Subacute sclerosing panencephalitis in pregnancy

Abstract

We present a case of subacute sclerosing panencephalitis that developed in a previously healthy 29-year-old pregnant woman who had returned from a trip to rural India shortly before the onset of symptoms. She was admitted to hospital at 27 weeks' gestation with a history of cognitive decline and difficulty completing simple tasks. She had no clinical signs of infection. The working diagnosis was autoimmune encephalitis, although extensive investigations did not lead to a final classifying diagnosis. The patient became comatose and developed hypertension, and an emergency caesarean section was done at 31 weeks to deliver the child, who seemed healthy. The patient died about 6 weeks after the onset of symptoms. The patient was found to have had subacute sclerosing panencephalitis at autopsy. In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case.

Figure 1

T2 weighted MRI scans from the time of admission and shortly before death Normal findings early in the course of the disease at time of admission (A–C). Brain oedema and diffuse hyperintensity in the cerebral cortex, particularly in the occipital lobe, and throughout the brainstem shortly before death (D–F).

Figure 2

Microscopy findings Haematoxylin and eosin stained sections (A, B) showing ovoid-to-elongated eosinophilic nuclear and cytoplasmic inclusions, some of which showed a characteristic Cowdry type A morphology (arrows; magnification × 1000). Transmission electron microscopy image (C) of the specimen showing intranuclear inclusions (right) comprised of nucleocapsid filaments (magnification × 700). High magnification (× 25 000) transmission electron microscopy image (D) of the nucleus showing nucleocapsid filaments.