FATAL VARICELLA GENERALISATA IN A CHILD WITH IMMUNOPATHY AND HEREDITARY NEUROLOGICAL SYNDROME - A REPORT WITH AUTOPSY ELECTRON MICROSCOPY AND VIRUS ISOLATION

A 4-year-old girl with immunopathy and a hereditary neurological syndrome [previously described in Acta Paediat. Scand. 55, 264 (1966)] succumbed with varicella generalisata. At autopsy hemorrhagic lesions were found in the skin, liver, lungs, pancreas and spleen. With the light microscope intranuclear inclusion bodies were seen in the liver, lungs and spleen. Under the electron microscope viral particles were found in these three organs and in the pancreas. Virus isolation in human embryonic cells with characteristics of varicella proved positive for heart-blood, skin and lungs. In the cerebrum there was a dysplasia of nerve cells in the cortex and basal ganglia and heterotopic nerve cells were found in the white matter. In the cerebellum there was a patchy loss of Purkinje cells, probably a dysplasia. Non-specific degeneration was seen in dorsal root ganglia and in the lateral corticospinal tracts. A younger brother of the patient shows a similar neurological syndrome. It seems reasonable to assume that the neurological syndrome is genetically determined. Whether an immunopathy is present in the brother is not known at present. © 1969 Springer-Verlag.