Abstract
Spastic paraplegia type 11 (SPG11) is the most frequent autosomal recessive HSP. SPG11 patients' fibroblasts and mouse models revealed endolysosomal system dysfunction; endolysosomal lipid accumulation, especially gangliosides, was observed in post-mortem brains. We report an atypical patient with clinical onset and biopsy findings mimicking a GM2 gangliosidoses.
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