Typically affects boys aged 5-10 years
The most common mode of inheritance is X-linked recessive. 
The disease starts around the lateral trigones. 
Coexists with adrenal insufficiency (neurological symptoms appear first). Death occurs within 2 years.

•  Preferential symmetrical involvement of the optic and auditory pathways, the splenium of the corpus callosum, posterior periventricular white matter.
•  Areas of active inflammation enhance with contrast.


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