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Washington University Experience | VASCULAR | Vasculitis - PACNS | 1A0 Case 1 History
Case 1 History ---- The decedent was a 20-year-old man in good health until early February 2016, when he spontaneously developed a left posterior frontoparietal intraparenchymal hemorrhage. Neurosurgical workup was initiated given the clinical concern for an underlying vascular abnormality; however, imaging studies (MRI, MRI Angiogram, and catheter angiogram) were unrevealing. He presented to an OSH on 5/31 with a severe headache. A head CT scan showed right cerebellar and inferior vermian intraparenchymal hemorrhages, ventriculomegaly, and early tonsillar herniation. He became unresponsive around 10:40 AM and was transferred to BJH for further evaluation. He arrived with 8 mm pupils fixed bilaterally in mid-gaze; absent cough, gag, and corneal reflexes; and no movement of extremities to stimulation. He was immediately intubated/oxygenated, mannitol was administered and an external ventricular drain was emergently placed. A repeat CT revealed an interval increase in ventriculomegaly, worsened tonsillar herniation, a new intraparenchymal hemorrhage within the right cerebellar hemisphere with surrounding vasogenic edema, and global cerebral swelling with abnormal gray-white matter differentiation. Serial examination showed unremitting neurologic deterioration with the eventual loss of all extensor posturing over time. He was pronounced dead on 6/1 after formal testing confirmed the diagnosis of brain death. ---- At autopsy his unfixed brain weighed 1780g, reflecting marked edema. Microscopic examination showed granulomatous vasculitis composed of lymphocytes, epithelioid histiocytes and multinucleated giant cells. Some vessels also contained intravascular thrombi. Histochemical stains (AFB, PAS and GMS) were negative for microorganisms.
