for septic shock, but had negative cultures. Over the following week, the patient became progressively unresponsive and was apneic off the respirator with only brain stem functions preserved despite treatment with antibiotics and pressor agents. She became hypothermic, hypotensive and had a cardiac arrest.
