G10 Huntington disease

Incl.:

Huntington chorea

G11 Hereditary ataxia

Excl.:

cerebral palsy ( G80.-)

hereditary and idiopathic neuropathy ( G60.-)

metabolic disorders ( E70-E90)

G11.0 Congenital nonprogressive ataxia

G11.1 Early-onset cerebellar ataxia

Note:

Onset usually before the age of 20

Incl.:

Early-onset cerebellar ataxia with:

• essential tremor
• myoclonus [Hunt ataxia]
• retained tendon reflexes

Friedreich ataxia (autosomal recessive)

X-linked recessive spinocerebellar ataxia

G11.2 Late-onset cerebellar ataxia

Note:

Onset usually after the age of 20

G11.3 Cerebellar ataxia with defective DNA repair

Incl.:

Ataxia telangiectasia [Louis-Bar]

Excl.:

Cockayne syndrome ( Q87.1)

xeroderma pigmentosum ( Q82.1)

G11.4 Hereditary spastic paraplegia

G11.8 Other hereditary ataxias

G11.9 Hereditary ataxia, unspecified

Incl.:

Hereditary cerebellar:

• ataxia NOS
• degeneration
• disease
• syndrome

G12 Spinal muscular atrophy and related syndromes

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

G12.1 Other inherited spinal muscular atrophy

Incl.:

Progressive bulbar palsy of childhood [Fazio-Londe]

Spinal muscular atrophy:

• adult form
• childhood form, type II
• distal
• juvenile form, type III [Kugelberg-Welander]
• scapuloperoneal form

G12.2 Motor neuron disease

Incl.:

Familial motor neuron disease

Lateral sclerosis:

• amyotrophic
• primary

Progressive:

• bulbar palsy
• spinal muscular atrophy

G12.8 Other spinal muscular atrophies and related syndromes

G12.9 Spinal muscular atrophy, unspecified

G13* Systemic atrophies primarily affecting central nervous system in diseases classified elsewhere

G13.0* Paraneoplastic neuromyopathy and neuropathy

Incl.:

Carcinomatous neuromyopathy ( C00-C97†)

Sensorial paraneoplastic neuropathy [Denny Brown] ( C00-D48†)

G13.1* Other systemic atrophy primarily affecting central nervous system in neoplastic disease

Incl.:

Paraneoplastic limbic encephalopathy ( C00-D48†)

G13.2* Systemic atrophy primarily affecting central nervous system in myxoedema (E00.1†, E03.-†)

G13.8* Systemic atrophy primarily affecting central nervous system in other diseases classified elsewhere

G14 Postpolio syndrome

Incl.:

Postpolio myelitic syndrome

Excl.:

sequelae of poliomyelitis ( B91)