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2008 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 330-337 > Cerebral Degenerations Usually Manifest In Childhood 330.* > 2008 ICD-9-CM Diagnosis 330.1 Cerebral lipidoses 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. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97) 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. (Menkes, Textbook of Pediatric Neurology, 5th ed pp89-96) 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 330.1 also known as: 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