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,<ref>,
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP): Integrating the literature on hereditary diffuse leukoencephalopathy with spheroids (HDLS) and pigmentary orthochromatic leukodystrophy (POLD), Journal of Clinical Neuroscience, Vol. 48(Issue: 1), pp. 42–49, DOI: 10.1016/j.jocn.2017.10.060, PMID: 29122458,</ref> in which case it can present MRIs that mimic those of progressive multiple sclerosis.<ref>, Sporadic adult-onset leukoencephalopathy with neuroaxonal spheroids mimicking cerebral MS, Neurology, Vol. 70(Issue: 13 Pt 2), pp. 1128–33, DOI: 10.1212/01.wnl.0000304045.99153.8f, PMID: 18287567,</ref>
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