Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 5361  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
   Next article
   Previous article 
   Table of Contents
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (323 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
    Viewed3959    
    Printed79    
    Emailed0    
    PDF Downloaded106    
    Comments [Add]    

Recommend this journal

 


 
NEUROIMAGE
Year : 2010  |  Volume : 58  |  Issue : 4  |  Page : 680

"Split thalamus": Internal medullary involvement in Wilson's disease


1 Department of Radiodiagnosis, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Psychiatry, Christian Medical College and Hospital, Ludhiana, Punjab, India

Date of Acceptance22-Jun-2010
Date of Web Publication24-Aug-2010

Correspondence Address:
Uttam George
Department of Radiodiagnosis, Christian Medical College & Hospital, Brown Road, Ludhiana - 141 008, Punjab
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.68701

Rights and Permissions



How to cite this article:
George U, Varte N, Rathore S, Jain V, Goyal S. "Split thalamus": Internal medullary involvement in Wilson's disease. Neurol India 2010;58:680

How to cite this URL:
George U, Varte N, Rathore S, Jain V, Goyal S. "Split thalamus": Internal medullary involvement in Wilson's disease. Neurol India [serial online] 2010 [cited 2019 Aug 24];58:680. Available from: http://www.neurologyindia.com/text.asp?2010/58/4/680/68701


A 32-year-old woman presented to psychiatry outpatient department with decreased sleep, irrelevant speech, aggressive and bizarre behavior over a period of 3 months. Investigations showed decreased serum cerruloplasmin, increased 24 h urinary copper levels, and mild elevation of alkaline phosphatase and bilirubin with decrease in total proteins. Slit lamp examination of cornea showed  Kayser-Fleischer ring More Details. Brain magnetic resonance imaging (MRI) revealed symmetrical hyperintensities on T2/T2 fluid attenuation inversion recovery images in the putamen [Figure 1]a, thalami, midbrain, pons, and subcortical white matter of both frontal lobes. Hyperintensity of Fields of Florel and internal medullary laminae was also seen [Figure 1]b and c. The patient was started on atypical antipsychotics along with a copper chelating agent. No psychiatric manifestations or worsening was observed during the hospital stay and during the 6 month follow-up.
Figure 1 :(a) T2 FLAIR axial image shows the bilateral putaminal (arrowhead) and thalamic involvement. (b) T2-weighted axial image shows the curvilinear hyperintense internal medullary lamina (arrowhead) between the medial and lateral thalamic groups of nuclei with a "split thalamus" appearance. (c) Medial and lateral nuclear groups of the thalamus on either side of the hyperintense internal medullary lamina (arrowhead) seen on the coronal T2 image. Hyperintensity in the fields of florel is also seen (arrow)

Click here to view


Psychiatric manifestations may be the initial presentation in significant proportion of patients with Wilson's disease (WD). [1] MRI provides an insight into the pathologic and anatomic correlates of clinical manifestations in WD. [2] Based on neuropsychiatric symptoms and structural brain lesions, Oder et al. identified 3 subgroups. Imaging showed dilatation of the third ventricle, focal thalamic lesions, and putaminal and pallidal lesions in the 3 subgroups, respectively. [3] The findings in our case were similar to an MRI study of 100 patients with WD showing atrophic changes of the cerebrum, brainstem, and cerebellum, as well as signal abnormalities in the basal ganglia, thalami, brainstem, cerebellum, and cerebral white matter. [2] An additional interesting, previously unreported finding in our case was the involvement of the Fields of Florel and the internal medullary lamina (IML) between the medial and lateral thalamic groups of nuclei. The IML involvement seen as a curvilinear hyperintensity appeared to divide the thalamus into 2 halves giving a characteristic "split thalamus" appearance. While thalamic involvement in WD is common, no mention of changes in the IML is previously reported. The only MR differential for such IML involvement is Fucosidosis type 1, a rare autosomal recessive lysosomal storage disease. [4]

 
  References Top

1.Schwartz M, Fuchs S, Polak H, Sharf B. Psychiatric manifestations in Wilson's disease. Harefuah 1993;124:75-7.  Back to cited text no. 1  [PUBMED]    
2.Sinha S, Taly AB, Ravishankar S, Prashanth LK, Venugopal KS, Arunodaya GR, et al. Wilson's disease: Cranial MRI observations and clinical correlation. Neuroradiology 2006;48:613-21.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Oder W, Prayer L, Grimm G, Spatt J, Ferenci P, Kollegger H, et al. Wilson's disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology 1993;43:120-4.   Back to cited text no. 3  [PUBMED]    
4.Galluzzi P, Rufa A, Balestri P, Cerase A, Federico A. MR brain imaging of fucosidosis type I. AJNR Am J Neuroradiol 2001;22:777-80.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1]



 

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