Subacute sclerosing panencephalitis in an infant: Diagnostic role of viral genome analysis (original) (raw)

1994, Annals of Neurology

Subacute sclerosing panencephalitis (SSPE) is related to "defective" measles virus or vaccination, though an association with parainfluenza viruses has been reported. SSPE is characterized by a slow, erratic course and elevated cerebrospinal fluid measles titers. An immunocompetent, vaccinated infant, with onset of symptoms in parainfiuenza virus season and a catastrophic course is described. Cerebrospinal fluid titers were negative, but postmortem brain had typical SSPE lesions. Patient brain-derived RNA, subjected to reverse transcription followed by polymerase chain reaction yielded polymerase chain reaction products with measles virus but not parainfluenza virus genes. The sequenced fragment revealed multiple mutations, typical for SSPE. SSPE can thus present in infants, with short latency and no cerebrospinal fluid antibodies. Viral genomic analysis may be diagnostic, permitting early therapy. The incidence of subacute sclerosing panencephalitis (SSPE), a fatal neurodegenerative disease most commonly associated with prior measles infection [1, 2], has significantly decreased in the United States [3]. Since the introduction of measles vaccines, age of onset and vaccine-associated cases have increased [3]. Cases associated with parainfluenza virus (PIV), relatively common in eastern Europe and the Middle East [4-6], have recently been reported in this country [7]. New therapies, e.g., interferon, hold hope for affected individuals, making timely diagnosis important [8, 9]. We report an infant who presented in PIV season with no cerebrospinal fluid (CSF) measles antibodies and document the diagnostic utility of molecular analysis of the virus.