A 19-year-old African American female presented acutely with headache, nausea, emesis, blurring of vision followed by hallucinations. Her sodium was noted to be 119. MRI Brain and pituitary obtained during this time demonstrated infiltrative signal abnormality in hypothalamus, thalamus and mamillary bodies. Diagnosis of SIADH was made and hyponatremia was corrected as per guidelines. Patient returned to her baseline.
She presented 2 weeks later with headache and acute memory loss which progressed to hypothermia, bradycardia and somnolence. She was drowsy but answering to questions with vigorous stimulation. MRI Brain showed worsening infiltrative signal abnormality extending into midbrain, thalamus, basal ganglia with contrast enhancement. She was empirically started on broad spectrum antibiotics, thiamine and Methylprednisolone 125mg Q6H. Initial labs were remarkable for low Vitamin B1 and elevated CSF protein. MR Spectroscopy showed decreased NAA and elevated choline peak. MRI orbits showed features concerning for bilateral optic neuritis. MethylPrednisolone dose was increased to 1g daily and she completed a 4 day course along with 3 day course of IVIG. Clinically, patient's mentation improved; hypothermia and bradycardia resolved. Repeat MRI Brain showed improving signal alteration. AQP 4 antibody returned positive. Patient was discharged home after 5 day course of Plasmapheresis.
Repeat MRI Brain obtained after 4 months of hospital discharge showed significantly improved signal abnormalities. Clinically patient has been stable without any relapse.