The underlying cause is a deficiency in vitamin B complex.
The condition is most often seen in chronic alcoholics. Early diagnosis, vitamin replacement and abstinence may be associated with improved outcomes.
Clinical presentations:
(1) acute with sudden onset, seizures, stupor, coma and death
(2) chronic with dysarthria and dementia
Clinical findings may include:
(1) frontal lobe syndrome with personality change and/or psychomotor slowing
(2) disorientation
(3) quadriparesis
(4) incontinence
(5) interhemispheric disconnection
Examination of the brain shows symmetrical demyelination and necrosis of the middle portion of the corpus callosum. Clinical variation occurs based on the extent of damage in the corpus callosum and when there is damage to other structures.
The lesions can be visualized on MRI with high T2 signal intensity and with diffusion-weighted imaging (DWI). Late disease may be associated with atrophy and cystic degeneration.
