Systemic atrophies primarily affecting the central nervous system (G10-G14)

G10Huntington's disease
› Huntington's chorea
› Huntington's dementia
Use Additional Code: code, if applicable, to identify:
› dementia with anxiety (F02.84, F02.A4, F02.B4, F02.C4)
› dementia with behavioral disturbance (F02.81-, F02.A1-, F02.B1-, F02.C1-)
› dementia with mood disturbance (F02.83, F02.A3, F02.B3, F02.C3)
› dementia with psychotic disturbance (F02.82, F02.A2, F02.B2, F02.C2)
› dementia without behavioral disturbance (F02.80, F02.A0, F02.B0, F02.C0)
› mild neurocognitive disorder due to known physiological condition (F06.7-)

G11Hereditary ataxia
Excludes 2: cerebral palsy (G80.-)
› hereditary and idiopathic neuropathy (G60.-)
› metabolic disorders (E70-E88)

G11.0Congenital nonprogressive ataxia

G11.1Early-onset cerebellar ataxia

G11.10Early-onset cerebellar ataxia, unspecified

G11.11Friedreich ataxia
› Autosomal recessive Friedreich ataxia
› Friedreich ataxia with retained reflexes

G11.19Other early-onset cerebellar ataxia
› Early-onset cerebellar ataxia with essential tremor
› Early-onset cerebellar ataxia with myoclonus [Hunt's ataxia]
› Early-onset cerebellar ataxia with retained tendon reflexes
› X-linked recessive spinocerebellar ataxia

G11.2Late-onset cerebellar ataxia

G11.3Cerebellar ataxia with defective DNA repair
› Ataxia telangiectasia [Louis-Bar]
Excludes 2: Cockayne's syndrome (Q87.19)
› other disorders of purine and pyrimidine metabolism (E79.-)
› xeroderma pigmentosum (Q82.1)

G11.4Hereditary spastic paraplegia

G11.5Hypomyelination - hypogonadotropic hypogonadism - hypodontia
› 4H syndrome
› Pol III-related leukodystrophy

G11.6Leukodystrophy with vanishing white matter disease

G11.8Other hereditary ataxias

G11.9Hereditary ataxia, unspecified
› Hereditary cerebellar ataxia NOS
› Hereditary cerebellar degeneration
› Hereditary cerebellar disease
› Hereditary cerebellar syndrome

G12Spinal muscular atrophy and related syndromes

G12.0Infantile spinal muscular atrophy, type I [Werdnig-Hoffman]

G12.1Other inherited spinal muscular atrophy
› Adult form spinal muscular atrophy
› Childhood form, type II spinal muscular atrophy
› Distal spinal muscular atrophy
› Juvenile form, type III spinal muscular atrophy [Kugelberg-Welander]
› Progressive bulbar palsy of childhood [Fazio-Londe]
› Scapuloperoneal form spinal muscular atrophy

G12.2Motor neuron disease

G12.20Motor neuron disease, unspecified

G12.21Amyotrophic lateral sclerosis

G12.22Progressive bulbar palsy

G12.23Primary lateral sclerosis

G12.24Familial motor neuron disease

G12.25Progressive spinal muscle atrophy

G12.29Other motor neuron disease

G12.8Other spinal muscular atrophies and related syndromes

G12.9Spinal muscular atrophy, unspecified

G13Systemic atrophies primarily affecting central nervous system in diseases classified elsewhere

G13.0Paraneoplastic neuromyopathy and neuropathy
› Carcinomatous neuromyopathy
› Sensorial paraneoplastic neuropathy [Denny Brown]
Code First: underlying neoplasm (C00-D49)

G13.1Other systemic atrophy primarily affecting central nervous system in neoplastic disease
› Paraneoplastic limbic encephalopathy
Code First: underlying neoplasm (C00-D49)

G13.2Systemic atrophy primarily affecting the central nervous system in myxedema
Code First: underlying disease, such as:
› hypothyroidism (E03.-)
› myxedematous congenital iodine deficiency (E00.1)

G13.8Systemic atrophy primarily affecting central nervous system in other diseases classified elsewhere
Code First: underlying disease

G14Postpolio syndrome
Includes: postpolio myelitic syndrome
Excludes 1: sequelae of poliomyelitis (B91)