Tumoural transformation in Van der Knaap syndrome

Previous MRI was suggestive of cystic areas in bilateral anterior frontal and temporal regions with hypointensity in bilateral periventricular and subcortical white matter areas, representative of leukoencephalopathy or demyelination, suggesting the diagnosis of Van der Knaap syndrome [Figure 1]. Th...

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Published inNeurology India Vol. 67; no. 3; pp. 890 - 892
Main Authors Gupta, Pankaj, Kumar, Varsha, Kataria, Rashim, Srivastava, Trilochan, Sardana, V
Format Journal Article
LanguageEnglish
Published India Wolters Kluwer India Pvt. Ltd 01.05.2019
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Subjects
Brain Neoplasms - diagnosis
Brain Neoplasms - pathology
Brain Neoplasms - surgery
Cell Transformation, Neoplastic - pathology
Convulsions & seizures
Cysts
Female
Gliosis - diagnosis
Gliosis - pathology
Gliosis - surgery
Hereditary Central Nervous System Demyelinating Diseases
Humans
Learning disabilities
Middle Aged
India
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Summary:Previous MRI was suggestive of cystic areas in bilateral anterior frontal and temporal regions with hypointensity in bilateral periventricular and subcortical white matter areas, representative of leukoencephalopathy or demyelination, suggesting the diagnosis of Van der Knaap syndrome [Figure 1]. The recent contrast CT scan showed a 55 X 30 X 50 mm solid-cystic left temporal mass with heterogenous enhancement in the medial part of the lesion with subfalcine herniation and midline shift of 7 mm to the right side [Figure 2]. {Figure 1}{Figure 2}{Figure 3} Van der Knaap syndrome is an autosomal recessive disorder with mutation in the megalencephalic leukoencephalopathy (MLC1) gene, producing a defect in myelin.
ISSN:0028-3886
1998-4022
DOI:10.4103/0028-3886.263185