`E75.4` Neuronal ceroid lipofuscinosis

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Risk adjustment (HCC)

Defines the logic of DRG(s) 056-057 in MDC 01.

Code First: F02 Dementia in other diseases classified elsewhere ("cerebral lipidosis (E75.4)")

Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
Code First: G73.7 Myopathy in diseases classified elsewhere ("lipid storage disorders (E75.-)")
Code First: H36 Retinal disorders in diseases classified elsewhere ("lipid storage disorders (E75.-)")
Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of lipid metabolism (E75.-)")

Amaurotic idiocy (infantile) (juvenile) (late)
Batten (-Mayou) disease
Batten (-Mayou) disease › retina
Bielschowsky (-Jansky) disease
Ceroid-lipofuscinosis, neuronal
Degeneration, degenerative › brain (cortical) (progressive) › in › lipidosis › cerebral
Disease, diseased › retina, retinal › Batten's or Batten-Mayou
Idiot, idiocy (congenital) › amaurotic (Bielschowsky(-Jansky)) (family) (infantile (late)) (juvenile (late)) (Vogt-Spielmeyer)
Jansky-Bielschowsky amaurotic idiocy
Kufs' disease
Lipidosis › cerebral (infantile) (juvenile) (late)
Lipidosis › cerebroretinal
Lipofuscinosis, neuronal (with ceroidosis)
Spielmeyer-Vogt disease
Vogt-Spielmeyer amaurotic idiocy or disease