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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 939-942
Radiological quiz - neuroradiology


Dept. of Radiodiagnosis, Lady Hardinge Medical College & Associated Hospitals, New Delhi, India

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Date of Submission23-Aug-2006
Date of Acceptance20-Nov-2006
 

How to cite this article:
Narula M K, Kapoor G, Deria K, Patwari A K, Anand R. Radiological quiz - neuroradiology. Indian J Radiol Imaging 2006;16:939-42

How to cite this URL:
Narula M K, Kapoor G, Deria K, Patwari A K, Anand R. Radiological quiz - neuroradiology. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 22];16:939-42. Available from: http://www.ijri.org/text.asp?2006/16/4/939/32392
A 4-year-old boy, born of consanguineous marriage in a Muslim community presented with history of progressive enlargement of head and delayed acquisition of motor milestones and speech. There was no history of seizures, any visual, or auditory deficit. His head circumference was 53.5-cm. The reflexes were brisk in the lower limbs with bilateral external planter responses. EEG showed generalized dysrythmia. MRI and in vivo MRS performed are shown in [Figure - 1][Figure - 2][Figure - 3][Figure - 4][Figure - 5][Figure - 6]. Similar findings were present in younger sibling. What is your diagnosis?


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   References Top

1.Van der knaap MS, Barth PG, Stroink, H et al. Leucoencephalopathy with swelling and a discrepantly mild clinical course in eight children. Ann Neurol 1995; 37: 324-334.   Back to cited text no. 1    
2.Topcu N, Saatci I, Topcuoglu MA, Kose G, Kunak B. Megalencephalopathy and Leucodystrophy with mild clinical course: a report on 12 new cases. Brain Dev 1998; 20: 142-153.  Back to cited text no. 2    
3.Singhal BS, Gursahani RD, Udani BP et al. Megalencephalic leukodystrophy in an Asian Indian ethnic group. Pediatr Neurol 1996; 14: 291-296.  Back to cited text no. 3    
4.Leegwater PAJ, Yuan BQ, Van der Steen J et al. Mutation of the MLC1 (KIAAOO27), encoding a putative membrane protein causing megalencephalic leucoencephalopathy with suubcortical cysts. Am J Hum Genet 2001; 68: 831-838.  Back to cited text no. 4    
5.Pande S, Agarwal R, Nagpal T, Kaur A. Infantile onset leukoencephalopathy with swelling and a discrepantly mild clinical course- A case report. Ind J Radiol Imag 2004; 14: 2: 125-127.  Back to cited text no. 5    
6.Chandrashekar HS, Guruprasad AS, Jaykumar PN, Srikanth SG, Taly AB. Megalencephalic leukoencephalopathy with subcortical cysts: MRI and proton spectroscopic features. Neurology India 2003; 51:4:525-527   Back to cited text no. 6    
7.Yi-Fang Tu, Cheng-Yu Chen, Chao-Ching Huang and Chang-Shin Lee. Vacuolating megaencephalic leukoencephalopathy with mild clinical course validated by Diffusion Tensor imaging and MR Spectroscopy. Am J Neuroradiol 2004 Jun-Jul; 25(6): 1041-5.  Back to cited text no. 7    

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Correspondence Address:
M K Narula
Dept. of Radiodiagnosis, Lady Hardinge Medical College & Associated Hospitals, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32392

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]



 

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