Leukoencephalopathy with neuroaxonal spheroids

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Leukoencephalopathy with neuroaxonal spheroids is a special kind of leukoencephalopathy. It is a cause of severe and subacute dementia. It is inherited, following an autosomal dominant pattern.

It usually presents in childhood but it can also have an adult onset,[1] in which case it can present MRIs that mimic those of progressive multiple sclerosis.[2]

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Contributors: Prab R. Tumpati, MD