GM_2 gangliosidosis / 临床神经病学杂志

Objective To probe into the clinical features and pathological change of GM 2 gangliosidosis. Methods The clinical manifestations of 2 patients with late onset GM 2 gangliosidosis were reported, a biopsy of the right frontal lobe was performed for each case.Results The clinical manifestations of the late onset GM 2 gangliosidosis were nonspecific, the ballooned neurons with cytoplasmic deposits were found under the light microscopy.The deposits were membranous cytoplasmic bodies(MCB) together with zebra bodies.Conclusion The result suggested that light microscopy with electron microscopy in taking brain biopsy was very important for the diagnosis of GM 2 gangliosidosis.