Article type: Research Article

Authors: . Gowda, Vykuntaraju K | Vignesh, Sukanya | V. Madivala, Bhaskar | Mamatha, | Prahalad Kumar, | Premalatha Ramaswamy and, | Sarala H. Gowda,

Affiliations: Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India | Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India | Department of Neuroradiology, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India | Department of Anatomy, Kempegowda Institute of Medical Science, Bangalore, India

Note: [] Correspondence: Vykuntaraju K. Gowda, Bangalore Child Neurology and Rehabilitation Center HANS complex, 8/A 1st Main 1st Cross, Manuvana, Near Adhichunchanagiri Choultry, Vijaynagar, Bangalore, 560040, Karnataka, India. Tel.: +91 80 23301212; Mobile: +91 9535212556; Fax: +91 80 26541799; E-mail: [email protected], [email protected]

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.

Keywords: Vanishing white matter disease, whole cell DPT vaccine, hepatosplenomegaly, microcephaly

DOI: 10.3233/JPN-140659

Journal: Journal of Pediatric Neurology, vol. 12, no. 3, pp. 167-170, 2014