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14 Encephalitis in Immunocompetent Patients

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Abstract:

This chapter considers viruses that cause subacute and chronic encephalitis in immunocompetent persons. Subacute sclerosing panencephalitis (SSPE) is a rare late manifestation of measles virus infection, developing years after natural measles infection. During the first half of the 20th century several papers were written describing the clinical and pathological features of SSPE, but different pathological variants were described as if they were different diseases. The absence of recent reports suggests that progressive rubella panencephalitis (PRP) may have essentially disappeared. The reasons for its appearance and disappearance are mysterious. It shares certain features with SSPE. The great majority of cases of HAM/TSP are now known to be caused by human T-cell leukemia virus type 1 (HTLV-1), with occasional cases caused by a related retrovirus, HTLV- 2. Rasmussen's encephalitis is a condition of chronic intractable epilepsy in children that is associated with signs of progressive hemispheral dysfunction and unilateral brain atrophy. Resected brain tissue can demonstrate perivascular infiltrates and glial nodules, usually in the absence of clinical features of encephalitis. Viral studies are performed to exclude other causes of encephalitis. In contrast to encephalitis lethargica, however, Vilyuisk encephalitis appears to be an endemic disease, and the chronic phase has more widespread central nervous system (CNS) destruction. Experimental models have been useful to examine the pathogenesis of neurodevelopmental abnormalities and also behavioral defects, the role of immune-mediated damage, and the development of chronic Borna disease.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Figures

Image of FIGURE 14.1
FIGURE 14.1

Close-up of fixed, sliced brain from a case of longstanding SSPE. The granular, rubbery consistency and discoloration of the white matter are evident.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.2
FIGURE 14.2

Sliced cerebrum from the case in Fig. 14.1. There is loss of white and gray matter, ventricular dilatation, and narrowed corpus callosum.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.3
FIGURE 14.3

Cortical inflammation consisting predominantly of lymphocytes and macrophages in a case of SSPE of 3 months' duration. Hematoxylin and eosin stain.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.4
FIGURE 14.4

Immunoperoxidase staining using antimeasles antibody to demonstrate measles virus antigen in (a) neuron cytoplasm, processes, and nucleus and (b) oligodendrocyte nuclei in a case of SSPE of 9 months' duration. Counterstained with hematoxylin.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.5
FIGURE 14.5

White matter from a case of long-standing SSPE. There is an increase in glial nuclei and widened perivascular spaces containing reticulin fibers, but little inflammation. Hematoxylin and eosin stain.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.6
FIGURE 14.6

Electron micrograph of intranuclear paramyxovirus tubules from the temporal lobe at autopsy from a case of SSPE. × 125,000. Courtesy of J. E. Richmond.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.7
FIGURE 14.7

Low-power view of myelin-stained section of the spinal cord in HAM/TSP. Note pallor of staining in the lateral corticospinal tracts and posterior columns.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.8
FIGURE 14.8

Inflammation in the spinal cord in HAM/TSP with small veins and cord parenchyma infiltrated with mononuclear inflammatory cells.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.9
FIGURE 14.9

Meningeal thickening and inflammation and slight perivascular and meningeal inflammation in HAM/TSP.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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Image of FIGURE 14.10
FIGURE 14.10

MRI of Rasmussen's syndrome. Coronal FLAIR image demonstrates hyperintensity in the cortical and subcortical regions of the medial superior frontal lobe. Courtesy of Dennis Spencer, Yale University School of Medicine.

Citation: Booss J, Esiri M. 2003. 14 Encephalitis in Immunocompetent Patients, p 237-252. In Viral Encephalitis in Humans. ASM Press, Washington, DC. doi: 10.1128/9781555817831.ch14
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