Some aspects about the clinical and pathogenetic characteristics of the presumed persistent measles infections: SSPE and MINE

Abstract

It is well known that Subacute Sclerosing PanEncephalitis (SSPE) is due to an aborted form of the wild measles virus which results from a faulty immune reaction of the human body (1). The pathogenesis of this particular slow virus disease begins when an immunologically incompetent individual is attacked by the virus and is thereby unable to launch a full immune response, allowing parts of the aborted virus to harbor in the central nervous system. As is typical of all slow virus diseases, the aborted virus remains quiescent for a number of months to years only to break out to produce the neurological and ophthalmic symptoms of SSPE. The major components of the disease results from the progressive deterioration of the host’s nervous system, first starting with sometimes subtle and nonspecific higher cortical symptoms such as abnormal behavior, learning disabilities, single epileptic seizures and visual disturbances which formulate in months into a characteristic clinical period called stage I. Then the near diagnostic period of repetitive massive myoclonic jerks, beginning mental failure, motor disability in its early stages and immobility are seen which constitutes what has been called stage II. This stage is also progressive and in a few months the involuntary movements disappear and the dementia worsens. Stage III is a nonambulatory state of clinical affairs with ultimately complete motor failure, loss of the previously seen involuntary movements and severe dementia. Stage IV represents a severe “vegetative” stage which plateaus to severe neurological disability of at least 90% or death. This typical clinical progression is closely correlated with a rostral-caudal neural destruction. Most of what is thought to be clinically typical about this disease, is a manifestation of the neuropathological and clinical happenings which occur in stage II. This stage represents the classical subacute progressive clinical picture of what I call the subacute progressive form (SPF) of SSPE. There are other more atypical forms of the disease. The classical or SPF form and presentation represents only about 75% of the cases of SSPE which has been reported to the USA/World SSPE Registry since 1989. In the 1960s, in the heyday of SSPE and natural