| Article Access Statistics|
| Viewed||2790 |
| Printed||19 |
| Emailed||0 |
| PDF Downloaded||39 |
| Comments ||[Add] |
Click on image for details.
|Year : 2018 | Volume
| Issue : 2 | Page : 572-573
Isolated striatal lesions: A rare imaging finding of neurofibromatosis type I
Lokesh Saini1, Prashant Jauhari1, Biswaroop Chakrabarty1, Atin Kumar2, Sheffali Gulati1
1 Child Neurology Division, Department of Pediatrics, All Institute of Medical Sciences, New Delhi, India
2 Department of Radiodiagnosis, Jai Prakash Narayan Trauma Centre, All Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||15-Mar-2018|
Dr. Sheffali Gulati
Child Neurology Division, Department of Pediatrics, Room No. 3056, Third floor, Teaching block, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Saini L, Jauhari P, Chakrabarty B, Kumar A, Gulati S. Isolated striatal lesions: A rare imaging finding of neurofibromatosis type I. Neurol India 2018;66:572-3
An ever-expanding list of neurological disorders with striatal lesions exists. The spectrum ranges from acquired infective etiologies to neurometabolic and neurodegenerative disorders. Neurofibromatosis (NF) is an extremely rare cause of isolated striatal lesions and is generally not considered in the differential diagnosis of symmetrical isolated striatal hyperintensity. We came across a 9-year old boy with mild intellectual disability. There was no history of any associated movement disorder. Examination revealed multiple (>6) cafe-au-lait spots of >5 mm and an axillary freckling, fulfilling the National Institutes of Health diagnostic criteria of NF type 1. His magnetic resonance imaging (MRI) of the brain revealed bilateral symmetrical T2 hyperintense globus pallidi lesions [Figure 1]. The palladi lesions had rounded swollen appearance without any mass effect representing unidentified bright objects (UBO) [Figure 2]. UBOs are seen in upto 75% patients with NF type I. Pathologically, they represent vacuolar degeneration of myelin sheaths and are mostly asymptomatic. Basal ganglia involvement is common in NF, however, isolated symmetrical involvement is uniquely exceptional. Such atypical MRI pattern may result in unnecessary investigations for ruling out various neurometabolic and neurodegenerative disorders. This case highlights the importance of proper clinical and physical examination before ordering/interpreting MRI in patients with cognitive disorders.
|Figure 1: Axial T2-weighted (a) and fluid-attenuated inverse recovery [FLAIR] (b) MR images show bilateral symmetrical globus pallidi hyperintense lesions with well-defined margins|
Click here to view
|Figure 2: T1 weighted axial MRI brain showing bilateral symmetrical globus pallidi hypointensity|
Click here to view
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
The authors have no conflicts of interest to disclose with regard to this article.
| » References|| |
Tonduti D, Chiapparini L, Moroni I, Ardissone A, Zorzi G, Zibordi F, et al
. Neurological disorders associated with striatal lesions: Classification and diagnostic approach. Curr Neurol Neurosci Rep 2016;16:54.
Dipaolo DP, Zimmerman RA, Rorke LB, Zackai EH, Bilaniuk LT, Yachnis AT. Neurofibromatosis type 1: Pathologic substrate of high-signal-intensity foci in the brain. Radiology 1995;195:721-4.
[Figure 1], [Figure 2]