Homepage (Cockayne-Syndrome.org)
 

Brain injury in CS

 

Calcification

Hydrocephalus 
Loss of Myelin
Purkinje Cells
Developmental delay is expected when CS is present in a sufficiently severe or advanced form to allow confident clinical diagnosis. Here are some brain changes that occur in CS patients.
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Calcification    

 

Here is a CT scan of a CS patient, showing calcium deposits in the cerebellum (white area next to + sign).

 

             

Calcification (click to enlarge)

           Calcification

 

 

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Hydrocephalus    

 

The amount of brain tissue also is likely to be unusually small. Here is a CT image of a CS patient showing hydrocephalus ex vacuo i.e., abnormally large amount of fluid in skull filling space normally occupied by brain tissue; the ventricles (large, dark, rounded, symmetrical areas near center) are abnormally dilated with cerebrospinal fluid instead of being compressed and slit.like. Also, there are symmetrical, wavy, white areas of dense calcium deposition in the basal ganglia of the brain (marked on left with +).

 

          

Hydrocephalus (click to enlarge)

          Hydrocephalus

 

 

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Loss of Myelin    

 

The white matter of the brain may be deficient in myelin in CS patients. Here is a photomicrograph of cerebral tissue appearing foamy due to loss of myelin in the white matter.

 

         

Loss of Myelin (click to enlarge)

            Loss of Myelin

 

 

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Purkinje Cells   

Neurons are likely to be scarcer than expected. Here is a photomicrograph of cerebellar tissue, showing abnormally few, irregularly spaced large cerebellar neurons (Purkinje cells).

 

         

Purkinje Cells (click to enlarge)

             Purkinje Cells

 

 

 

 

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Windows CD-ROM    

Illustrations on this page came from "Xeroderma Pigmentosum and Cockayne's Syndrome A Multimedia Overview" (beta version), copyright 1998 by American Registry of Pathology. For more information about this CD ROM product, please click here:

Windows CD-ROM (beta) for CS and XP