Specific code 2015 ICD-9-CM Diagnosis Code 330.0
Leukodystrophy
  • 2015
  • Billable Thru Sept 30/2015
  • Non-Billable On/After Oct 1/2015

  • ICD-9-CM 330.0 is a billable medical code that can be used to indicate a diagnosis on a reimbursement claim, however, 330.0 should only be used for claims with a date of service on or before September 30, 2015. For claims with a date of service on or after October 1, 2015, use an equivalent ICD-10-CM code (or codes).
Convert to ICD-10-CM: 330.0 converts approximately to:
  • 2015/16 ICD-10-CM E75.23 Krabbe disease
    Or:
  • 2015/16 ICD-10-CM E75.25 Metachromatic leukodystrophy
    Or:
  • 2015/16 ICD-10-CM E75.29 Other sphingolipidosis
Approximate Synonyms
  • Canavan disease
  • Galactosylceramide beta-galactosidase deficiency
  • Globoid cell leukodystrophy, early onset
  • Globoid cell leukodystrophy, late onset
  • Globoid cell leukodystrophy, late-onset
  • Krabbe disease
  • Krabbes disease
  • Metachromatic leucodystrophy (disorder)
  • Metachromatic leukodystrophy
  • Pelizaeus Merzbacher disease
  • Pelizaeus-Merzbacher disease
  • Spongy degeneration of central nervous system
Clinical Information
  • A degenerative disease of the central and peripheral nervous system caused by abnormal breakdown and turnover of myelin and marked by reduced galacosylceramide beta-galactosidase activity (ec 3.2.1.46). Two types based on the age of onset are recognized: infantile-onset krabbe disease is marked by the appearance of symptoms at ages 3-6 months, which include irritability, frequent crying, and increase of muscle tonus. They are followed by opisthotonos, less of tendon reflexes, visual failure, elevated cerebrospinal fluid proteins, and delayed nerve conduction velocity. Most infants die during the second year of life. Late-onset krabbe disease (lokd) has first symptoms at ages 5 to 10 years, consisting of focal neurological signs, hemiparesis, cerebellar ataxia, cortical blindness, and spastic paraplegia, followed by mental and physical deterioration. Some patients survive into adulthood
  • A dysmyelinating disorder of the central nervous system associated with a decrease in all myelin proteins, especially proteolipid protein. Several types are recognized: acute infantile types synonyms: familial chronic infantile diffuse sclerosis perinatal sudanophilic leukodystrophy chronic infantile diffuse cerebral sclerosis adult-onset leukodystrophy synonyms: late-onset type multiple sclerosis-like disorder autosomal dominant pelizaeus-merzbacher disease pelizaeus-merzbacher-like disease (pmld) synonym: pelizaeus-medrzbacher disease with normal proteolipid protein in its classical form, the disease has an onset in the first three months of life with nystagmus followed by slowly progressive psychomotor retardation, involuntary movements, ataxia, and death in the second decade of life. The chronic infantile type may begin as early as the eight day of life and is slowly progressive so that the patient may survive to middle age. The early symptoms include rotary movements of the head and eyes, which may vanish later in life, usually followed by spasticity of the legs and arms, cerebellar ataxia, dementia, and parkinsonian tremor. The adult form has its onset in the fourth or fifth decades and is marked mainly by cerebellar, autonomic, and pyramidal disorders. Urinary and rectal incontinence, orthostatic hypotension, and progressive spasticity are the main symptoms. Pelizaeus-merzbacher-like disease is marked by symptoms characteristic of the classical form in, but with normal myelin which, however, is arranged into ball-like structures in the oligodendrocyte perikarya and terminal processes
  • Autosomal recessive lysosomal storage disease caused by a deficiency of cerebroside sulfatase leading to an accumulation of cerebroside sulfate in the nervous system and other organs
  • Group of diseases affecting the white matter of the brain, especially the cerebral hemispheres, due to defects in the formation and maintenance of myelin in infants and children
  • Inherited, demyelinating, human lipid storage disease caused by a deficiency of galactosylceramidase; manifestations include convulsions, quadriplegia, blindness, deafness, and mental retardation
Applies To
  • Krabbe's disease
  • Leukodystrophy:
    • NOS
    • globoid cell
    • metachromatic
    • sudanophilic
  • Pelizaeus-Merzbacher disease
  • Sulfatide lipidosis
ICD-9-CM Volume 2 Index entries containing back-references to 330.0:
  • Aplasia - see also Agenesis
    • axialis extracorticalis (congenital) 330.0
    • extracortical axial 330.0
    • Pelizaeus-Merzbacher 330.0
  • Canavan's disease 330.0
  • Disease, diseased - see also Syndrome
    • Canavan's 330.0
    • Greenfield's 330.0
    • Krabbe's 330.0
    • Merzbacher-Pelizaeus 330.0
    • Pelizaeus-Merzbacher 330.0
      • with dementia
        • with behavioral disturbance 330.0 [294.11]
        • without behavioral disturbance 330.0 [294.10]
    • Scholz's 330.0
    • van Bogaert-Nijssen (-Peiffer) 330.0
  • Encephalopathy (acute) 348.30
    • leukopolio 330.0
  • Greenfield's disease 330.0
  • Krabbe's
    • disease (leukodystrophy) 330.0
  • Leukodystrophy (cerebral) (globoid cell) (metachromatic) (progressive) (sudanophilic) 330.0
  • Leukoencephalopathy (see also Encephalitis) 323.9
    • metachromatic 330.0
  • Leukopolioencephalopathy 330.0
  • Lipidosis 272.7
    • sulfatide 330.0
  • Merzbacher-Pelizaeus disease 330.0
  • Myeloleukodystrophy 330.0
  • Pelizaeus-Merzbacher
    • disease 330.0
    • sclerosis, diffuse cerebral 330.0
  • Scholz's disease 330.0
  • Scholz (-Bielschowsky-Henneberg) syndrome 330.0
  • Sclerosis, sclerotic
    • brain (general) (lobular) 348.89
      • Alzheimer's - see Alzheimer's, dementia
      • artery, arterial 437.0
      • atrophic lobar 331.0
      • diffuse 341.1
        • familial (chronic) (infantile) 330.0
        • infantile (chronic) (familial) 330.0
        • Pelizaeus-Merzbacher type 330.0
      • disseminated 340
      • hereditary 334.2
      • hippocampal 348.81
      • infantile (degenerative) (diffuse) 330.0
      • insular 340
      • Krabbe's 330.0
      • mesial temporal 348.81
      • miliary 340
      • multiple 340
      • Pelizaeus-Merzbacher 330.0
      • progressive familial 330.0
      • senile 437.0
      • temporal 348.81
      • tuberous 759.5
    • centrolobar, familial 330.0
  • Sulfatidosis 330.0
  • Syndrome - see also Disease
    • Scholz (-Bielschowsky-Henneberg) 330.0
  • van Bogaert-Nijssen (-Peiffer) disease 330.0
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 330.0 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2015 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.