title: Early onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection.
abstract: We report an 18-month-old girl with rapidly progressive subacute sclerosing panencephalitis, whose non immunised mother had measles at the time of delivery. The patient presented with repetitive episodes of myoclonic jerks of the head and arms, followed by a drop of head and trunk with frequent falls. EEG, CSF studies and MRI confirmed the diagnosis. Despite therapy with isoprinosine and valproate, seizure activity continued and she became vegetative within 2 months, with severe spasticity and swallowing difficulties, and died at the age of 28 months. Early age of onset and rapid progression were most likely related to haematogenous in utero acquisition of the measles virus prior to delivery, as well as immaturity of neuronal and immune systems.
  
  
  CONCLUSIONS
  this case emphasises the importance of a high measles vaccine coverage in the population in order to prevent the risk of disease in general and, in particular, gestational measles.
authors.affiliation: Department of Paediatrics, Child Neurology, Children's Hospital, Rue Willy-Donzé 6, 1211 Geneva 14, Switzerland.
authors.forename: Dounia
authors.initials: D
authors.lastname: Cruzado
authors.forename: Virginie
authors.initials: V
authors.lastname: Masserey-Spicher
authors.forename: Laurent
authors.initials: L
authors.lastname: Roux
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Delavelle
authors.forename: Fabienne
authors.initials: F
authors.lastname: Picard
authors.forename: Charles-Antoine
authors.initials: CA
authors.lastname: Haenggeli
mesh_terms: D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D018450:Disease Progression; D004569:Electroencephalography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D018445:Infectious Disease Transmission, Vertical; D007542:Inosine Pranobex / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008457:Measles / Q000151:congenital* / Q000635:transmission; D009207:Myoclonus / Q000821:virology; D012307:Risk Factors; D013344:Subacute Sclerosing Panencephalitis / Q000175:diagnosis* / Q000188:drug therapy / Q000821:virology*; D014635:Valproic Acid / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D000998:Antiviral Agents; D014635:Valproic Acid; D007542:Inosine Pranobex