Neuroprogressive disease of post‐infectious origin: A review of a resurging subacute sclerosing panencephalitis (SSPE)

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive, essentially untreatable, disease of the nervous system. When first described in the 20th Century, it was characterized more for its neuropathological features than for its pathophysiology or cause. It was not until the 1960s that a clear relationship to the measles virus was established. It is now thought that this uncommon infectious encephalopathy is caused by a "slow," altered or persistent form of the wild measles virus which has harbored in the nervous system for years. Then a "breakout" occurs and the more lytic and virulent organisms produce the progressive and spreading inflammatory and destructive lesions which are confined to the nervous system. Epidemiological study of the disease confirms its relationship to measles. In the years since the development of national measles immunization programs, there has been a dramatic decline in the incidence of measles exanthem and until recently a corresponding decline in the incidence of SSPE. In recent years there has been a mild to moderate increase in cases of SSPE as reported to the USA/International SSPE Registry. As yet, there has not been a totally effective treatment. The purpose of this paper is to give an overall review on SSPE and its relationship to measles. This review will include a prospectus of its history, considerations as to its etiology, correlation of clinicopathological features, and thoughts on the past and present epidemiology and treatment.

Subacute Sclerosing Panencephalitis: More Cases of This Fatal Disease Are Prevented by Measles Immunization than Was Previously Recognized

“…Diagnosis depended on a high index of suspicion, as well as patient access to either specialized neurologists or a physician familiar with the rare disease SSPE. The numerous case reports and reviews of SSPE underscore the complexity of the clinical aspects of the disease and the challenges associated with the recognition and diagnosis of SSPE, particularly adult-onset SSPE and SSPE that follows a fulminating course [1,2,19,52,53].…”

“…The SSPE Registry was established in 1969 and was maintained at the University of Tennessee (Memphis) until 1980 [18,19], although it was never considered to be complete. SSPE cases were reported to the National Registry for SSPE primarily by members of the Child Neurology Society, and additional data were collected via questionnaires or surveys directed to pediatric neurologists [17,18,51].…”

“…SSPE cases were reported to the National Registry for SSPE primarily by members of the Child Neurology Society, and additional data were collected via questionnaires or surveys directed to pediatric neurologists [17,18,51]. In addition to having been underreported, many cases of SSPE likely were undiagnosed, because relatively little was known about the various clinical presentations of SSPE [19]. Diagnosis depended on a high index of suspicion, as well as patient access to either specialized neurologists or a physician familiar with the rare disease SSPE.…”

“…By the late 1980s, the number of reported cases of SSPE had decreased to 1-2 cases/year (from a high of 40-50 reported cases/ year in the 1970s), on the basis of the number of cases reported to a registry of SSPE cases that was established in 1969 [18,19]. The observed decrease in the number of SSPE cases in the late 1980s occurred after a period during which the incidence of measles was low (1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988), with an average total number of 3000 cases of measles reported annually [20].…”

Transtornos invasivos do desenvolvimento não-autísticos: síndrome de Rett, transtorno desintegrativo da infância e transtornos invasivos do desenvolvimento sem outra especificação

“…mucopolysaccharoidosis San Filippo), neurological conditions (e.g. slow virus encephalitis 16 or epilepsy) though in the latter cases language is far more affected than in the case reports on CDD. It should also be differentiated from autism in which a near normal development in the first one or two years is seen in up to 30% of all cases.…”