J Pediatr Neurol 2014; 12(03): 167-170
DOI: 10.3233/JPN-140659
Case Report
Georg Thieme Verlag KG Stuttgart – New York

Atypical vanishing white matter disease with microcephaly and hepatosplenomegaly provoked after diphtheria pertussis tetanus vaccination

Vykuntaraju K. Gowdaa, Sukanya Vigneshb, Bhaskar V. Madivalac, Dr. Mamathab, Prahalad Kumarb, Premalatha Ramaswamyb, Sarala H. Gowdad
  • aDepartment of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
  • bDepartment of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
  • cDepartment of Neuroradiology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
  • dDepartment of Anatomy, Kempegowda Institute of Medical Science, Bangalore, India

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21. August 2014

21. August 2014

30.Juli 2015 (online)


Vanishing white matter (VWM) disease is a rare leukoencephalopathy. Normal development in early childhood with regression of milestones after trauma or infection is typical clinical presentation. We are reporting a child with atypical VWM disease. A 1.5-year-old female child presented with fever followed by altered sensorium and convulsions following first booster dose of diphtheria pertussis tetanus vaccination. Her development was normal till 1 yr of age. Her weight and head size were below 3 standard deviations. She had hepatosplenomegaly. Her routine investigations including cerebrospinal fluid examination were normal. Magnetic resonance imaging (MRI) of brain shows diffuse white matter signals changes (hyperintensity on T2-weighted and hypointensity on T1-weighted images) involving the subcortical “U” fibers sparing basal ganglia. MRI shows diffuse white matter hyperintensity on T2-weighted images with areas of low signal on fluid-attenuated inversion recovery, close to the signal of cerebrospinal fluid. Based on MRI findings we diagnosed as VWM disease.