ICD-10-CM 2027
ICD-10 code for lipofuscinosis
From the official ICD-10-CM alphabetic index entry “Lipofuscinosis, neuronal (with ceroidosis)”. Page updated September 29, 2026.
About coding lipofuscinosis
The ICD-10-CM code for lipofuscinosis is E75.4 (Neuronal ceroid lipofuscinosis).
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.
Common questions about lipofuscinosis ICD-10 codes
What is the ICD-10 code for lipofuscinosis?
- E75.4 — Neuronal ceroid lipofuscinosis.
Is E75.4 billable?
- Yes. E75.4 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.4 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.4 as a principal diagnosis.
Is E75.4 a CC or MCC?
- E75.4 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.