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Case 2010 - 6

Case 2010 - 6. Julia Kofler, MD, Geoffrey Murdoch, MD PhD University of Pittsburgh. Clinical history. 52 yo male H/o Hodgkin lymphoma 2006 - Chemotherapy - Autologous stem cell transplant 02/2009 - Mediastinal radiation 07/2009

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Case 2010 - 6

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  1. Case 2010 - 6 Julia Kofler, MD, Geoffrey Murdoch, MD PhDUniversity of Pittsburgh

  2. Clinical history • 52 yo male • H/o Hodgkin lymphoma 2006 - Chemotherapy - Autologous stem cell transplant 02/2009 - 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

  3. Diagnosis?

  4. VZV

  5. VZV

  6. 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

  7. 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

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

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

  10. Final diagnosis • Varicella zoster radiculitis, meningitis and vasculitis associated with intraparenchymal hemorrhage

  11. References • Kleinschmidt-DeMasters BK, Gilden DH. Varicella zoster virus infections of the central nervous system. Clinical and pathologic correlates. Arch Pathol Lab Med 2001; 125:770-780 • Kleinschmidt-DeMasters BK, Amlie-Lefond C, Gilden DH. The patterns of Varicella zoster virus encephalitis. Hum Pathol 1996; 27:927-938. • Gilden D, Cohrs RJ, Mahalingham R, Nagel MA. Varicella zoster virus vasculopathies: Diverse clinical manifestations, laboratory features, pathogenesis and treatment. Lancet Neurol 2009; 8:731-740.

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