Autoimmune Myelitis and Myocarditis in a Patient With Anti-Aquaporin-4, Antinuclear, and Antiphospholipid Autoantibodies: The Neuromyelitis Optica-Systemic Lupus Erythematosus (NMO-SLE) Overlap Syndrome.

The coexistence of two or more autoimmune diseases is well-known, e.g., a person can have neuromyelitis optica (NMO) and systemic lupus erythematosus (SLE) at the same time. We report a case of NMO-SLE overlap syndrome with myelitis and myocarditis as the initial manifestations. The patient, a 64-year-old man, presented with a 15-day history of ascending sensory loss and a 10-day history of exertional dyspnea. Magnetic resonance imaging (MRI) revealed longitudinally extensive transverse myelitis (LETM) from C7 to T6. Serology showed a high anti-aquaporin-4 antibody level. We diagnosed NMO based on these findings. Echocardiography showed a hypokinetic left ventricle with a severely reduced ejection fraction. Cardiac MRI demonstrated delayed gadolinium enhancement in the myocardium consistent with active inflammation. Because the cardiac findings could not be explained on the basis of NMO, we started searching for another autoimmune disease. Serology came back positive for a variety of autoantibodies, including antinuclear, anti-dsDNA, anti-chromatin, anti-cardiolipin, anti-ß2-glycoprotein-1, and lupus anticoagulant. These findings, along with leukopenia and low serum complement C4, prompted us to diagnose SLE, in addition to NMO. He was initially treated with plasmapheresis and methylprednisolone. Maintenance therapy consisted of rituximab, hydroxychloroquine, and aspirin. One year later, he only complained of mild paresthesia in the feet. Patients with NMO should always be screened for SLE especially if they have signs and symptoms that cannot be accounted for by NMO alone, e.g., our patient had myocarditis. Conversely, patients with SLE and evidence of transverse myelitis should be screened for anti-AQP4 antibodies.

Delayed Toxic-Hypoxic Leukoencephalopathy As Sequela of Opioid Overdose and Cerebral Hypoxia-Ischemia.

Delayed leukoencephalopathy in the aftermath of toxic exposure and cerebral hypoxia-ischemia is known as "delayed post-hypoxic leukoencephalopathy" (DPHL) but the name "delayed toxic-hypoxic leukoencephalopathy" (DTHL) may be more accurate if toxic and hypoxic mechanisms are both involved in the pathogenesis of delayed leukoencephalopathy. DTHL is characterized by initial recovery from toxic exposure and cerebral hypoxia-ischemia, clinical stability over a few weeks, and subsequent neurological deterioration with the sudden emergence of diffuse white matter disease. A 46-year-old man suffered respiratory failure and hypotension as a result of opioid overdose. Brain MRI showed watershed infarcts and EEG showed diffuse theta-delta slowing consistent with global cerebral hypoperfusion. He recovered fully and was discharged with intact cognitive function. Three weeks later, he presented with abulia and psychomotor retardation. MRI revealed extensive white matter hyperintensity and EEG showed diffuse polymorphic delta activity. DTHL was diagnosed based on classic MRI features, history of opioid overdose and hypoxic brain injury, and negative test results for etiology of white matter disease. He developed akinetic mutism prompting administration of methylprednisolone 1000-mg IV q24h for five days. He also received amantadine 100-mg PO q12h. His cognition, motivation, and psychomotor function slowly improved and returned to baseline about two months after the overdose. Clinic reassessment two and a half months after the overdose revealed normal cognitive function, slight residual MRI hyperintensity, and mild EEG slowing anteriorly. Toxic-metabolic myelinopathy causing diffuse demyelination in the deep white matter is a perfect explanation for the patient's neurological symptoms, MRI changes, EEG findings, and time course of recovery.