title: Fulminant subacute sclerosing panencephalitis presenting with acute ataxia and hemiparesis in a 15-year-old boy.
abstract: BACKGROUND
  Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading.
  
  
  METHODS
  We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy.
  
  
  CONCLUSIONS
  This case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.
authors.affiliation: Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Rukmini Mridula
authors.initials: RM
authors.lastname: Kandadai
authors.affiliation: Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Praveen
authors.initials: P
authors.lastname: Yada
authors.affiliation: Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Megha S
authors.initials: MS
authors.lastname: Uppin
authors.affiliation: Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Shaik Afshan
authors.initials: SA
authors.lastname: Jabeen
authors.affiliation: Department of Neurology, Medical College Hospital, Trivandrum, Kerala, India.
authors.forename: Ajith
authors.initials: A
authors.lastname: Cherian
authors.affiliation: Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Meena Angamuthu
authors.initials: MA
authors.lastname: Kanikannan
authors.affiliation: Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Rupam
authors.initials: R
authors.lastname: Borgohain
authors.affiliation: Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, India.
authors.forename: Sundaram
authors.initials: S
authors.lastname: Challa
keywords: GFAP; asymmetric presentation; autopsy; measles; steroids