Specific code 2014 ICD-9-CM Diagnosis Code 333.0
Other degenerative diseases of the basal ganglia
  • Billable Code
  • Short description: Degen basal ganglia NEC.
  • ICD-9-CM 333.0 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 333.0 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Aicardi Goutieres syndrome
  • Autosomal dominant late onset basal ganglia degeneration
  • Azorean disease (disorder)
  • Basal ganglia degeneration with calcification
  • Choreoacanthocytosis
  • Cortical basal ganglionic degeneration
  • Corticobasal degeneration (disorder)
  • Corticobasalganglionic degeneration
  • Disorder of basal ganglia
  • Fahr's syndrome
  • Josephs disease
  • Juvenile paralysis agitans of Hunt
  • Machado Joseph disease
  • Multiple system atrophy
  • Multiple system atrophy, cerebellar variant (disorder)
  • Multiple system atrophy, Parkinson variant
  • Olivopontocerebellar atrophy
  • Olivopontocerebellar atrophy with blindness
  • Olivopontocerebellar atrophy with slow eye movement
  • Olivopontocerebellar degeneration
  • Pallidal degeneration
  • Pallidoluysian degeneration
  • Pallidonigral degeneration
  • Pallidonigrospinal degeneration
  • Pallidopontonigral degeneration
  • Parkinsonian syndrome associated with idiopathic orthostatic hypotension
  • Parkinsonism w orthostatic hypotension
  • Parkinsonism with orthostatic hypotension
  • Parkinsons variant
  • Pigmentary pallidal degeneration
  • Progressive supranuclear ophthalmoplegia
  • Progressive supranuclear palsy; ophthalmoplegia
  • Shy Drager syndrome
  • Shy-Drager syndrome
  • Sporadic olivopontocerebellar atrophy
  • Striatonigral degeneration
Clinical Information
  • A progressive neurological disorder characterized by ophthalmoplegia, dystonia, memory impairment, personality disorders, and dementia. Etiology is unknown.
  • A rare neuroaxonal dystrophy, histologically characterized by axonal spheroids, iron deposition, lewy body (lb)-like intraneuronal inclusions and neurofibrillary tangles.
  • Spastic weakness of the muscles innervated by the cranial nerves, i.e., the muscle of the face, pharynx, and tongue, due to bilateral lesions of the corticospinal tract.
Applies To
  • Atrophy or degeneration:
    • olivopontocerebellar [Déjérine-Thomas syndrome]
    • pigmentary pallidal [Hallervorden-Spatz disease] striatonigral
  • Parkinsonian syndrome associated with:
    • idiopathic orthostatic hypotension
    • symptomatic orthostatic hypotension
  • Progressive supranuclear ophthalmoplegia
  • Shy-Drager syndrome
Convert to ICD-10-CM: 333.0 converts approximately to:
  • 2014 ICD-10-CM G23.0 Hallervorden-Spatz disease
    Or:
  • 2014 ICD-10-CM G23.1 Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]
    Or:
  • 2014 ICD-10-CM G23.2 Striatonigral degeneration
    Or:
  • 2014 ICD-10-CM G23.8 Other specified degenerative diseases of basal ganglia
ICD-9-CM Volume 2 Index entries containing back-references to 333.0:
  • Atrophy, atrophic
    • Déjérine-Thomas 333.0
    • olivopontocerebellar 333.0
  • Degeneration, degenerative
    • basal nuclei or ganglia NEC 333.0
    • olivopontocerebellar (familial) (hereditary) 333.0
    • pallidal, pigmentary (progressive) 333.0
    • pigmentary (diffuse) (general)
      • localized - see Degeneration, by site
      • pallidal (progressive) 333.0
      • secondary 362.65
    • strionigral 333.0
  • Déjérine-Thomas atrophy or syndrome 333.0
  • Disease, diseased - see also Syndrome
    • basal ganglia 333.90
      • degenerative NEC 333.0
      • specified NEC 333.89
    • Hallervorden-Spatz 333.0
  • Hallervorden-Spatz disease or syndrome 333.0
  • Hypotension (arterial) (constitutional) 458.9
    • orthostatic (chronic) 458.0
      • dysautonomic-dyskinetic syndrome 333.0
  • Insufficiency, insufficient
    • idiopathic autonomic 333.0
  • Ophthalmoplegia (see also Strabismus) 378.9
    • supranuclear, progressive 333.0
  • Palsy (see also Paralysis) 344.9
    • progressive supranuclear 333.0
    • supranuclear NEC 356.8
      • progressive 333.0
  • Parkinsonism (arteriosclerotic) (idiopathic) (primary) 332.0
    • associated with orthostatic hypotension (idiopathic) (symptomatic) 333.0
  • Shy-Drager syndrome (orthostatic hypotension with multisystem degeneration) 333.0
  • Steele-Richardson (-Olszewski) Syndrome 333.0
  • Syndrome - see also Disease
    • Déjérine-Thomas 333.0
    • Hallervorden-Spatz 333.0
    • orthostatic hypotensive-dysautonomic-dyskinetic 333.0
    • progressive pallidal degeneration 333.0
    • Shy-Drager (orthostatic hypotension with multisystem degeneration) 333.0
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 333.0 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 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.