Hereditary degenerative disease of central nervous system   106018006

SNOMED CT code


SNOMED code106018006
nameHereditary degenerative disease of central nervous system
statusactive
date introduced2002-01-31
fully specified name(s)Hereditary degenerative disease of central nervous system (disorder)
synonymsHereditary degenerative disease of central nervous system
attributes - group1
Associated morphologyDegenerative abnormality   107669003
Finding siteCentral nervous system structure   21483005
parents
  • Hereditary disorder of nervous system   363235000
  • Degenerative disease of the central nervous system   80690008
children
  • 4H leukodystrophy   1208933000
  • Adrenoleukodystrophy   65389002
  • Adult onset autosomal dominant leukodystrophy   448054001
  • Aicardi Goutieres syndrome   230312006
  • Alexander's disease   81854007
  • Alkaline ceramidase 3 deficiency   1237515001
  • Amyotrophic lateral sclerosis type 4   784341001
  • Arrested hydrocephalus   4113009
  • Atypical pantothenate kinase associated neurodegeneration   1186856001
  • Auditory neuropathy, optic atrophy syndrome   1222649004
  • Autosomal dominant Alzheimer disease due to mutation of amyloid precursor protein   1156789004
  • Autosomal dominant Alzheimer disease due to mutation of presenilin 1   1156800008
  • Autosomal dominant Alzheimer disease due to mutation of presenilin 2   1156798001
  • Autosomal dominant late onset basal ganglia degeneration   230313001
  • Autosomal dominant late onset Parkinson disease   716662004
  • Autosomal dominant striatal neurodegeneration   725392005
  • Autosomal recessive cerebral atrophy   776087007
  • Autosomal recessive familial Parkinson disease   1156822001
  • Autosomal recessive isolated optic atrophy   1197151003
  • Autosomal recessive spastic ataxia, optic atrophy, dysarthria syndrome   784347002
  • Behavioral variant of frontotemporal dementia   716994006
  • Brain calcification Rajab type   720576001
  • C11ORF73-related autosomal recessive hypomyelinating leukodystrophy   1172595004
  • CAMOS syndrome   726031001
  • Cerebroretinal microangiopathy with calcifications and cysts   711482008
  • Childhood-onset basal ganglia degeneration syndrome   1172584005
  • Childhood-onset motor and cognitive regression syndrome with extrapyramidal movement disorder   1167373005
  • CHMP2B-related frontotemporal dementia   702393003
  • Cholestanol storage disease   63246000
  • Classical pantothenate kinase associated neurodegeneration   1186861004
  • Combined immunodeficiency with faciooculoskeletal anomalies syndrome   770625006
  • Combined oxidative phosphorylation defect type 27   1172844009
  • Congenital microcephaly, severe encephalopathy, progressive cerebral atrophy syndrome   782757004
  • Craniosynostosis and intracranial calcification syndrome   720816004
  • Dermatoleukodystrophy   733044009
  • Early onset parkinsonism and intellectual disability syndrome   716107009
  • Early-onset calcifying leukoencephalopathy, skeletal dysplasia   1222661007
  • Encephalopathy, intracerebral calcification, retinal degeneration syndrome   733049004
  • Facial onset sensory and motor neuronopathy syndrome   723306004
  • Familial Alzheimer-like prion disease   721219005
  • Familial Creutzfeldt-Jakob   715807002
  • Familial infantile bilateral striatal necrosis   1208478005
  • Fatal familial insomnia   83157008
  • Fatal post-viral neurodegenerative disorder   774206008
  • Ferro-cerebro-cutaneous syndrome   774151000
  • Galactosylceramide beta-galactosidase deficiency   192782005
  • Gemignani syndrome   782690007
  • Gerstmann-Straussler-Scheinker syndrome   67155006
  • GRN-related frontotemporal dementia   702426001
  • Hereditary cerebellar degeneration   37650008
  • Hereditary oculoleptomeningeal amyloid angiopathy   43532007
  • Hereditary optic atrophy   26360005
  • Hereditary spastic paraplegia   39912006
  • Huntington disease-like 1   784371009
  • Huntington disease-like 2   721228006
  • Huntington disease-like syndrome due to C9ORF72 expansions   782743001
  • Huntington's chorea   58756001
  • Hypomyelination with brain stem and spinal cord involvement and leg spasticity   777999008
  • Hypotonia, speech impairment, severe cognitive delay syndrome   763722004
  • Infantile choroidocerebral calcification syndrome   724228005
  • Infantile inflammatory bowel disease with neurological involvement   1186721005
  • ITM2B-related amyloidosis   1187126002
  • Juvenile-onset diabetes mellitus, central and peripheral neurodegeneration syndrome   1255271005
  • Keratosis follicularis, dwarfism, cerebral atrophy syndrome   723830005
  • LAMA5-related multisystemic syndrome   1217370006
  • Leigh's disease   29570005
  • Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation   703537008
  • Leukoencephalopathy with calcifications and cysts   1186710001
  • Leukoencephalopathy with metaphyseal chondrodysplasia syndrome   719405005
  • Metachromatic leukodystrophy   396338004
  • Microphthalmia with brain atrophy syndrome   720010009
  • Microphthalmia, retinitis pigmentosa, foveoschisis, optic disc drusen syndrome   778021002
  • Multiple mitochondrial dysfunctions syndrome type 4   1208621008
  • Multiple mitochondrial dysfunctions syndrome type 5   1279890001
  • Multiple mitochondrial dysfunctions syndrome type 6   1279891002
  • Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome   733028000
  • Muscle eye brain disease with bilateral multicystic leukodystrophy   785298001
  • NAD(P)HX dehydratase deficiency   1251446004
  • NAD(P)HX epimerase deficiency   1251447008
  • Neurodegeneration due to 3-hydroxyisobutyryl coenzyme A hydrolase deficiency   722488009
  • NKX6-2-related autosomal recessive hypomyelinating leukodystrophy   1217379007
  • Non-progressive predominantly posterior cavitating leukodystrophy with peripheral neuropathy   1220598005
  • Ocular anomalies, axonal neuropathy, developmental delay syndrome   1172586007
  • Optic atrophy, ataxia, peripheral neuropathy, global developmental delay syndrome   1222655009
  • Pelizaeus Merzbacher like disease   717042001
  • Pelizaeus-Merzbacher disease   64855000
  • Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease   765325002
  • Phytanic acid storage disease   25362006
  • Prieto Badia Mulas syndrome   719140001
  • Prion protein systemic amyloidosis   733422008
  • PRKAR1B-related neurodegenerative dementia with intermediate filaments   774069007
  • Progressive cerebellar ataxia with hypogonadism   230240004
  • Progressive cone-rod dystrophy   80328002
  • Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy syndrome   442511009
  • Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy-like syndrome   770678005
  • Progressive myoclonic epilepsy type 3   783064000
  • Progressive sclerosing poliodystrophy   20415001
  • Progressive sensory ataxia of Charolais   40259002
  • Psychomotor regression, oculomotor apraxia, movement disorder, nephropathy syndrome   1187043002
  • Rambaud Gallian syndrome   724002003
  • RARS-related autosomal recessive hypomyelinating leukodystrophy   1220600004
  • Recurrent metabolic encephalomyopathic crises, rhabdomyolysis, cardiac arrhythmia, intellectual disability syndrome   1172698005
  • RNA polymerase III-related leukodystrophy   712637001
  • Severe X-linked intellectual disability Gustavson type   722213009
  • Severe X-linked mitochondrial encephalomyopathy   722212004
  • Short stature, optic nerve atrophy, Pelger-Huët anomaly syndrome   1237618009
  • Spastic paraparesis and deafness   715504003
  • Spondylometaphyseal dysplasia with cone-rod dystrophy syndrome   719205008
  • Spongy degeneration of central nervous system   80544005
  • USP18 deficiency   1251449006
  • Vanishing white matter disease   447351004
  • VPS11-related autosomal recessive hypomyelinating leukodystrophy   1187249005
  • Wilson's disease   88518009
  • Wolfram syndrome   70694009
hierarchies
a selection of possible paths
SNOMED CT Concept   138875005
  Clinical finding   404684003
    Disease   64572001
      Disorder of body system   362965005
        Disorder of nervous system   118940003
          Hereditary disorder of nervous system   363235000
            Hereditary degenerative disease of central nervous system   106018006

SNOMED CT Concept   138875005
  Clinical finding   404684003
    Disease   64572001
      Degenerative disorder   362975008
        Degenerative disease of the central nervous system   80690008
          Hereditary degenerative disease of central nervous system   106018006

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