Progressive Rubella Panencephalitis

This disease develops years after a neonatal or childhood rubella infection. The pathology is characterized by a slowly progressive encephalitis and diffuse neuronal and myelin degenerations.


Mycoses of the CNS commonly occur in patients with malignancies, lymphoproliferative and hematopoietic diseases, poorly controlled diabetes, infected prosthetic heart valves, and organ transplants, and also in patients receiving long-term antibiotics, chemotherapy, highdose corticosteroids, and hyperalimentation. Patients with AIDS are particularly susceptible to fungal infections. Endemic infections are confined to the southeastern and southwestern United States.

The primary infection, usually in a distant organ, spreads hematogenously to the neural tissue. Less often, it extends directly to the brain from adjacent infectious foci. Cerebral involvement is often one manifestation of systemic mycosis. Once the fungi have reached the brain and/or spinal cord, a broad spectrum of inflammatory responses may occur, including granulomatous meningitis, meningoencephalitis, solitary or multiple granulomas, abscesses, and necrotizing vasculitis with septic hemorrhagic necrosis. The fungi are demonstrated readily in histologic sections using periodic acid-Schiff (PAS) and methenamine silver stains.

Mycotic infections may present acutely or may progress insidiously and slowly over weeks or months. Headaches, visual symptoms, nuchal rigidity, cranial nerve deficits, and low-grade fever are common presenting symptoms. Depending on the location of the pathologic lesions, focal symptoms and signs develop. Confusion, disorientation, psychiatric symptoms, and cognitive impairment evolve in chronic cases and may present diagnostic difficulties. A high suspicion for fungal infection and the use of appropriate tests secures the correct diagnosis.

An examination of the CSF reveals a moderate pleo-cytosis with lymphocytes and high protein and low sugar levels. Specific diagnostic tests include India ink preparation of the CSF sediment and blood smear, culture of CSF and blood, and immunologic tests for fungal antigen using enzyme linked immunosorbent assay (ELISA) and latex agglutination (LA) tests. Immu-nohistochemical-fluorescent antibody stain allows the rapid identification of fungi. Contrast-enhanced CT scan and MRI demonstrate meningeal involvement and parenchymal lesions.

Major mycotic infections of the nervous system are summarized in Table 6.5. Three of the most common are presented here.

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