Degenerative disease of the central nervous system	Hereditary disorder of nervous system
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Hereditary degenerative disease of central nervous system (disorder)
	Hereditary degenerative disease of central nervous system
	hereditaire degeneratieve aandoening van centraal zenuwstelsel
	erfelijke degeneratieve aandoening van CZS

Id	106018006
Status	Defined

Associated morphology	Degenerative abnormality
Finding site	Structure of central nervous system

ICD-10 complex map reference set

Target	G31.9
Rule	TRUE
Advice	ALWAYS G31.9
Correlation	SNOMED CT source code to target map code correlation not specified

4H leukodystrophy3

Adult onset autosomal dominant leukodystrophy

Aicardi Goutieres syndrome type 1

Aicardi Goutieres syndrome type 2

Aicardi Goutieres syndrome type 3

Aicardi Goutieres syndrome type 4

Aicardi Goutieres syndrome type 5

Alexander's disease

Amyotrophic lateral sclerosis type 4

Arrested hydrocephalus

Atypical pantothenate kinase associated neurodegeneration

Auditory neuropathy, optic atrophy syndrome

Autosomal dominant Alzheimer disease due to mutation of amyloid precursor protein

Autosomal dominant Alzheimer disease due to mutation of presenilin 1

Autosomal dominant Alzheimer disease due to mutation of presenilin 2

Autosomal dominant late onset basal ganglia degeneration

Autosomal dominant late onset Parkinson disease

Autosomal dominant striatal neurodegeneration

Autosomal recessive cerebral atrophy

Autosomal recessive familial Parkinson disease

Autosomal recessive isolated optic atrophy

Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome

Behavioral variant of frontotemporal dementia

Brain calcification Rajab type

CAMOS syndrome

Cerebroretinal microangiopathy with calcifications and cysts

Childhood-onset basal ganglia degeneration syndrome

Childhood-onset motor and cognitive regression syndrome with extrapyramidal movement disorder

CHMP2B-related frontotemporal dementia

Classical pantothenate kinase associated neurodegeneration

Combined immunodeficiency with faciooculoskeletal anomalies syndrome

Combined oxidative phosphorylation defect type 27

Congenital microcephaly, severe encephalopathy, progressive cerebral atrophy syndrome

Craniosynostosis and intracranial calcification syndrome

Dermatoleukodystrophy

Diabetes mellitus AND insipidus with optic atrophy AND deafness

Early onset parkinsonism and intellectual disability syndrome

Early-onset calcifying leukoencephalopathy, skeletal dysplasia

Encephalopathy, intracerebral calcification, retinal degeneration syndrome

Facial onset sensory and motor neuronopathy syndrome

Familial Alzheimer-like prion disease

Familial Creutzfeldt-Jakob

Familial infantile bilateral striatal necrosis

Fatal familial insomnia

Fatal post-viral neurodegenerative disorder

Ferro-cerebro-cutaneous syndrome

Galactosylceramide beta-galactosidase deficiency2

Gemignani syndrome

Gerstmann-Straussler-Scheinker syndrome

GRN-related frontotemporal dementia

Hereditary cerebellar degeneration12

Hereditary oculoleptomeningeal amyloid angiopathy

Hereditary optic atrophy17

Hereditary spastic paraplegia6

HSMN IV2

Huntington disease-like 1

Huntington disease-like syndrome due to C9ORF72 expansions

Huntington's chorea4

Hypomyelination with brain stem and spinal cord involvement and leg spasticity

Hypotonia, speech impairment, severe cognitive delay syndrome

Infantile choroidocerebral calcification syndrome

Infantile inflammatory bowel disease with neurological involvement

ITM2B-related amyloidosis2

Keratosis follicularis, dwarfism, cerebral atrophy syndrome

LAMA5-related multisystemic syndrome

Leigh's disease4

Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation

Leukoencephalopathy with calcifications and cysts

Leukoencephalopathy with metaphyseal chondrodysplasia syndrome

Metachromatic leukodystrophy, adult type

Microphthalmia with brain atrophy syndrome

Microphthalmia, retinitis pigmentosa, foveoschisis, optic disc drusen syndrome

Multiple mitochondrial dysfunctions syndrome type 4

Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome

Muscle eye brain disease with bilateral multicystic leukodystrophy

Neurodegeneration due to 3-hydroxyisobutyryl coenzyme A hydrolase deficiency

Non-progressive predominantly posterior cavitating leukodystrophy with peripheral neuropathy

Ocular anomalies, axonal neuropathy, developmental delay syndrome

Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome

Pelizaeus Merzbacher like disease4

Pelizaeus-Merzbacher disease8

Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease

Prieto Badia Mulas syndrome

Prion protein systemic amyloidosis

Progressive cerebellar ataxia with hypogonadism

Progressive cone-rod dystrophy

Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy syndrome

Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy-like syndrome

Progressive myoclonic epilepsy type 3

Progressive sclerosing poliodystrophy

Progressive sensory ataxia of Charolais

Psychomotor regression, oculomotor apraxia, movement disorder, nephropathy syndrome

Rambaud Gallian syndrome

Recurrent metabolic encephalomyopathic crises, rhabdomyolysis, cardiac arrhythmia, intellectual disability syndrome

RNA polymerase III-related leukodystrophy

Severe X-linked intellectual disability Gustavson type

Severe X-linked mitochondrial encephalomyopathy

Spastic paraparesis and deafness

Spondylometaphyseal dysplasia with cone-rod dystrophy syndrome

Vanishing white matter disease1

Wilson's disease2