2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Diseases Of The Nervous System And Sense Organs 320-389 > Hereditary And Degenerative Diseases Of The Central Nervous System 330-338 > Cerebral Degenerations Usually Manifest In Childhood 330.* > 2006 ICD-9-CM Diagnosis 330.1 Cerebral lipidoses View the most recent version of ICD-9-CM 330.1 An autosomal recessive inherited gangliosidosis characterized by the onset in the first 6 months of life of an exaggerated startle response, delay in psychomotor development, hypotonia (followed by spasticity), visual loss, and a macular cherry red spot. Hexosaminidase A (see BETA-N-ACETYLHEXOSAMINIDASE) is deficient, leading to the accumulation of GM2 ganglioside in neurons of the central nervous system and retina. This condition is strongly associated with Askenazic Jewish ancestory. A group of often fatal inherited diseases marked by the accumulation of GANGLIOSIDES in LYSOSOMES secondary to enzymatic deficiency states. Gangliosidoses include TAY-SACHS DISEASE; GANGLIOSIDOSIS GM1; GANGLIOSIDOSES GM2; and SANDHOFF DISEASE; which share the infantile or childhood onset of CENTRAL NERVOUS SYSTEM deterioration. 330.1 is a specific code that can be used to specify a diagnosis 330.1 contains 28 index entries View the ICD-9-CM Volume 1 330.* hierarchy Alternate Terminology Amaurotic (familial) idiocy Disease: Batten Jansky-Bielschowsky Kufs' Spielmeyer-Vogt Tay-Sachs Gangliosidosis  Index entries containing 330.1: Amaurotic familial idiocy (infantile) (juvenile) (late) 330.1 Bielschowsky's disease 330.1 Bielschowsky-Jansky amaurotic familial idiocy 330.1 disease 330.1 Cerebromacular degeneration 330.1 Degeneration, degenerative brain (cortical) (progressive) 331.9 in lipidosis cerebral 330.1 cerebromacular 330.1 Disease, diseased - see also Syndrome Bielschowsky (-Jansky) 330.1 Jansky-Bielschowsky 330.1 Kufs' 330.1 Sachs (-Tay) 330.1 Sandhoff's 330.1 Spielmeyer-Stock 330.1 Spielmeyer-Vogt 330.1 Tay-Sachs 330.1 Vogt-Spielmeyer 330.1 Gangliosidosis 330.1 Idiot, idiocy (congenital) 318.2 amaurotic (Bielschowsky) (-Jansky) (family) (infantile (late)) (juvenile (late)) (Vogt-Spielmeyer) 330.1 Jansky-Bielschowsky amaurotic familial idiocy 330.1 Kufs' disease 330.1 Lipidosis 272.7 cerebral (infantile) (juvenile) (late) 330.1 Sachs (-Tay) disease (amaurotic familial idiocy) 330.1 Sandhoff's disease 330.1 Spielmeyer-Stock disease 330.1 Spielmeyer-Vogt disease 330.1 Tay-Sachs amaurotic familial idiocy 330.1 disease 330.1 Vogt-Spielmeyer disease (amaurotic familial idiocy) 330.1