Hereditary And Degenerative Diseases Of The Central Nervous System ICD 9 Codes (330-337)

HEREDITARY AND DEGENERATIVE DISEASES OF THE CENTRAL NERVOUS SYSTEM (ICD 9 CODES: 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 intellectual disabilities

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

Use additional code, where applicable, to identify dementia:

with behavioral disturbance (294.11)

without behavioral disturbance (294.10)

331.0    Alzheimer's disease

331.1    Frontotemporal dementia

331.11  Pick's disease

331.19  Other frontotemporal dementia

Frontal dementia

331.2    Senile degeneration of brain

Excludes: senility NOS (797)

331.3    Communicating hydrocephalus

Secondary normal pressure hydrocephalus

Excludes: congenital hydrocephalus (742.3)

idiopathic normal pressure hydrocephalus (331.5)

normal pressure hydrocephalus (331.5)

spina bifida with hydrocephalus (741.0)

331.4    Obstructive hydrocephalus

Acquired hydrocephalus NOS

Excludes: congenital hydrocephalus (742.3)

idiopathic normal pressure hydrocephalus (331.5)

normal pressure hydrocephalus (331.5)

spina bifida with hydrocephalus (741.0)

331.5    Idiopathic normal pressure hydrocephalus (INPH)

Normal pressure hydrocephalus NOS

Excludes: congenital hydrocephalus (742.3)

secondary normal pressure hydrocephalus (331.3)

spina bifida with hydrocephalus (741.0)

331.6    Corticobasal degeneration

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 (046.11-046.19)

progressive multifocal leukoencephalopathy (046.3)

subacute spongiform encephalopathy (046.1)

331.8    Other cerebral degeneration

331.81  Reye's syndrome

331.82  Dementia with Lewy bodies

Dementia with Parkinsonism

Lewy body dementia

Lewy body disease

331.83  Mild cognitive impairment, so stated

Excludes: altered mental status (780.97)

cerebral degeneration (331.0-331.9)

change in mental status (780.97)

cognitive deficits following (late effects of) cerebral hemorrhage or infarction (438.0)

cognitive impairment due to intracranial or head injury (850-854, 959.01)

cognitive impairment due to late effect of intracranial injury (907.0)

cognitive impairment due to skull fracture (800-801, 803-804)

dementia (290.0-290.43, 294.20-294.21)

mild memory disturbance (310.89)

neurologic neglect syndrome (781.8)

personality change, nonpsychotic (310.1)

331.89  Other

Cerebral ataxia

331.9    Cerebral degeneration, unspecified

332       Parkinson's disease

Excludes:    dementia with Parkinsonism (331.82)

332.0    Paralysis agitans

Parkinsonism or Parkinson's disease:

NOS

idiopathic

primary

332.1    Secondary Parkinsonism

Neuroleptic-induced 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)

sleep related movement disorders (327.51-327.59)

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

Shy-Drager syndrome

333.1    Essential and other specified forms of tremor

Benign essential tremor

Familial tremor

Medication-induced postural tremor

Use additional E code to identify drug, if drug-induced

Excludes: tremor NOS (781.0)

333.2    Myoclonus

Familial essential myoclonus

Palatal myoclonus

Use additional E code to identify drug, if drug-induced

Excludes: Progressive myoclonic epilepsy (345.1)

Unverricht-Lundborg disease (345.1)

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    Genetic torsion dystonia

Dystonia:

deformans progressiva

musculorum deformans

(Schwalbe-) Ziehen-Oppenheim disease

333.7    Acquired torsion dystonia

333.71  Athetoid cerebral palsy

Double athetosis (syndrome)

Vogt's disease

Excludes: infantile cerebral palsy (343.0-343.9)

333.72  Acute dystonia due to drugs

Acute dystonic reaction due to drugs

Neuroleptic-induced acute dystonia

Use additional E code to identify drug

Excludes: blepharospasm due to drugs (333.85)

orofacial dyskinesia due to drugs (333.85)

secondary Parkinsonism (332.1)

subacute dyskinesia due to drugs (333.85)

tardive dyskinesia (333.85)

333.79  Other acquired torsion dystonia

333.8    Fragments of torsion dystonia

Use additional E code to identify drug, if drug-induced

333.81  Blepharospasm

Excludes: blepharospasm due to drugs (333.85)

333.82  Orofacial dyskinesia

Excludes: orofacial dyskinesia due to drugs (333.85)

333.83  Spasmodic torticollis

Excludes: torticollis:

NOS (723.5)

hysterical (300.11)

psychogenic (306.0)

333.84  Organic writers' cramp

Excludes: psychogenic (300.89)

333.85  Subacute dyskinesia due to drugs

Blepharospasm due to drugs

Orofacial dyskinesia due to drugs

Tardive dyskinesia

Use additional E code to identify drug

Excludes: acute dystonia due to drugs (333.72)

acute dystonic reaction due to drugs (333.72)

secondary Parkinsonism (332.1)

333.89  Other

333.9    Other and unspecified extrapyramidal diseases and abnormal movement disorders

333.90  Unspecified extrapyramidal disease and abnormal movement disorder

Medication-induced movement disorders NOS

Use additional E code to identify drug, if drug-induced

333.91  Stiff-man syndrome

333.92  Neuroleptic malignant syndrome

Use additional E to identify drug

Excludes: neuroleptic induced Parkinsonism (332.1)

333.93  Benign shuddering attacks

333.94  Restless legs syndrome (RLS)

333.99  Other

Neuroleptic-induced acute akathisia

Use additional E code to identify drug, if drug-induced

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 in diseases 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 (323.02, 323.1, 323.2, 323.42, 323.52, 323.63, 323.72, 323.82, 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

337.00  Idiopathic peripheral autonomic neuropathy, unspecified

337.01  Carotid sinus syndrome

Carotid sinus syncope

337.09  Other idiopathic peripheral autonomic neuropathy

Cervical sympathetic dystrophy or paralysis

337.1    Peripheral autonomic neuropathy in disorders classified elsewhere

Code first underlying disease, as:

amyloidosis (277.30-277.39)

diabetes (249.6, 250.6)

337.2    Reflex sympathetic dystrophy

337.20  Reflex sympathetic dystrophy, unspecified

Complex regional pain syndrome type I, unspecified

337.21  Reflex sympathetic dystrophy of the upper limb

Complex regional pain syndrome type I of the upper limb

337.22  Reflex sympathetic dystrophy of the lower limb

Complex regional pain syndrome type I of the lower limb

337.29  Reflex sympathetic dystrophy of other specified site

Complex regional pain syndrome type I of other specified site

337.3    Autonomic dysreflexia

Use additional code to identify the cause, such as:

fecal impaction (560.32)

pressure ulcer (707.00-707.09)

urinary tract infection (599.0)

337.9    Unspecified disorder of autonomic nervous system