A group of recessively inherited diseases characterized by the intralysosomal accumulation of g m2) ganglioside in the neuronal cells. Subtypes include mutations of enzymes in the beta-n-acetylhexosaminidases system or g m2) activator protein leading to disruption of normal degradation of gangliosides, a subclass of acidic glycosphingolipids.
E75.0 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
The 2023 edition of ICD-10-CM E75.0 became effective on October 1, 2022.
This is the American ICD-10-CM version of E75.0 – other international versions of ICD-10 E75.0 may differ.