Case 2010 - 6

Julia Kofler, MD, Geoffrey Murdoch, MD PhDUniversity of Pittsburgh

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

Diagnosis?

VZV

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/whitejunction

Intranuclear viral inclusionsmore 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; 125:770-780

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.