Fatal Measles Complication Killed Patients Years Later

[kristopher]

2. Source for more information

http://pmj.bmj.com/content/78/916/63.full

Postgrad Med J 2002;78:63-70 doi:10.1136/pmj.78.916.63
Review
Subacute sclerosing panencephalitis
R K Garg
Correspondence to:
 Dr Ravindra Kumar Garg, Department of Neurology, King George's Medical College, Lucknow 226 003, India;
 garg50{at}yahoo.com
Received 22 May 2001
Accepted 4 September 2001
Abstract
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.

Subacute sclerosing panencephalitis (SSPE) is a serious disorder of the central nervous system. It is a slow virus infection caused by defective measles virus (table 1). The term subacute sclerosing panencephalitis has been used since Greenfield suggested it in 1960 to designate a condition due to a persistent infection by a virus involving both grey matter and white matter.1 In fact, SSPE had originally been described as three different neuropathological entities. In 1933 Dawson, for the first time, described a child with progressive mental deterioration and involuntary movements who, at necropsy, was found to have a dominant involvement of grey matter in which neuronal inclusion bodies were abundant.2 He suggested the term “subacute inclusion body encephalitis”. Later Pette and Doring (1939) reported a single case of what they called “nodular panencephalitis” a disease with equally severe lesions in both grey and white matter.3 Six years later, Van Bogaert drew attention to the presence of dominant demyelination and glial proliferation in the white matter and suggested the term “subacute sclerosing leukoencephalitis”.4 A viral aetiology was suggested by Dawson, but it was Bouteille et al, in 1965, who on electron microscopy demonstrated the presence of viral structures resembling measles virus in the brain.5 In 1969 measles virus was actually recovered from the brain of a patient with SSPE.6 Since then a lot of progress has been made towards understanding of this potentially lethal disorder. Various treatment modalities have been tried with little success. In this article all recent information will be reviewed.

Note the review is as of 1992.

More current NIH page: http://www.ninds.nih.gov/disorders/subacute_panencephalitis/subacute_panencephalitis.htm