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Leukodystrophies 01

Topic: Imaging

Created on Saturday, December 8 2007 by jdmiles

Last modified on Saturday, December 8 2007.

During the workup of a child with a developmental delay, you obtain the above MRI. This image is most consistent with which of the following diagnoses?


 
        A) Alexander disease
 
        B) Zellweger syndrome
 
        C) Leigh disease
 
        D) Adrenoleukodystrophy
 
        E) Normal variant
 

 


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This question was created on December 08, 2007 by jdmiles.
This question was last modified on December 08, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Alexander disease

This answer is correct.


The findings in this MRI are typical for Alexander disease. MRI criteria for the diagnosis of Alexander disease include:

  1. Extensive, frontally-predominant cerebral white matter changes
  2. A periventricular rim which is bright on T1 and dark on T2
  3. Brainstem abnormalities
  4. Abnormalities of hte basal ganglia and thalami
  5. Contrast enhancement of particular gray and white matter structures.
Four of 5 criteria should be met to make the diagnosis based on imaging.   (See References)

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B) Zellweger syndrome

This answer is incorrect.


Typical findings in Zellweger syndrome include abnormal gyration and diffuse demyelination. This image shows a frontally-predominant demyelination, which is not characteristic of Zellweger syndrome.   (See References)

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C) Leigh disease

This answer is incorrect.


Leigh disease does not often affect the cerebral white matter. Demyelination in Leigh disease occurs in the caudate, globus pallidus, and brainstem.   (See References)

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D) Adrenoleukodystrophy

This answer is incorrect.


Adrenoleukodystrophy typically has a characteristic pattern on MRI, in which the occipital and parietal white matter is affected first. As the disease progresses, the leukodystrophy moves anteriorly and caudally. There are atypical cases in which the anterior white matter is affected first; however, classically, the initial demyelination occurs in peritrigonal areas.   (See References)

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E) Normal variant

This answer is incorrect.


This MRI shows hyperintense lesions in the frontal white matter. This is not normal.   (See References)

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References:

1. Fenichel, G.M. (2005). Clinical Pediatric Neurology, 5th ed. Elsevier, Philadelphia.
2. Robert I. Grossman, David M. Yousem. Neuroradiology: the requisites. Philadelphia, Pa. : Mosby, c2003. (ISBN:978032300508X) Advertising:
3. Sklar, E.M.L., Ruiz, A., Quencer, R.M., and Falcone, S.F. (2004). Structural neuroimaging. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 521-597 (ISBN:0750674695). Advertising:
4. Cheon, J., Kim, I., Hwang, Y.S., Kim, K.J., Wang, K., Cho, B., Chi, J.G., Kim, C.J., Kim, W.S., and Yeon, K.M. (2002). "Leukodystrophy in children: a pictorial review of MR imaging features." Radiographics, 22(3) 461-76. (PMID:12006681)
5. Magnaldi, S. (1991). "[Leukodystrophies: clinical aspects and findings with computerized tomography and magnetic resonance imaging]" Radiol Med (Torino), 82(1-2) 13-26. (PMID:1896564)
6. van der Knaap, M.S., Naidu, S., Breiter, S.N., Blaser, S., Stroink, H., Springer, S., Begeer, J.C., van Coster, R., Barth, P.G., Thomas, N.H., Valk, J., and Powers, J.M. (2001). "Alexander disease: diagnosis with MR imaging." AJNR Am J Neuroradiol, 22(3) 541-52. (PMID:11237983)
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imaging
Leukodystrophies 01
Question ID: 101207183
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 12/08/2007
Modified: 12/08/2007
Estimated Permutations: 8400

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