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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