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We analyzed survival by decade to assess the impact of historical changes in the management of care. Mean age at death and the 5-year survival from onset of symptoms for late infantile, juvenile, and adult phenotypes were 4.2 years and 24.9%, 17.4 years and 70.3%, and 43.1 years and 88.6%, respectively. The 5-year survival of cases reported after 1990 was significantly better than cases reported before 1970 in all subtypes of metachromatic leukodystrophy (late infantile: 52% vs 14%, juvenile: 100% vs 46%, adult: 95% vs 67%). Survival in the late infantile subtype was worse than that in other subtypes. Survival significantly improved over time in all subtypes.<\/jats:p>","DOI":"10.1177\/0883073809341669","type":"journal-article","created":{"date-parts":[[2009,12,28]],"date-time":"2009-12-28T20:54:56Z","timestamp":1262033696000},"page":"572-580","source":"Crossref","is-referenced-by-count":53,"title":["Metachromatic Leukodystrophy: A Case of Triplets With the Late Infantile Variant and a Systematic Review of the Literature"],"prefix":"10.1177","volume":"25","author":[{"given":"Asif","family":"Mahmood","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of Texas Medical Branch, Galveston, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jay","family":"Berry","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Children's Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Wenger","sequence":"additional","affiliation":[{"name":"Department of Neurology, Jefferson Medical College, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Escolar","sequence":"additional","affiliation":[{"name":"Program for Neurodevelopmental Function in Rare Disorders, University of North Carolina, Chapel Hill, North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdi","family":"Sobeih","sequence":"additional","affiliation":[{"name":"Department of Neurology, Children's Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald","family":"Raymond","sequence":"additional","affiliation":[{"name":"Department of Neurogenetics, Kennedy Krieger Institute, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian S.","family":"Eichler","sequence":"additional","affiliation":[{"name":"Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2009,12,28]]},"reference":[{"key":"atypb1","unstructured":"von-Figura K., Gieselmann V., Jaeken J. 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