title: Case of fulminant-SSPE associated with measles genotype D7 from India: an autopsy study.
abstract: Subacute sclerosing panencephalitis (SSPE), a post-measles progressive neurological disorder is still common in India because of indifferent vaccination compliance. However, the acute fulminant form of SSPE is extremely rare. An unusual case of fulminant SSPE in an 18-year-old man from south India with an ultra-short course of 19 days presenting with hemiparesis in absence of myoclonus and progressive cognitive decline, is reported. MRI showed frontal and parieto-occipital demyelination extending to nuclear areas. Antimeasles antibodies were demonstrable in CSF and serum with oligoclonal bands in CSF despite normal CSF protein and cell count. At autopsy, unlike classical SSPE, oligodendroglia containing measles viral antigen was sparse despite florid necrotizing leukoencephalitis with acute demyelination. Measles virus was isolated from the brain with hypermutation in M gene confirming the diagnosis. Phylogenetic analysis of the viral genotype indicated that it belonged to D7 genotype which is considered rare in India.
authors.affiliation: Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India.
authors.forename: Anita
authors.initials: A
authors.lastname: Mahadevan
authors.forename: Sunil R
authors.initials: SR
authors.lastname: Vaidya
authors.forename: Niteen S
authors.initials: NS
authors.lastname: Wairagkar
authors.forename: Deepika
authors.initials: D
authors.lastname: Khedekar
authors.forename: Jerry M E
authors.initials: JM
authors.lastname: Kovoor
authors.forename: Vani
authors.initials: V
authors.lastname: Santosh
authors.forename: T C
authors.initials: TC
authors.lastname: Yasha
authors.forename: P
authors.initials: P
authors.lastname: Satishchandra
authors.forename: V
authors.initials: V
authors.lastname: Ravi
authors.forename: S K
authors.initials: SK
authors.lastname: Shankar
mesh_terms: D000293:Adolescent; D000914:Antibodies, Viral / Q000097:blood / Q000134:cerebrospinal fluid; D001344:Autopsy; D001483:Base Sequence; D001921:Brain / Q000473:pathology* / Q000821:virology; D001927:Brain Diseases / Q000473:pathology / Q000821:virology; D003711:Demyelinating Diseases / Q000235:genetics / Q000473:pathology; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008459:Measles virus / Q000145:classification* / Q000235:genetics / Q000276:immunology / Q000302:isolation & purification; D009154:Mutation; D010802:Phylogeny; D012367:RNA, Viral / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D017423:Sequence Analysis, RNA; D013344:Subacute Sclerosing Panencephalitis / Q000473:pathology* / Q000821:virology*
chemical_list: D000914:Antibodies, Viral; D012367:RNA, Viral