2013 ICD-9-CM Diagnosis Code 330.0
- 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.
- 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 220.127.116.11). 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.
- 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.
- 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.
- ICD-9-CM 330.0 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Convert 330.0 to ICD-10-CM
ICD-9-CM Volume 2 Index entries containing back-references to 330.0: