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

Détails

ID Serval
serval:BIB_81A8D5EB2367
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Leukoencephalopathy with spheroids (HDLS) and pigmentary leukodystrophy (POLD): a single entity?
Périodique
Neurology
Auteur(s)
Wider C., Van Gerpen J.A., DeArmond S., Shuster E.A., Dickson D.W., Wszolek Z.K.
ISSN
1526-632X[electronic], 0028-3878[linking]
Statut éditorial
Publié
Date de publication
2009
Volume
72
Numéro
22
Pages
1953-1959
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
Résumé
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).
Mots-clé
Age of Onset, Axons/pathology, Dementia/pathology, Dementia/physiopathology, Dementia, Vascular/pathology, Dementia, Vascular/physiopathology, Disease Progression, Humans, Leukodystrophy, Metachromatic/pathology, Leukodystrophy, Metachromatic/physiopathology, Macrophages/pathology, Neuroglia/pathology, Wallerian Degeneration/pathology, Wallerian Degeneration/physiopathology
Pubmed
Création de la notice
24/09/2010 19:03
Dernière modification de la notice
03/03/2018 18:48
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