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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | Inflammatory Demyelination | 9A0 Case 9 History
Case 9 History ---- The patient is a 12-year-old girl with an approximately two month course which began with a polio-like syndrome with subsequent now-resolved myocarditis. She initially presented with headache, poor p.o. intake, emesis and lethargy, and was febrile to 104 degrees Fahrenheit. A lumbar puncture showed elevated nucleated cells and elevated protein with relatively normal glucose. The patient's gait became unsteady with multiple falls and she remained persistently febrile. On September 20th, the patient was found to be tachycardiac, tachypneic, and showed full-body tremors with bowel incontinence and was not responsive. An EEG showed slowing and encephalopathy with elevated troponin and a ST segment elevation, concerning for cardiomyopathy and was placed on ECMO for persistent hypotension and narrow pulse pressures. The patient was started on IVIG and plasma exchange for a non-specific immune-related illness. On September 27, the patient was started on high dose (1 gram/day) Solumedrol despite a negative ANA, negative ANCA, negative NMDA receptor antibody and normal complement levels. ECMO was discontinued. By mid-October, the patient was unresponsive with minimal bilateral pupillary activity and mechanically ventilated via a tracheostomy. Neurologically, the patient showed no motor function and no cranial nerve function except for pupillary change. MR imaging performed on October 20 shows interval progression of a profound white matter predominant injury involving nearly all of the brain and spine with mild sparing of occipital poles. Exhaustive laboratory examination showed a negative ANA, negative ANCA, normal compliment levels, normal CRP, negative ENA, elevated ESR at 60 (normal 0-20), normal magnesium, negative NMDA receptor antibody, negative neuromyelitis optica antibody, slightly low copper levels, slightly elevated selenium levels, undetectable lead levels, normal Vitamin E, elevated Vitamin B12, and slightly low Vitamin D levels. Zinc levels were slightly increased. A histoplasma antigen was negative on September 30th. HIV, p24 antigen antibody, as well as RNA viral nodes were also negative. A serum lymphocytic choriomeningitis virus antibody was negative. The patient was positive for an IgG to mumps, but negative for IgM. RPR was non-reactive. Ann Arbor virus antibody panel was negative. CMV antibody was non-reactive for IgG and IgM. Epstein-Barr antibodies for IgG and IgM were negative. CSM has been negative for fungus and mycobacteria, as well as mycoplasma pneumoniae by PCR. Aerobic cultures in the CSF were negative. PCR for HSVC, enterovirus, CMV, EBV, HHV-6, parechovirus, VZV, influenza A and influenza B, as well as RSV, adenovirus, and parvovirus were negative. Operative procedure: Right frontal brain biopsy, given a diagnosis of "patchy myelin loss with preserved and degenerating axons, highly suspicious for acute disseminated encephalomyelitis (ADEM).
