Huntingtonova nemoc [Huntington Disease]

tematický
321
Termíny

Huntingtonova chorea
Huntingtonova chorea - pozdní nástup
Huntingtonova chorea akineticko-rigidní
Huntingtonova chorea u mladistvých
Huntingtonova choroba
Huntingtonova nemoc - akinetický strnulý typ
Huntingtonova nemoc - pozdní nástup
Huntingtonova nemoc s pozdním nástupem
Huntingtonova nemoc u mladistvých
juvenilní Huntingtonova nemoc

 

Akinetic-Rigid Variant of Huntington Disease
Chorea, Chronic Progressive Hereditary (Huntington)
Chronic Progressive Hereditary Chorea (Huntington)
Huntington Chorea
Huntington Chronic Progressive Hereditary Chorea
Huntington Disease, Akinetic-Rigid Variant
Huntington Disease, Juvenile
Huntington Disease, Juvenile-Onset
Huntington Disease, Late Onset
Huntington's Chorea
Huntington's Disease
Juvenile Huntington Disease
Juvenile-Onset Huntington Disease
Late-Onset Huntington Disease
Progressive Chorea, Chronic Hereditary (Huntington)
Progressive Chorea, Hereditary, Chronic (Huntington)

Perzistentní odkaz   https://www.medvik.cz/link/D006816
Definice

Autozomálně dominantně dědičné nevyléčitelné onemocnění mozku, projevující se ve středním věku poruchami hybnosti (chorea) a demencí. Počáteční projevy zahrnují nekoordinovanou motoriku, paranoiu, snížené sebeovládání, deprese, halucinace, bludy. Na závěr přichází rozpad původní osobnosti, nesoběstačnost, demence a smrt.

A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

DUI
D006816 MeSH Prohlížeč
CUI
M0010660
Historická pozn.
2000(1963)
Veřejná pozn.
2000; see HUNTINGTON'S DISEASE 1993-1999; for HUNTINGTON DISEASE see HUNTINGTON'S DISEASE 1993-1999, see HUNTINGTON CHOREA 1977-1992, see CHOREA, HEREDITARY 1963-1976

C Nemoci
C10.228.140 nemoci mozku 1 177
C10.228.140.079 nemoci bazálních ganglií 100
C10.228.140.079.294 chorea gravidarum 2
C10.228.140.079.357 dystonia musculorum deformans 3
C10.228.140.079.493 hepatolentikulární degenerace 243
C10.228.140.079.545 Huntingtonova nemoc 321
C10.228.140.079.590 Meigeho syndrom 9
C10.228.140.079.612 multisystémová atrofie 56
C10.228.140.079.737 maligní neuroleptický syndrom 46
C10.228.140.079.800 Hallervordenův-Spatzův syndrom 17
C10.228.140.079.862 parkinsonské poruchy 301
C10.228.140.079.898 Tourettův syndrom 86
C10.228.140.380 demence 2 191
C10.228.140.380.070 AIDS dementia complex 26
C10.228.140.380.100 Alzheimerova nemoc 2 626
C10.228.140.380.132 primární progresivní afázie 18
C10.228.140.380.165 Creutzfeldtova-Jakobova nemoc 245
C10.228.140.380.230 vaskulární demence 194
C10.228.140.380.278 Huntingtonova nemoc 321
C10.228.140.380.326 Klüverův-Bucyho syndrom 1
C10.228.140.380.422 demence s Lewyho tělísky 115
C10.228.140.380.711 smíšená demence
C10.228.662 pohybové poruchy 914
C10.228.662.262 dyskineze 88
C10.228.662.262.249 chorea 84
C10.228.662.262.249.500 chorea gravidarum 2
C10.228.662.262.249.750 Huntingtonova nemoc 321
C10.228.662.262.249.937 choreoakantocytóza 5
C10.574.500.024 Alexanderova nemoc 6
C10.574.500.300 syndrom Canavanové 9
C10.574.500.362 Cockayneův syndrom 4
C10.574.500.497 Huntingtonova nemoc 321
C10.574.500.529 Laforova nemoc 1
C10.574.500.545 myotonia congenita 17
C10.574.500.547 myotonická dystrofie 94
C10.574.500.549 neurofibromatózy 28
C10.574.500.850 Tourettův syndrom 86
C10.574.500.865 tuberózní skleróza 141
C16.320.400.024 Alexanderova nemoc 6
C16.320.400.150 syndrom Canavanové 9
C16.320.400.200 Cockayneův syndrom 4
C16.320.400.430 Huntingtonova nemoc 321
C16.320.400.480 Laforova nemoc 1
C16.320.400.540 myotonia congenita 17
C16.320.400.542 myotonická dystrofie 94
C16.320.400.550 choreoakantocytóza 5
C16.320.400.560 neurofibromatózy 28
C16.320.400.820 Tourettův syndrom 86
C16.320.400.880 tuberózní skleróza 141

F Psychiatrie a psychologie
F03.615.250 kognitivní poruchy 1 320
F03.615.250.100 poruchy sluchové percepce 73
F03.615.250.400 Huntingtonova nemoc 321
F03.615.250.700 kognitivní dysfunkce 460
F03.615.400 demence 2 191
F03.615.400.050 AIDS dementia complex 26
F03.615.400.100 Alzheimerova nemoc 2 626
F03.615.400.350 vaskulární demence 194
F03.615.400.390 Huntingtonova nemoc 321
F03.615.400.431 Klüverův-Bucyho syndrom 1
F03.615.400.512 demence s Lewyho tělísky 115
F03.615.400.756 smíšená demence