Abstract:

Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with high mortality rate. The disease has been associated with a persistent infection with an aberrant measles virus and no effective treatment has been available. Herein, we describe the case of a nine-year-old female who presented with neurological findings, i.e. myoclonic jerks of the head and upper extremities and abnormalities in the executive function, and was diagnosed with SSPE. Her family reported that these complaints and findings have been present for three weeks. Past medical history revealed that the girl was not immunized according to the immunization calendar and had a febrile rash illness at the age of six years old. Ophthalmic examination showed bilateral papilledema. Cranial computed tomography (CT), magnetic resonance (MR) imaging and MR venography findings were not remarkable. The measles immunoglobulin G titers in the cerebrospinal fluid (CSF) and serum were 347 IU/mL and 265 IU/mL, respectively. Lumbar CSF opening pressure was elevated (270 mm water); intracranial hypertension secondary to SSPE was the remarkable feature of the present case.

Keywords: