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The progressive CNS pathology has its onset at different ages, leading to neurodegeneration and early death. <jats:italic>Methods<\/jats:italic>: Literature review provided insight into the current clinical neurological findings, phenotypic spectrum, and pathogenic mechanisms of LASDs with primary neurological involvement. <jats:italic>Conclusions<\/jats:italic>: CNS signs and symptoms are variable and related to the disease\u2010specific underlying pathogenesis. LAS dysfunction leads to diverse global cellular consequences in the CNS ranging from specific axonal and dendritic abnormalities to neuronal death. Pathogenic mechanisms for disease progression vary from impaired autophagy, massive storage, regional involvement, to end\u2010stage inflammation. Some of these features are also found in adult neurodegenerative disorders, for example, Parkinson's and Alzheimer's diseases. Lack of effective therapies is a significant unmet medical need. \u00a9 2013 Wiley Periodicals, Inc. 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