HEREDITARY AND DEGENERATIVE DISEASES OF THE CENTRAL NERVOUS SYSTEM (330-337)

• Excludes: hepatolenticular degeneration (275.1)
  • multiple sclerosis (340)
  • other demyelinating diseases of central nervous system (341.0-341.9)
• 330 Cerebral degenerations usually manifest in childhood
  • Use additional code to identify associated mental retardation
  • 330.0 Leukodystrophy
    • Krabbe's disease
    • Leukodystrophy:
      • NOS
      • globoid cell
      • metachromatic
      • sudanophilic
    • Pelizaeus-Merzbacher disease
    • Sulfatide lipidosis
  • 330.1 Cerebral lipidoses
    • Amaurotic (familial) idiocy
    • Disease:
      • Batten
      • Jansky-Bielschowsky
      • Kufs'
      • Spielmeyer-Vogt
      • Tay-Sachs
      • Gangliosidosis
  • 330.2 Cerebral degeneration in generalized lipidoses
    • Code first underlying disease, as:
      • Fabry's disease (272.7)
      • Gaucher's disease (272.7)
      • Niemann-Pick disease (272.7)
      • sphingolipidosis (272.7)
  • 330.3 Cerebral degeneration of childhood in other diseases classified elsewhere
    • Code first underlying disease, as:
      • Hunter's disease (277.5)
      • mucopolysaccharidosis (277.5)
  • 330.8 Other specified cerebral degenerations in childhood
    • Alpers' disease or gray-matter degeneration
    • Infantile necrotizing encephalomyelopathy
    • Leigh's disease
    • Subacute necrotizing encephalopathy or encephalomyelopathy
  • 330.9 Unspecified cerebral degeneration in childhood
• 331 Other cerebral degenerations
  • 331.0 Alzheimer's disease
  • 331.1 Pick's disease
  • 331.2 Senile degeneration of brain
    • Excludes: senility NOS (797)
  • 331.3 Communicating hydrocephalus
    • Excludes: congenital hydrocephalus (741.0, 742.3)
  • 331.4 Obstructive hydrocephalus
    • Acquired hydrocephalus NOS
    • Excludes: congenital hydrocephalus (741.0, 742.3)
  • 331.7 Cerebral degeneration in diseases classified elsewhere
    • Code first underlying disease, as:
      • alcoholism (303.0-303.9)
      • beriberi (265.0)
      • cerebrovascular disease (430-438)
      • congenital hydrocephalus (741.0, 742.3)
      • neoplastic disease (140.0-239.9)
      • myxedema (244.0-244.9)
      • vitamin B12 deficiency (266.2)
    • Excludes: cerebral degeneration in:
      • Jakob-Creutzfeldt disease (046.1)
      • progressive multifocal leukoencephalopathy (046.3)
      • subacute spongiform encephalopathy (046.1)
  • 331.8 Other cerebral degeneration
    • 331.81 Reye's syndrome
    • 331.89 Other
      • Cerebral ataxia
  • 331.9 Cerebral degeneration, unspecified
• 332 Parkinson's disease
  • 332.0 Paralysis agitans
    • Parkinsonism or Parkinson's disease:
      • NOS
      • idiopathic
      • primary
  • 332.1 Secondary Parkinsonism
    • Parkinsonism due to drugs
    • Use additional E code to identify drug, if drug-induced
    • Excludes: Parkinsonism (in):
      • Huntington's disease (333.4)
      • progressive supranuclear palsy (333.0)
      • Shy-Drager syndrome (333.0)
      • syphilitic (094.82)
• 333 Other extrapyramidal disease and abnormal movement disorders
  • Includes: other forms of extrapyramidal, basal ganglia, or striatopallidal disease
  • Excludes: abnormal movements of head NOS (781.0)
  • 333.0 Other degenerative diseases of the basal ganglia
    • 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 or palsy
    • Shy-Drager syndrome
  • 333.1 Essential and other specified forms of tremor
    • Benign essential tremor
    • Familial tremor
    • Use additional E code to identify drug, if drug-induced
    • Excludes: tremor NOS (781.0)
  • 333.2 Myoclonus
    • Familial essential myoclonus
    • Progressive myoclonic epilepsy
    • Unverricht-Lundborg disease
    • Use additional E code to identify drug, if drug-induced
  • 333.3 Tics of organic origin
    • Excludes: Gilles de la Tourette's syndrome (307.23)
      • habit spasm (307.22)
      • tic NOS (307.20)
    • Use additional E code to identify drug, if drug-induced
  • 333.4 Huntington's chorea
  • 333.5 Other choreas
    • Hemiballism(us)
    • Paroxysmal choreo-athetosis
    • Excludes: Sydenham's or rheumatic chorea (392.0-392.9)
    • Use additional E code to identify drug, if drug-induced
  • 333.6 Idiopathic torsion dystonia
    • Dystonia:
      • deformans progressiva
      • musculorum deformans
    • (Schwalbe-) Ziehen-Oppenheim disease
  • 333.7 Symptomatic torsion dystonia
    • Athetoid cerebral palsy [Vogt's disease]
    • Double athetosis (syndrome)
    • Use additional E code to identify drug, if drug-induced
  • 333.8 Fragments of torsion dystonia
    • Use additional E code to identify drug, if drug-induced
    • 333.81 Blepharospasm
    • 333.82 Orofacial dyskinesia
    • 333.83 Spasmodic torticollis
      • Excludes: torticollis:
        • NOS (723.5)
        • hysterical (300.11)
        • psychogenic (306.0)
    • 333.84 Organic writers' cramp
      • Excludes: psychogenic (300.89)
    • 333.89 Other
  • 333.9 Other and unspecified extrapyramidal diseases and abnormal movement disorders
    • 333.90 Unspecified extrapyramidal disease and abnormal movement disorder
    • 333.91 Stiff-man syndrome
    • 333.92 Neuroleptic malignant syndrome
      • Use additional E code to identify drug
    • 333.93 Benign shuddering attacks
    • 333.99 Other
      • Restless legs
• 334 Spinocerebellar disease
  • Excludes: olivopontocerebellar degeneration (333.0)
    • peroneal muscular atrophy (356.1)
  • 334.0 Friedreich's ataxia
  • 334.1 Hereditary spastic paraplegia
  • 334.2 Primary cerebellar degeneration
    • Cerebellar ataxia:
      • Marie's
      • Sanger-Brown
    • Dyssynergia cerebellaris myoclonica
    • Primary cerebellar degeneration:
      • NOS
      • hereditary
      • sporadic
  • 334.3 Other cerebellar ataxia
    • Cerebellar ataxia NOS
    • Use additional E code to identify drug, if drug-induced
  • 334.4 Cerebellar ataxia indiseases classified elsewhere
    • Code first underlying disease, as:
      • alcoholism (303.0-303.9)
      • myxedema (244.0-244.9)
      • neoplastic disease (140.0-239.9)
  • 334.8 Other spinocerebellar diseases
    • Ataxia-telangiectasia [Louis-Bar syndrome]
    • Corticostriatal-spinal degeneration
  • 334.9 Spinocerebellar disease, unspecified
• 335 Anterior horn cell disease
  • 335.0 Werdnig-Hoffmann disease
    • Infantile spinal muscular atrophy
    • Progressive muscular atrophy of infancy
  • 335.1 Spinal muscular atrophy
    • 335.10 Spinal muscular atrophy, unspecified
    • 335.11 Kugelberg-Welander disease
      • Spinal muscular atrophy:
        • familial
        • juvenile
    • 335.19 Other
      • Adult spinal muscular atrophy
  • 335.2 Motor neuron disease
    • 335.20 Amyotrophic lateral sclerosis
      • Motor neuron disease (bulbar) (mixed type)
    • 335.21 Progressive muscular atrophy
      • Duchenne-Aran muscular atrophy
      • Progressive muscular atrophy (pure)
    • 335.22 Progressive bulbar palsy
    • 335.23 Pseudobulbar palsy
    • 335.24 Primary lateral sclerosis
    • 335.29 Other
  • 335.8 Other anterior horn cell diseases
  • 335.9 Anterior horn cell disease, unspecified
• 336 Other diseases of spinal cord
  • 336.0 Syringomyelia and syringobulbia
  • 336.1 Vascular myelopathies
    • Acute infarction of spinal cord (embolic) (nonembolic)
    • Arterial thrombosis of spinal cord
    • Edema of spinal cord
    • Hematomyelia
    • Subacute necrotic myelopathy
  • 336.2 Subacute combined degeneration of spinal cord in diseases classified elsewhere
    • Code first underlying disease, as:
      • pernicious anemia (281.0)
      • other vitamin B12 deficiency anemia (281.1)
      • vitamin B12 deficiency (266.2)
  • 336.3 Myelopathy in other diseases classified elsewhere
    • Code first underlying disease, as:
      • myelopathy in neoplastic disease (140.0-239.9)
    • Excludes: myelopathy in:
      • intervertebral disc disorder (722.70-722.73)
      • spondylosis (721.1, 721.41-721.42, 721.91)
  • 336.8 Other myelopathy
    • Myelopathy:
      • drug-induced
      • radiation-induced
    • Use additional E code to identify cause
  • 336.9 Unspecified disease of spinal cord
    • Cord compression NOS
    • Myelopathy NOS
    • Excludes: myelitis (323.0-323.9)
      • spinal (canal) stenosis (723.0, 724.00-724.09)
• 337 Disorders of the autonomic nervous system
  • Includes: disorders of peripheral autonomic, sympathetic, parasympathetic, or vegetative system
  • Excludes: familial dysautonomia [Riley-Day syndrome] (742.8)
  • 337.0 Idiopathic peripheral autonomic neuropathy
    • Carotid sinus syncope or syndrome
    • Cervical sympathetic dystrophy or paralysis
  • 337.1 Peripheral autonomic neuropathy in disorders classified elsewhere
    • Code first underlying disease, as:
      • amyloidosis (277.3)
      • diabetes (250.6)
  • 337.2 Reflex sympathetic dystrophy
    • 337.20 Reflex sympathetic dystrophy, unspecified
    • 337.21 Reflex sympathetic dystrophy of the upper limb
    • 337.22 Reflex sympathetic dystrophy of the lower limb
    • 337.29 Reflex sympathetic dystrophy of other specified site
  • 337.9 Unspecified disorder of autonomic nervous system