E75.19
BillableOther gangliosidosis
HCC Category Mapping
RxHCCHCC 41 — Lysosomal Storage Disorders
0.000What This Code Means
A rare inherited metabolic disorder where fatty substances (gangliosides) accumulate in cells, causing neurological problems, with a specific type not listed in other gangliosidosis categories.
Coding Tips
- •Use this code only when the gangliosidosis type is documented but doesn't fit E75.11 or other specific codes
- •Verify genetic testing documentation supports the specific gangliosidosis diagnosis
Clinical Significance
Other gangliosidosis captures ganglioside storage disorders not classified as GM2 gangliosidosis or mucolipidosis IV, most notably GM1 gangliosidosis (beta-galactosidase deficiency). GM1 gangliosidosis causes progressive neurodegeneration, skeletal abnormalities, and hepatosplenomegaly. Accurate coding of the specific gangliosidosis type is important for genetic counseling and disease management.
Documentation Requirements
- ✓Specific gangliosidosis variant documented (e.g., GM1 gangliosidosis with type specification)
- ✓Enzyme assay results (beta-galactosidase for GM1) or genetic testing
- ✓Clinical form specified: infantile, late infantile/juvenile, or adult/chronic
- ✓Documentation of skeletal, neurological, and visceral manifestations
- ✓Family history and genetic counseling documentation
Commonly Confused Codes
E75.10 — Unspecified gangliosidosis: use only when no specific type can be identifiedE75.11 — Mucolipidosis IV: separate specific code for ML IVE75.00-E75.09 — GM2 gangliosidosis: for GM2 variants specificallyE76.01-E76.02 — Mucopolysaccharidosis Type I: can have overlapping skeletal features with GM1E75.22 — Gaucher disease: different lysosomal storage disorder