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. Gowda
a  Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Sukanya Vignesh
b  Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Bhaskar V. Madivala
c  Department of Neuroradiology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
,
Dr. Mamatha
b  Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Prahalad Kumar
b  Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Premalatha Ramaswamy
b  Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India
,
Sarala H. Gowda
d  Department of Anatomy, Kempegowda Institute of Medical Science, Bangalore, India
› Author Affiliations

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Further Information

Publication History

21 August 2014

21 August 2014

Publication Date:
30 July 2015 (online)

Abstract

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.