brintellex
ValprolBanner
Annals of Indian Academy of Neurology
  Users Online: 2016 Home | About the Journal | InstructionsCurrent Issue | Back IssuesLogin      Print this page Email this page  Small font size Default font size Increase font size
IMAGE IN NEUROLOGY
Year : 2016  |  Volume : 19  |  Issue : 2  |  Page : 272-274

An unusual case of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy with occipital lobe involvement


1 Department of Neurology, Olive View-UCLA Medical Center, Sylmar; Department of Radiology, VA Greater Los Angeles Healthcare System, Los Angeles, California, USA
2 Department of Neurology, Olive View-UCLA Medical Center, Sylmar, Los Angeles, California, USA
3 Department of Neurology, Olive View-UCLA Medical Center, Sylmar; Department of Radiology, VA Greater Los Angeles Healthcare System; University of California, Los Angeles (UCLA), California, USA
4 Department of Neurology, Olive View-UCLA Medical Center, Sylmar; Department of Radiology, VA Greater Los Angeles Healthcare System; University of California, Los Angeles (UCLA); Keck School of Medicine, University of Southern California (USC), Los Angeles, California, USA

Correspondence Address:
Shrikant Mishra
16111 Plummer Street, North Hills - 91343, California
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.173403

Rights and Permissions

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed2652    
    Printed42    
    Emailed1    
    PDF Downloaded60    
    Comments [Add]    

Recommend this journal