Chirwere cheParkinson & Parkinsonism

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Chirwere cheParkinson

Chirwere cheParkinson (PD) chirwere chinonzi neurodegenerative chinoratidzwa zvakanyanya nekurasikirwa kwe dopamine neurons mu substantia nigra. Zviratidzo zvinowanzokura kune rumwe rutivi rwomuviri zvishoma nezvishoma mumakore asi kufambira mberi kunogona kusiyana kubva kune mumwe munhu kune mumwe nekuda kwekusiyana kwechirwere. Vanhu vane PD vanogona kusangana nekudengenyeka, kunyanya pakuzorora (kunotsanangurwa sepiritsi rinotenderera kudengenyeka mumaoko), bradykinesia, kusimba kwemakumbo, kufamba uye kuenzanisa matambudziko. Hwakawanda hunosvika mazana maviri ezviitiko pa200 vanhu, uye chiitiko chacho chingangoita makumi maviri neshanu pahuwandu hwevanhu zviuru zana, asi huwandu uhu hunogona kuratidza mutsauko kubva kune imwe nzvimbo yepasi kuenda kune imwe.

Kana maratidziro emota aonekwa, vanhu vane PD vakarasikirwa nepamusoro pe50% yenigral dopamine masero anoratidza kuti shanduko dzepathological dzinogona kutanga makumi emakore akawanda kusati kwaonekwa zviratidzo zvekiriniki. The premotor phase inoratidzirwa muzviitiko zvakawanda ne-non-motor zviratidzo zvakadai seREM-sleep behaviour disorder, kusava nehanya, kuchinja kwemafungiro, kuzvidya mwoyo, kuvimbiswa uye kurasikirwa kwemafuta.

Ona tsanangudzo yakazara yePD

Chikonzero chePD chingangove chakawanda, chine mipiro kubva kunhaka predisposition, chepfu yezvakatipoteredza, uye kuchembera. Mumakore achangopfuura zvakava pachena kuti kunewo genetic mupiro kune PD uye shanduko dzakawanda dzakaonekwa (GBA, LRRK2, PRKN, SNCA), kunyange zvazvo munzvimbo dzakawanda dzepasi pose nyaya shomanana inotsanangurwa ne genetics.

Kuongororwa kunoramba kuri kwekiriniki uye kunobva pane kuratidzwa kwemota. Brain MRI kana CT uye kufungidzira kwemamorekuru (kureva kuti dopamine transporter mu striatum) ye striatum inogona kuitwa kutsigira kuongororwa kwekiriniki. Makiriniki maficha ePD anosanganisira ese ari maviri mota zviratidzo (zvinotsanangurwa pamusoro), pamwe nenyaya dzisiri dzemota. Zviratidzo izvi zvisiri zvemotokari zvinosanganisira zviratidzo zveurouropsychiatric zvinosanganisira kuvhiringidzika kwemafungiro uye kuchinja kwekuziva; autonomic dysfunction, kurwadziwa uye nyaya dzekurara.

Levodopa yakaramba iri dombo repakona rekurapa kwePD kweanopfuura makore makumi mashanu. Zvisinei, mushure memakore mashomanana ekurapa uye kunyanya nekuda kwekufambira mberi kwechirwere, kubatsirwa kwe levodopa kunopfupisa uye zvinetso zvemotokari zvinoonekwa muvarwere vakawanda. Izvi zvakange zvaita kuti pave nemimwe mishonga yakawanda inosanganisira inhibitors yecatechol-O-methyltransferase (COMT), monoamine oxidase type B (MAO-B) inhibitors uye dopamine agonists. Enzyme blockers vanoita nekuwedzera levodopa kana dopamine hafu yeupenyu nepo dopamine agonists vachitevedzera chiito che dopamine pauropi dopamine receptors.

Nguva pfupi yadarika, marapirwo ekuvhiya uye infusion akavepo ekuvandudza manejimendi mune vanosarudza varwere vane mota zvinonetsa. Kuvhiya kunosanganisira kushandiswa kwekukurudzira kwehuropi hwakadzika hwe subthalamic nucleus uye globus pallidus internus. Kushandiswa kwemishonga infusions kunobva pakukwanisa kuunza nguva dzose kana levodopa kana apomorphine (a dopamine agonist ane high affinity kune dopamine receptors), kutevedzera zvakasikwa tonic receptor stimulation mu basal ganglia.

Yakanyorwa naMarcelo Merello, MD
Mutungamiriri, Dhipatimendi reNeuroscience
Musoro Movement Disorders Chikamu
Institute for Neurological Research Raul Carrea (FLENI)
Buenos Aires, Argentina

2019 Zvigadziriso zvakapihwa naAngelo Antonini, MD, PhD
Purofesa, Dhipatimendi reNeuroscience
Yunivhesiti yePadua, Italy

Parkinsonism

Iyo inotsanangura chimiro che parkinsonism ndeye bradykinesia, kana kunonoka nekuderera uye kuderedzwa kwekudzokorora mafambiro ("kuneta"). Zvisinganzwisisike "bradykinesia" zvakataurwa kuti zviitike mu "vanhu vakwegura" vanhu, asi izvi zvinogona kuratidza kunonoka kusiri kwakananga pane bradykinesia sezvinotsanangurwa pamusoro apa. Chirwere cheParkinson ndicho chinonyanya kukonzera neurodegenerative chikonzero che parkinsonism. Zvimwe zvikonzero zvinosanganisira multiple system atrophy, progressive supranuclear palsy uye corticobasal degeneration.

Ona tsanangudzo yakazara yeparkinsonism

Aya mamwe mamiriro euoodegenerative dzimwe nguva anounganidzwa pamwechete pasi peshoko rekuti "atypical parkinsonism" kana "parkinson-plus syndromes". Ivo havapindure pamwe chete ne dopaminergic kurapwa uye kazhinji vane fungidziro yakaipisisa kana ichienzaniswa neyakajairika chirwere cheParkinson. Zvinokonzeresa zvinokonzeresa parkinsonism zvinogona kunetsa kuongorora mumatanho ekutanga uye kuferefeta kwekuwedzera kunogona kuve kweukoshi hushoma mune ino chiitiko.

Parkinsonism inogonawo kuva chiratidzo, semugumisiro wezvakasiyana-siyana zvevascular, zvinodhaka, zvinotapukira, zvine chepfu, zvimiro uye zvimwe zvinozivikanwa zvikonzero zvechipiri. Pakati peizvi, parkinsonism inokonzerwa nezvinodhaka ndiyo inonyanya kuwanda uye inosanganisira vamiririri vanovhara post-synaptic dopamine D2 receptors vane hukama hwepamusoro (senge antipsychotic uye anti-emetic mishonga) uye sodium valproate. Vascular parkinsonism ("arteriosclerotic pseudoparkinsonism") inowanzova nekusimbisa kwemuviri wakaderera nekukanganiswa kwekufamba uye kunoenderana nekukanganisa kwekuziva.

Yakapiwa naDavid John Burn, MD, FRCP, Purofesa, Clinical Aging Reserach Unit, Campus for Aging and Vitality, Newcastle University, Newcastle Upon Tyne, UK.

Multiple System Atrophy (MSA)

Multiple system atrophy (MSA) isingawanzo uye inofambira mberi α-synucleinopathy yakabatana ne oligodendroglial inclusions uye neuronal kurasikirwa kunobata striatum, substantia nigra, pons, uye cerebellum pakati pedzimwe nzvimbo dzehuropi. Muchipatara, zviratidzo zvinogona kusanganisira zvakanyanya parkinsonism isingateereri kune levodopa, cerebellar ataxia, autonomic kana pyramidal dysfunction mukusiyana-siyana.

Ona tsanangudzo yakazara yeMSA

Zvinoenderana nehukuru hwe parkinsonian kana cerebellar zviratidzo varwere vanoiswa mu subtype MSA-P kana MSA-C, zvichiteerana. Kurebesa zera pachiratidzo chemotokari kutanga makore 56.2 ± 8.4 pasina mutsauko mukugovera bonde, uye kurarama kwepakati makore matanhatu kusvika gumi (makore 6). Isati yatanga chiratidzo chemotokari 10-9.8% yevarwere vanoona prodromal chikamu chinotora kubva kumwedzi yakati wandei kusvika kumakore uye chinoratidzwa nekutadza kwe autonomic kunokanganisa moyo, kufema, urogenital, gastrointestinal, uye sudomotor mabasa. Mukuwedzera, kukurumidza kwekufamba kwemaziso ekurara kwehutano hwehutano (RBD) inowanzoonekwa mu premotor stage ye α-synucleinopathies ine inopfuura hafu yevarwere vanotaura RBD vasati vatanga motokari uye iripo kusvika ku20% yevarwere vanoonekwa kuti vane MSA inogona kuitika. Parizvino, iyo etiology yeMSA ichiri kushaikwa, asi kupindirana kwakaoma kunosanganisira genetic predisposition uye zvakatipoteredza zvinokurudzirwa kutyaira kutanga kwechirwere uye kufambira mberi, sekuunganidzwa kwemhuri kunotevera autosomal inotonga kana recessive yenhaka maitiro yakashumwa mune akati wandei eEurope neJapan mhuri. Zvakangodaro, MSA inofungidzirwa kuti inoitika pano neapo.

Parizvino, nzira dzekurapa dzinotarisa pane chaiwo zviratidzo uye zviratidzo, uye dzinosanganisira mishonga yekusimudza BP kana kuderedza zviratidzo zveParkinsonism (yenguva pfupi yekupindura chiyero chemakore 1-3 mu38%), zvinodhaka zvekushaya simba, kuchengetedza dundira, physiotherapy uye kurapa kwekutaura.

Yakanyorwa naGregor Wenning, MD, PhD, Purofesa weClinical Neurbiology, Medical University yeInnsbruck, Austria (Chikunguru 2019)

Inofambira mberi Supranuclear Palsy (PSP)

Progressive Supranuclear Palsy (PSP) chirwere chepfungwa chinotanga kukura chevanhu vakuru chine cerebral four-repeat (4R-) tau pathology mumasero etsinga, oligodendrocytes uye astrocytes. Kukanganisika kwemasero etsinga muPSP kunonyanya kuitika muuropi nebasal ganglia uye zvishoma mu frontal ne temporal cortices uye cerebellum. Oligodendroglial coiled bodies dziripo zvakasiyana-siyana. Tau-positive tufted astrocytes inosimbisa kuongororwa. Kusiyana kweanatomical distribution ye tau pathology kunoita sekunosarudza zviratidzo zvePSP zvakasiyana-siyana.

Ona kukwikwidza PSP tsananguro

Chiratidzo chikuru chePSP chinodaidzwa kuti Richardson's syndrome, kureva musanganiswa wekusagadzikana kwepashure nekunonoka kwemasakedhi akatwasuka uye kuoma kwepanuclear vertical gaze palsy mukutanga kwekiriniki kosi. Chiratidzo chechipiri chinonyanyozivikanwa iPSP ine chirwere cheParkinsonism chakanyanya, kureva kuti anetic-rigid syndrome inoita kusashanda zvakanaka kwemota yenyukireya pane imwe nguva yechirwere. Zvimwe zviratidzo zvemakiriniki zvePSP zvinosanganisira kufambira mberi kwechando, kuratidzwa kwepamberi, kutaura kwakanyanya / mutauro kusagadzikana, uye corticobasal syndrome, pakati pevamwe.

PSP chirwere chesporadic, chine misiyano yakajairika muMAPT iri iyo inonyanya kukosha njodzi. PSP inowanzoratidza zviratidzo zvayo zvekutanga zvekiriniki mushure memakore makumi mana, nemakore makumi matanhatu nematanhatu paavhareji. Avhareji yenguva yekupona kubva kuchirwere ichitanga kusvika pakufa makore 40., neaspiration pneumonia nekuda kwe bulbar dysphagia iriyo inonyanya kukonzera. Hapana mushonga unobvumidzwa unowanikwa wePSP. Mishonga inokurudzirwa isina-label, physiotherapy uye kurapa kwekutaura kunopa yakanyanya kuganhurirwa uye yenguva kuvandudzwa mumabasa emotokari.

Chinonyanya kukosha chisingakwaniswe mukutsvaga kwePSP kuratidzwa kwemamiriro eprodromal anoratidza PSP, imaging kana fluid biomarkers kuti aongorore zvine hungwaru uye kuronda chirwere, uye kuvandudzwa kwekiriniki ine chinangwa chekugadzirisa zvirwere.

Yakanyorwa naGünter Höglinger, MD, Sachigaro, Dhipatimendi reNeurology, Hannover Medical School, Hannover, Germany (Chikunguru 2019)

Corticobasal Syndrome / Degeneration (CBS/CBD)

Corticobasal syndrome (CBS) chirwere chekiriniki, chiri muboka rezvirwere zveparkinsonian zvisingawanzoitiki. CBS inoratidzwa neprogressive asymmetric akinetic-rigid parkinsonism, ideomotor apraxia, kureva, kutadza kuita zviito zvehunyanzvi kana warairwa, zvinowanzobatanidzwa nedystonia, myoclonus, cortical sensory loss, uye chiitiko chemitezo yekunze, uko varwere vanonzwa sekuti mitezo yavo haisi yavo uye inodzorwa nesimba rekunze.

Ona tsananguro yemakwikwi eCBS/CBD

Kune zvikonzero zvakawanda zveCBS. Chikonzero chinonyanya kukosha icorticobasal degeneration (CBD), 4-repeat (4R) tauopathy. Kuongororwa kwakasiyana kunosanganisira progressive supranuclear palsy (PSP), Alzheimer's disease uye frontotemporal lobar degeneration (FTD) ine TDP-43 inclusions, iyo inogonawo kuonekwa seCBS muchipatara. Zviitiko zvakawanda zveCBS zvinoitika nguva nenguva, asi CBS yemhuri yakaonekwa. Mukupedzisira, progranulin gene (GRN) mutations ndiyo inonyanya kukonzera CBS yemhuri. Pasi rose, progranulin mutations haiwanzoitiki. Kurapwa kweCBS kuchiri zviratidzo nekuwedzera kwemiedzo yekiriniki inotarisa pachirwere ichi.

Yakanyorwa naGiorgia Sciacca, MD, PhD, University of Catania, Catania, Italy (Chivabvu 2026)

Dementia neLewy Bodies (DLB)

Dementia ine Lewy body (DLB) ndiyo yechipiri inonyanya kuzivikanwa chirwere chepfungwa chinoparadza tsinga dzeropa, mushure mechirwere cheAlzheimer, chinotsanangurwa nekusangana kwechirwere chepfungwa nepfungwa dzeParkinson. Kuwanda kweDLB kunowedzera nekukura. Vanhu vakwegura vanosvika 0.3–24.4% vane chirwere chepfungwa vane DLB, iyo inoumba pakati pe4% kusvika 10% pakati pezvirwere zvese zvepfungwa.

Pachivanhu, chirwere cheDLB nechirwere cheParkinson zvinobvumirana kuti kune miviri yeLewy ine alpha-synuclein-positive muuropi. MuDLB inowanikwa munzvimbo dze neocortical ne limbic, kuwedzera kune hunhu hwe degeneration yechirwere cheParkinson.

Ona tsananguro yeDLB yemakwikwi

Zviratidzo zvikuru zveDLB zvinosanganisira kufambisa pfungwa pamwe chete nekuchinja-chinja kweruzivo rwepfungwa, kunyanya kukanganisa kugona kuona zvakanaka pamwe nekushanda zvakanaka kwemaziso, kuona zvisizvo pamwe nekushanda zvakanaka kwemaziso, kuona zvisizvo, uye REM sleep behavior disorder (RBD). Zviratidzo zvekurapa zvinotsigira zvinosanganisira kuvapo kwekunzwa kwakanyanya kune mishonga yekurwisa pfungwa, syncopal episodes kana zvimwe zviitiko zvenguva pfupi zvekusagadzikana uye kusagadzikana kwakanyanya kwekuzvitonga. Zvinonyanya kukosha ndezvekuti, dementia—netsananguro—ndiyo chinhu chikuru chinoratidza, nezviratidzo zveparkinsonian zvichibuda panguva imwe chete kana mukati megore rimwe chete kubva pakutanga kwekuderera kweruzivo rwepfungwa. Saka, DLB inofanira kuongororwa kana dementia ikaitika pamberi kana panguva imwe chete neparkinsonism. Kusiyana neizvi, izwi rekuti Parkinson's disease dementia (PDD) rakachengeterwa kutsanangura dementia inoitika mumamiriro ezvinhu ePD akasimbiswa nekutanga kwekuderera kweruzivo rwepfungwa mushure mekutanga kwezviratidzo zvekufamba.

Mumamiriro ezvinhu ekiriniki, izwi rakakodzera zvikuru ndiro chirwere chemuviri cheLewy. Muzvidzidzo zvekutsvagisa, umo mutsauko unofanirwa kuitwa pakati peDLB nePDD, mutemo wegore rimwe chete uripo pakati pekutanga kwedementia neParkinsonism unoramba uchikurudzirwa.

Yakanyorwa naGiorgia Sciacca, MD, PhD, University of Catania, Catania, Italy (Chivabvu 2026)

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