Case Julia Kofler, MD, Geoffrey Murdoch, MD PhD University of Pittsburgh

Clinical history  52 yo male  H/o Hodgkin lymphoma Chemotherapy - Autologous stem cell transplant 02/ Mediastinal radiation 07/2009  09/2009: dyspnea, cough, low-grade fever, chest wall pain and rash - Chest x-ray and CT c/w radiation pneumonitis  Mental status changes - Head CT unremarkable  One day later, patient collapsed, minimally responsive with seizure-like activity

Diagnosis?

VZV

VZV infections of the nervous system  Inflammation, hemorrhage and necrosis in spinal ganglia  Polyradiculitis  Myelitis  Meningitis  Encephalitis - Large vessel disease - Small vessel vasculopathy - Ventriculitis/periventriculits - Mixed patterns

Large vessel disease  Vessel wall inflammation, necrotizing vasculitis, thrombosis  Bland or hemorrhagic infarctions in distribution of large or medium-sized arteries  Vasculopathy with fibrointimal proliferation but without inflammation  Immunocompetent and immunosuppressed patients Kleinschmidt-DeMasters, Arch Pathol Lab Med 2001

Small vessel vasculopathy  Variable lymphocytic infiltrate and endothelial swelling  Often associated with infection of oligodendrocytes  Mixed ischemic/demyelinating lesions near cortical gray/white junction  Intranuclear viral inclusions more readily identified  Lesions often lack significant inflammation Kleinschmidt-DeMasters, Arch Pathol Lab Med 2001

Ventriculitis/periventriculitis  Rare in pure form  Infection of ependymal cells Kleinschmidt-DeMasters, Arch Pathol Lab Med 2001 VZV ISH

Final diagnosis  Varicella zoster radiculitis, meningitis and vasculitis associated with intraparenchymal hemorrhage

References  Kleinschmidt-DeMasters BK, Gilden DH. Varicella zoster virus infections of the central nervous system. Clinical and pathologic correlates. Arch Pathol Lab Med 2001; 125:  Kleinschmidt-DeMasters BK, Amlie-Lefond C, Gilden DH. The patterns of Varicella zoster virus encephalitis. Hum Pathol 1996; 27:  Gilden D, Cohrs RJ, Mahalingham R, Nagel MA. Varicella zoster virus vasculopathies: Diverse clinical manifestations, laboratory features, pathogenesis and treatment. Lancet Neurol 2009; 8: