Leukoencephalopathy with spheroids (HDLS) and pigmentary leukodystrophy (POLD): A single entity?

C. Wider, J. A. Van Gerpen, S. Dearmond, E. A. Shuster, D. W. Dickson, Z. K. Wszolek

Research output: Contribution to journalReview article

78 Scopus citations

Abstract

Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) and familial pigmentary orthochromatic leukodystrophy (POLD) present as adult-onset dementia with motor impairment and epilepsy. They are regarded as distinct diseases. We review data from the literature that support their being a single entity. Apart from a slightly older age at onset, a more rapid course, and more prominent pyramidal tract involvement, familial POLD is clinically similar to HDLS. Moreover, the pathologic hallmarks of the two diseases, axonal spheroids in HDLS and pigmented macrophages in POLD, can be identified in both conditions. This supports HDLS and POLD being referred collectively as adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).

Original languageEnglish (US)
Pages (from-to)1953-1959
Number of pages7
JournalNeurology
Volume72
Issue number22
DOIs
StatePublished - Jun 2 2009

ASJC Scopus subject areas

  • Clinical Neurology

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