Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 13183  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (549 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures

 Article Access Statistics
    Viewed1741    
    Printed27    
    Emailed0    
    PDF Downloaded57    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
NEUROIMAGE
Year : 2015  |  Volume : 63  |  Issue : 4  |  Page : 636-637

Fetal hemimegalencephaly


1 Department of Radiology, Sri Ramachandra University, Tamil Nadu, India
2 Department of Radiology, Mediscan Systems, Tamil Nadu, India

Date of Web Publication4-Aug-2015

Correspondence Address:
V V Vaishnavathi
Department of Radiology, Sri Ramachandra University, Porur, Chennai - 600 116, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.162126

Rights and Permissions



How to cite this article:
Vaishnavathi V V, Rangasami R, Suresh S, Suresh I. Fetal hemimegalencephaly. Neurol India 2015;63:636-7

How to cite this URL:
Vaishnavathi V V, Rangasami R, Suresh S, Suresh I. Fetal hemimegalencephaly. Neurol India [serial online] 2015 [cited 2019 Nov 12];63:636-7. Available from: http://www.neurologyindia.com/text.asp?2015/63/4/636/162126


A 21-year-old female patient with 25 weeks of gestation was referred for fetal magnetic resonance imaging (MRI) for the evaluation of unilateral ventriculomegaly with the right lateral ventricle measuring 11 mm on sonography [Figure 1]a and b. Fetal MRI using half-Fourier acquisition single-shot turbo spin echo sequence revealed an enlarged right cerebral hemisphere with diffuse hypointensity of the cerebral white matter as compared to the left side, suggesting advanced myelination [Figure 1]c-f. The cerebral convexity sulci were visualized on the right side suggesting a sulcal pattern corresponding to 27-28 weeks on that side. The sulcal pattern corresponded to 25 weeks on the left side [Figure 1]d and f. The lateral ventricles measured 11 mm on the right side and 6 mm on the left side. A radiological diagnosis of hemimegalencephaly was made that was confirmed on autopsy (the couple opted for termination of pregnancy). Though the exact cause of hemimegalencephaly (HME) is unknown, the main possibility includes a genetic mutation. These are of three types: (1) an isolated form; (2) a syndromic form occurring with developmental disorders or neurocutaneous syndromes; and, (3) total hemimegalencephaly. [1] Clinically, macrocephaly is the first sign at birth, the main symptoms being epilepsy intractable to medications, and the presence of focal neurological deficits. The MRI findings are enlarged cerebral hemisphere with an enlarged lateral ventricle with straight frontal horns pointing anteriorly and superiorly, and, an ipsilateral normal or dysplastic cerebral cortex. [2],[3] There may be associated cortical thickening and cortical abnormalities like agyria, pachygyria or polymicrogyria. [1] Accelerated myelination in the white matter is diagnosed on MRI when one observes the white matter becoming hypointense on T2-weighted images (as seen in our case); or, hyperintense on T1-weighted images (T1/T2 shortening). [4]
Figure 1: (a and b) Axial sonographic images showing a unilateral ventriculomegaly (arrow); (c and d) axial, (e and f) coronal T2 - weighted half - Fourier acquisition single - shot turbo spin echo magnetic resonance images showing mild ventriculomegaly on the right side (arrow) and an enlarged right cerebral hemisphere (open arrow). There is hypointensity of the right cerebral white matter (asterisk). Cerebral convexity sulci are visualized on the right side (curved arrow)

Click here to view


Whenever the antenatal sonography shows an enlarged lateral ventricle or cerebral hemisphere, a complementary fetal MRI is helpful to diagnose the condition and for counseling regarding the treatment modalities. [5] Hemispherectomy is the surgical treatment of choice for the intractable epilepsy from HME. [5]

 
  References Top

1.
Broumandi DD, Hayward UM, Benzian JM, Gonzalez I, Nelson MD. Best cases from the AFIP: Hemimegalencephaly. Radiographics 2004;24:843-8.  Back to cited text no. 1
    
2.
Abdel Razek AA, Kandell AY, Elsorogy LG, Elmongy A, Basett AA. Disorders of cortical formation: MR imaging features. AJNR Am J Neuroradiol 2009;30:4-11.  Back to cited text no. 2
    
3.
Barkovich AJ, Chuang SH. Unilateral megalencephaly: Correlation of MR imaging and pathologic characteristics. AJNR Am J Neuroradiol 1990;11:523-31.  Back to cited text no. 3
    
4.
Yagishita A, Arai N, Tamagawa K, Oda M. Hemimegalencephaly: Signal changes suggesting abnormal myelination on MRI. Neuroradiology 1998;40:734-8.  Back to cited text no. 4
    
5.
Lang SS, Goldberg E, Zarnow D, Johnson MP, Storm PB, Heuer GG. Prenatal diagnosis of hemimegalencephaly. World Neurosurg 2014;82:241.e5-8.  Back to cited text no. 5
    


    Figures

  [Figure 1]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow