Measles Virus Associated Diseases

Subacute Sclerosing Panencephalitis (SSPE) The disease develops several years after an acute systemic measles infection. It is suggested that the risk is higher for those who had measles or measles immunization at a very early age. SSPE affects primarily children and adolescents. Behavioral changes, slowly progressive mental regression, seizures, myoclonus, and focal neurologic deficits characterize the clinical picture. In the acute stage, EEG often shows a burst-suppression pattern of high-amplitude slow-wave or spike and slow-wave complexes occurring at a rate of 4 to 20 seconds (Fig. 6.20). The CSF has high IgG levels with oligoclonal bands. Measles antibody titers in the CSF and serum rise during the course of the disease. Death occurs within several months or years after the initial symptoms.

Pathology: Grossly, the cerebral cortex is atrophic, and the hemispheric white matter is grayish and firm. The histology is characterized by (a) widespread perivascular lymphocyte and plasma-cell infiltrations in the cerebral hemispheres and brainstem; (b) variable neuronal losses in the cerebral cortex; (c) eosin-ophilic intranuclear inclusions in the neurons and oligo-dendrocytes; (d) diffuse myelin degeneration in the hemispheric white matter, accompanied by dense astro-cytic gliosis; and (e) in some neurons, neurofibrillary tangles that may contain measles genomes (see Fig. 6.20).

FIGURE 6.20

Subacute sclerosing panencephalitis (SSPE). A baby boy had measles at 11 months of age. He developed normally until 8 years of age, when his gait deteriorated and his behavior changed—he became hyperactive, distractible, and untidy. Seizures began around the same time. He continued to deteriorate, lost his motor skills and speech, became bedridden and incontinent, and displayed myoclonic jerks. At 10 years of age, he died. A. EEG shows periodic bursts of high-voltage slow complexes. B. Cerebral cortex shows perivascular cuffings with lymphocytes and plasma cells and (C) glial nodules (HE). White matter shows (D) myelin loss (Weil stain), (E) diffuse astrogliosis (Holzer stain), and (F) intranuclear inclusion in oligodendroglia (Lendrum stain).

FIGURE 6.20

Subacute sclerosing panencephalitis (SSPE). A baby boy had measles at 11 months of age. He developed normally until 8 years of age, when his gait deteriorated and his behavior changed—he became hyperactive, distractible, and untidy. Seizures began around the same time. He continued to deteriorate, lost his motor skills and speech, became bedridden and incontinent, and displayed myoclonic jerks. At 10 years of age, he died. A. EEG shows periodic bursts of high-voltage slow complexes. B. Cerebral cortex shows perivascular cuffings with lymphocytes and plasma cells and (C) glial nodules (HE). White matter shows (D) myelin loss (Weil stain), (E) diffuse astrogliosis (Holzer stain), and (F) intranuclear inclusion in oligodendroglia (Lendrum stain).

Measles Inclusion Body Encephalitis This is a rare condition which may develop several months after an acute measles infection. Immunocom-promised subjects are particularly susceptible to the disease, which is fatal within a few weeks. The pathology, as the name implies, is characterized by the presence of intranuclear eosinophilic inclusions in the neurons and the glial cells. The inclusions contain viral antigen, demonstrable with immunohistochemical stain.

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