E75.4Neuronal ceroid lipofuscinosis

E75.4 is the ICD-10-CM diagnosis code for Neuronal ceroid lipofuscinosis. It belongs to category E75 (disorders of sphingolipid metabolism and other lipid storage disorders), block E70-E88 (metabolic disorders) and chapter 4 (endocrine, nutritional and metabolic diseases). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

Within E75 (disorders of sphingolipid metabolism and other lipid storage disorders), E75.4 is specifically for Neuronal ceroid lipofuscinosis. Related codes cover GM2 gangliosidosis (E75.0), other and unspecified gangliosidosis (E75.1), other sphingolipidosis (E75.2), sphingolipidosis, unspecified (E75.3), other lipid storage disorders (E75.5) and lipid storage disorder, unspecified (E75.6).

As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.

In MS-DRG v44.0, it is part of the grouping logic for DRG 056 (Degenerative Nervous System Disorders with MCC, relative weight 2.2655) and DRG 057 (Degenerative Nervous System Disorders without MCC, relative weight 1.2838), in MDC 01 (Diseases and Disorders of the Nervous System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 330.1 (cerebral lipidoses).