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:
drknvraju@hotmail.com, drknvraju@yahoo.co.in
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.
