Koj puas paub Juvenile Parkinson's Disease?

Hu rau: Audrey Huaudrey.hu@wecistanche.com

Thaum nws los txog rau tus kab mob Parkinson, ntau tus neeg xav tias nws yog kab mob uas tsuas yog cov neeg laus tuaj yeem kis tau. Txawm li cas los xij, hauv kev faib cov kab mob Parkinson, muaj ib pab pawg neeg uas pib thaum hluas, uas peb hu ua "juvenile Parkinson's disease". Cov "First Batch of Rare Diseases List" ua ke tsim los ntawm Lub Xeev Lub Chaw Tswj Xyuas Khoom Noj thiab Tshuaj, Lub Xeev Kev Tswj Xyuas Tshuaj Khomob Suav thiab lwm tsib lub chaw haujlwm thiab cov thawj coj tau raug tso tawm, ntawm cov kab mob Parkinson (cov hluas-pib, pib ntxov) yog suav nrog.

Juvenile Parkinson's disease yog hais txog tus kab mob Parkinson uas muaj hnub nyoog qis dua lossis sib npaug li 40 xyoo (qee cov ntaub ntawv teev cov kev txwv siab tshaj li 50 xyoo), tseem hu ua Parkinson's disease EOPD thaum ntxov. Juvenile Parkinson's disease (JP) raug kuaj pom ua ntej hnub nyoog 21 xyoos, thiab cov menyuam yaus Parkinson's disease (YOPD) raug kuaj pom tom qab hnub nyoog 21 xyoos.

Kab mob sib kis

Juvenile Parkinson tus kab mob tsis tshua muaj, thiab nws qhov xwm txheej tshwm sim rau 5 feem pua ​​​​rau 10 feem pua ​​​​ntawm cov neeg mob Parkinson, kwv yees li 5 feem pua ​​​​ntawm cov teb chaws Europe thiab Asmeskas, thiab txog 10 feem pua ​​​​hauv Nyiv. Zoo ib yam li tus kab mob Parkinson's lig, qhov tshwm sim nce nrog hnub nyoog.

Juvenile Parkinson tus kab mob muaj qhov pom tseeb ntawm caj ces thiab kev sib sau ntawm tsev neeg, thiab nws cov keeb kwm zoo hauv tsev neeg muaj ntau dua li cov neeg mob Parkinson lig, qhia tias cov caj ces muaj lub luag haujlwm tseem ceeb.

Kev sib raug zoo ntawm ib puag ncig yam tseem ceeb thiab pib ntxov Parkinson tus kab mob tseem muaj teeb meem. Qee qhov kev tshawb fawb tau pom tias cov neeg mob uas muaj tus kab mob Parkinson thaum ntxov muaj feem ntau ntawm keeb kwm ntawm lub hlwb raug mob thiab feem ntau ntawm cov neeg mob uas muaj kev tawm dag zog ntau dua li cov neeg mob uas muaj tus kab mob Parkinson lig.

TsavCov tsos mob

Cov tsos mob thawj zaug ntawm tus menyuam yaus tus kab mob Parkinson tsis yog ib yam, thiab kev nruj thiab bradykinesia muaj ntau dua. Qee cov neeg mob uas muaj tus kab mob Parkinson thaum ntxov muaj ib daim ntawv tshwj xeeb, tshwj xeeb tshaj yog menyuam yaus-pib parkinsonism, uas tuaj yeem txhim kho nyob rau hauv daim ntawv ntawm dystonia, tshwj xeeb tshaj yog ko taw dystonia.

Cov tsos mob ntawm tus menyuam yaus-pib tus kab mob Parkinson thiab lig-pib Parkinson tus kab mob yog qhov pib zoo sib xws thiab feem ntau tshwm sim los ntawm triad ntawm tshee, rigidity, thiab bradykinesia. Tus kab mob no maj mam thiab teb zoo rau cov tshuaj, tab sis cov teeb meem ntawm lub cev muaj zog, xws li dyskinesia thiab "hloov" tshwm sim, tuaj yeem tshwm sim thaum ntxov.

Cov tsos mob tsis yog lub cev muaj zog

Kev paub txog kev khaws cia ntawm tus menyuam yaus tus kab mob Parkinson yog qhov tsis zoo, qhov kev paub tsis meej tshwm sim tom qab, thiab kev xav thiab kev coj tus cwj pwm yog qhov pom tseeb dua. Cov kev xav tsis zoo no feem ntau tshwm sim xws li kev nyuaj siab, kev ntxhov siab, kev chim siab, thiab lwm yam, uas muaj kev cuam tshuam ntau dua rau tus neeg mob txoj haujlwm, kev sib yuav, kev sib txuas lus ntawm tus kheej, thiab kev lag luam. Kev coj tus cwj pwm tsis zoo feem ntau suav nrog kev siv tshuaj ntau ntxiv, tsis tsuas yog txwv rau cov tshuaj tiv thaiv Parkinsonian, thiab cov nyhuv tshem tawm yog pom tseeb; gluttony, twv txiaj, compulsive kav khw, hypersexuality thiab lwm yam impulse tswj tsis meej; stereotyped ua thiab lwm yam.

Hais txog kev pw tsaug zog, cov kev tshawb fawb tau pom tias cov neeg mob uas muaj hnub nyoog qis dua Parkinson tus kab mob tsis tshua muaj kev cuam tshuam los ntawm kev pw tsaug zog, suav nrog kev npau suav ntau thiab kev tsaug zog nruab hnub ntau dua li cov neeg mob uas muaj tus kab mob Parkinson lig.

Ntau cov kev tshawb fawb sib txawv ntawm cov menyuam yaus-pib pib thiab lig-pib Parkinson tus kab mob nyob rau hauv cov nqe lus ntawm autonomic dysfunction (feem ntau tshwm sim raws li cem quav, nquag ceev, hypotension, nce hws, thiab lwm yam) tau mus txog qhov opposite xaus. Qee cov kev tshawb fawb qhia tias cov menyuam yaus salivation, cem quav, thiab lwm yam yog ntau dua, thaum qee qhov xav tias kev koom tes ntawm cov hlab ntsha tsis muaj zog tsawg dua.

Gene

Cov noob hloov pauv ntawm cov menyuam yaus-pib Parkinson tus kab mob muaj ntau dua thaum pib lig, tshwj xeeb tshaj yog nyob rau hauv cov menyuam yaus-pib Parkinson's syndrome. Muaj qhov sib txawv ntawm kev ua haujlwm ntawm cov noob caj noob ces sib txawv.

Pathology

Cov kab mob pathological thiab kev ua haujlwm zoo ntawm cov menyuam yaus-pib Parkinson tus kab mob zoo ib yam li cov kab mob lig pib, lees paub tias lawv yog tib yam kab mob. Tab sis nyob rau hauv cov me nyuam yaus Parkinson's syndrome, cov Lewy lub cev nyob rau hauv Parkinson tus kab mob ploj mus.

Juvenile Parkinson's Syndrome

Juvenile Parkinson's syndrome yog pathologically txawv ntawm classic Parkinson's, thiab classic Lewy lub cev deposition tsis pom.

Juvenile Parkinson's syndrome familial aggregation tendency yog ntau dua, thiab kev sib raug zoo ntawm caj ces yog qhov tseeb dua, thiab muaj ntau cov noob hloov pauv, feem ntau yog cov noob caj noob ces, uas feem ntau yog autosomal recessive. Dystonia, tshwj xeeb tshaj yog plantar dystonia, muaj ntau dua. Cov lus teb rau dopamine tau hais ntau dua. Kev hloov pauv ntawm cov tsos mob tuaj yeem tshwm sim, nrog cov tsos mob hnyav thaum nruab hnub thiab cov tsos mob me me thaum hmo ntuj.

Rau cov menyuam yaus Parkinson's syndrome, nws yuav tsum tau ua kom sib txawv ntawm lwm yam kab mob, xws li dopamine-responsive dystonia, hepatolenticular degeneration, ntxov ntxov chorea, spinocerebellar ataxia, theem nrab ntawm cov tshuaj los yog kab mob, thiab lwm yam Parkinson's syndrome.

Kev kho mob

Nyob rau hauv cov nqe lus ntawm kev kho mob, tus neeg mob txoj kev vam meej yog qeeb, cov tshuaj siv tau zoo, thiab lub cev muaj zog muaj teeb meem yuav tshwm sim. Yog li, nws raug nquahu kom siv cov tshuaj uas tsis yog dopaminergic rau kev kho mob ua ntej, xws li monoamine oxidase inhibitors thiab amantadine. Yog hais tias qhov tshee yog qhov tshwm sim tseem ceeb, antans tuaj yeem siv tau, uas yuav tsum tau ua tib zoo saib xyuas kev paub tsis meej. Yog tias cov tsos mob tsis tswj tau, tuaj yeem siv dopamine agonists (DR-A) . Yog tias tseem tsis tau tswj hwm, siv Medopa.

Cistanche

Cov neeg mob Parkinson yuav tsum muaj kev nkag siab zoo txog tus kab mob, ua tiav kev kuaj pom ntxov, kuaj pom ntxov, thiab tau txais kev kho mob raws li kev cai, tsis yog tsuas yog saib xyuas tus kab mob xwb, tab sis kuj txo cov kev puas siab puas ntsws, tswj kev tshawb fawb, thiab ua tiav lub neej zoo. Cov neeg mob Parkinson tuaj yeem kawm paub ntxiv txog thiab noj tshuaj tiv thaiv Parkinson cov zaub mov thiab tshuaj ntsuab xws li cistanche.Cistancheyog ib hom tshuaj ntsuab uas loj hlob hauv cov suab puam. Nws muaj ib tug loj npaum li cas ntawmCistanche glycosidesuas muaj kev tiv thaiv zoo heev ntawm cov hlab ntsha cranial. Kev tshawb fawb tshuaj niaj hnub tau lees paub tias phenylethanoid glycoside hauvcistanchetuaj yeem txo apoptosis, thiabechinacoside cov tshuajhauvcistanchetuaj yeem txo cov qib biliverdin thiab reductase B, thiab ua kom cov ntsiab lus ntawm dopamine thiab homovanillic acid zoo. Yog li ntawd,Cistanchemuaj txiaj ntsig zoo rau tus kab mob Parkinson.