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Washington University Experience | MYELIN (NON-IMMUNE MEDIATED) | Central Pontine Myelinolysis (CPM) | 6A0 Case 6 History
Case 6 History ---- The patient was a 77 year old woman with a past medical history significant for hypertension and hyperlipidemia who presented with numbness in the right lower extremity and progressed to bilateral lower extremity weakness. An outside hospital MRI was thought to represent transverse myelitis which was treated with IV steroids and improved. In April of 2012, the symptoms ceased to improve with steroids or Azathioprine. Further workup showed a normal CSF including a normal IgG index, negative NMO antibody, and positive Lyme IgM antibody. MRI showed T1-T3 and T5-T12 hyperintensities. A repeat MRI showed multiple diffuse restriction areas consistent with subacute infarcts. A T10-L1 hyperintensity in spinal cord concerning for a demyelinating disease, infectious process or vasculitis. A CSF analysis was again unrevealing. Serum ANA, ANCA, B12, RPR, anti-TPO and anti-TG were all normal/negative. An EMG was compatible with multi-radiculopathy versus plexopathy. A cerebral angiogram was negative for vasculitis. The patient developed anasarca most likely secondary to low protein/albumin (1.6 grams/dL). A TTE was performed on 4/23/2012 showing an ejection fraction of 65% and pericardial effusion. A PET scan performed on 4/24/2012 showed a hypermetabolic lesion within the left lingula, nonspecific mildly hypermetabolic bilateral adrenal hyperplasia, and diffuse mildly hypermetabolic bone marrow activity suggestive of an anemic state. The clinical team decided to empirically treat her for a possible antibody-negative NMO with plasma exchange. She underwent 1.5 sessions on 4/25/2012 and 4/27/2012, but during her second session she had chest pain with atrial fibrillation and hypotension. The patient was noted to be bicytopenic with hemoglobin ranging from 7.7 to 10.9 and platelets ranging from 78 to 128K. She was also found to have paraproteinemia with increased serum gamma peak and beta 2 protein. The patient was evaluated by hematology who found circulating plasmacytoid lymphocytes. Her white blood cell count increased from 8200 on 4/27/2012 to 40,000 on 4/30/2012. On return to the floor after her abdominal CT, the patient was found to be less responsive, hypotensive to the 70s systolic, with increased oxygen requirement. On 4/29/2012, she was still hypotensive with maximum pressors and agonal breathing. At this time her husband redirected her care to comfort care only and she died.
