Iintlobo zeeMoto ze-Neuron Izifo eziquka ii-ALS kunye nezinye
Ukuba abaninzi abantu bacinga nantoni na xa beva amagama "isifo se-neuron," bacinga nge-amyotrophic lateral sclerosis (ALS). Kukho, nangona kunjalo, ezinye iintlobo zezifo ze-neuron. Ngethamsanqa, zonke izifo ze-neuron ziqhelekanga.
Sibanzi
Xa sisuka, izibonakaliso zombane zithunyelwa ukusuka kwingqondo ukuya kumgca womgogodla kunye ne-neurons eziphezulu.
Iiseliti zesishukela se- synapse ekuphambeni kwangaphambili kwintambo yomthambo kwaye zithunyelwa ngaphandle kwee-motor neurons ezisezantsi. Izibonakaliso zombane ezihamba ngeempawu ze-neurons ze-muscle ukuya kwisivumelwano, okubangele ukuhamba. Ngokuxhomekeke kwiziphumo ezithile zokuhlola iziphumo, izazi ze-neurologists ziyakwazi ukubona ukuba ingxaki yintoni kwinkqubo ye-nervous, kwaye isekelwe kuyo, ukuxilongwa.
Iimeko ezichaphazela ukubonakaliswa ngokuqhelekileyo zibhekiswa njengezifo ze-neuron. Uphondo oluphambili lwentambo yomthambo luphethe ulwazi olubhekiselele kwintetho, kanti uphondo lwangaphambili luhamba nolwazi olunxulumene nokuhamba. Izifo ze-motor neurons, ngenxa yoko, zichaphazela kakhulu ukuhamba.
Iimpawu eziPhezulu kunye neMpawu
Izifo ze-neuron ziyakwazi ukwahlukana kwiindidi ezibalulekileyo, kuxhomekeke ekubeni zichaphazela i-motor neuron okanye i-motor neurons ephantsi. Ezinye izifo ze-neuron zithinta kuphela i-motor neurons eziphezulu, kanti ezinye zichaphazela kakhulu i-motor neurons ezantsi.
Ezinye, njenge-ALS, zichaphazela zombini.
Izibonakaliso zezifo eziphezulu ze-neuron ziquka:
- Ukuxhamla - Ukuhlanganiswa kokuqina komsipha, ukuqina, ukunyaniseka, nokungazinzi. Ngoxinzelelo olunzulu, iintlungu zakho zivakalelwa "zizinzile." Ngobuhlungu obukhulu, unokwazi ukuhambisa izihlunu zakho, kodwa ziphendula ngendlela engalindelekanga okanye enje.
- Ubunzima - "ukuqina" kokungabandakanyeki kwezihlunu.
- Ukwandisa i-tendon reflexes ejulile - Ngokomzekelo, i-knee jerk inokuba yinto ephakamileyo ngaphezu kweqhelekileyo.
Izibonakaliso zezifo ezisezantsi ze-neuron ziquka:
- I-Atrophy - Ukulahleka kwamandla kunye nobukhulu bemisipha.
- Ukucatshulwa - Ukuchithwa okungazenzisiyo kunye nokungazibandakanyeki kwezidumbu ezinokubonakala ngathi zikhupha phantsi kwesikhumba.
Iintlobo zeeMoto ze-Neuron
Kukho izifo ezininzi zeemoto ze-neuron ezahlukileyo ngokubhekiselele ekubeni zichaphazela i-neurons engaphezulu okanye ephantsi, iimpawu zokuqala, iqela elidala elichaphazelayo, kunye nokuxela. Ezinye zezi ziquka:
I-Amyotrophic Lateral Sclerosis
I-Amyotrophic lateral sclerosis (ALS), eyaziwa nangokuthi isifo sikaLou Gehrig, sisifo se-neuron esiqhubela phambili esichaphazela abantu abayi-6,000 ngonyaka ngamnye e-United States. Iqala ngobuthathaka bomzimba, ngokuqhelekileyo kwicala elinye lomzimba. Iimeko ziqala ezandleni ngokuphindaphindiweyo kuneenyawo. Ekuqaleni, uphawu oluphambili lunokuthi lube luncedo, kodwa ekugqibeleni luqhubela phambili kunye nezibonakaliso ezisemgangathweni kunye neempawu ze-neuron. Xa i-diaphragm ichaphazelekayo, kufuneka ukuba umoya wokungenisa umoya ufuneke. Isifo ngokuqhelekileyo asiqhelekanga sichaphazele ukuqonda, kwaye abaninzi abantu baqaphele (bengenakho ukugula komzimba) nangona isifo siphambili kakhulu.
Umyinge wexesha lokuphila lobomi kunye ne-ALS iphakathi kweminyaka emihlanu ukuya kwemihlanu kodwa ingahluka ngokubanzi, kunye neepesenti ezintlanu zabantu abaphila emva kweminyaka engama-20. Ngombulelo, abantu abanjengoososayensi uStephen Hawking banceda ekuphakanyiseni i-ALS kunye nezinye izifo ze-neuron.
I-Primary Lateral Sclerosis
I-Primary scalosis sclerosis (PLS) isifo se-motor neurons eziphezulu, ukuphazamisa iimpawu ezisuka kwingqondo ukuya kumgca wesipelini. Iiseli kwi-cortex yecerebral enoxanduva lokunyakaza ngokukhawuleza iyafa. Isiphumo sisinobuthakathaka obuthathaka obuncinane obuhambelana neempawu eziphezulu ze-neuron, ezinjenge-spasticity, rigidity, kunye nokwanda kwe-tendon reflexes.
Ngokungafani ne-amyotrophic lateral sclerosis, ukufunyanwa kwe-motor neuron ephantsi, okufana ne-atrophy kunye ne-fasciculations, ayikho into evelele. Akuqinisekanga ukuba i-PLS eqhelekileyo yintoni, kodwa sikholelwa ukuba ayifani kakhulu kune-ALS.
Ekuqaleni kweso sifo, i-primary scalosis sclerosis ingadideka kunye ne-ALS. Ekubeni i-ALS ingaqala ngeempawu eziphezulu ze-neuron, mhlawumbi iminyaka ngaphambi kokuba ukuxilongwa kwe-PLS kubonakala. Ngaloo xesha, kunokuba kunzima ukuxelela ukuba zeziphi iimeko ezibangela iimpawu, kuba abanye abantu abane-PLS abaza kuthi bahlakulele ukufunyanwa kwee-neuron eziphantsi, ukubonisa ukuba isifo sisona se-ALS. Yonke leyo yindlela edidekayo yokuthetha ukuba akunakwenzeka ukwazi ukuba imeko i-ALS okanye i-PLS iminyaka emininzi emva kokuqala kweempawu.
Ezinye iimeko, ezinjengeparaparesis ezidlulayo zelifa, kuya kufuneka zikhishwe ngaphandle. I-PLS iqhuba inkqubela phambili kancinci kune-ALS, kunye nezigulane ezihlala zifikelela kwiminyaka engama-20 kunye neempawu zazo.
I-Progressive Muscular Atrophy
Ngandlela-thile, i-muscular atrophy (PMA) eqhubela phambili iyaphambene ne-primary scalosis. Kwi-PMA, kuphela i-neurons eziphantsi ezithintekayo, ngelixa, kwi-PLS, kuphela i-motor neurons eziphezulu ezimazileyo. Ekubeni i-motor neurons ephantsi ichaphazelekayo, ubuthathaka obuqhubekayo bubonakaliso oluqhelekileyo. Ekubeni i-motor neurons ephezulu ayichaphazelekayo, iimpawu ze-motor neuron eziphezulu ezinjenge-rigidity ayikho. I-atrophy ye-muscular progress is less common than ALS kodwa i-prognosis engcono.
Ingaba yinkqubo enzima yokwenza ukuba uxilongo lwe-atrophy ye-muscular progression kuba iimpawu zifana nezinye iimeko. Ngokukodwa, izifo ezifana ne-ALS, i-multifocal motor neuropathy (uhlobo lwe- peripheral neuropathy ) kunye ne- sprotic muscular atrophy kufuneka liqale lilawulwe ngaphandle kokuqala ukuxilongwa.
Ukuguqulwa kweBarbar Palsy
Inkqubela ye-bulbar ehamba phambili iquka ukungcola okucothayo kwengqondo, equlethe iimbilini ( imiqobo yegazi ) elawula ubuso, ulwimi, nomqala. Ngenxa yoko, umntu onenkqubela yokuphalaza iya kuqala ukufumana ubunzima bokuthetha, ukugwinya nokuhlafuna. Ubuthakathaka be-Limb bungabonakala ngokugqithiseleyo xa isifo sifana, kunye nezibonakaliso eziphezulu kunye nezisezantsi ze-neuron. Abantu abanenkqubela yokuhlaselwa yintyatyambo banokungalawulwa kwaye ngezinye izihlandlo zokungahleki okanye ukukhala. Akuqhelekanga ukuba abantu abanokukhubazeka okuqhubekayo baqhubeke nokuphuhlisa ii-ALS. I-Myasthenia gravis yinkinga ye-neuromuscular autoimmune engabonakalisa ngendlela efanayo.
I-Post-Polio Syndrome
I-polio yintsholongwane ehlasele i-motor neurons ekuphambeni kwangaphambili kwintambo yomgudu, okubangelwa ukukhubazeka. Ngombulelo, ngenxa yokugonywa kwamagciwane, le ntsho longwane iphelile. Nangona kunjalo, abanye balabo bafumana isifo, banokukhalaza ngobuthathaka obubizwa ngokuba yi-post-polio syndrome. Oku kunokubangelwa ukuguga okanye ukulimala okubangela ukuba i-neurons ehamba phambili imoto ilawule ukuhamba kwelungu elichaphazelekayo ngaphambili. Ingxaki ichaphazela abantu abadala asebekhulile. Ngokuqhelekileyo akusongela ubomi.
Izifo zikaKennedy
Isifo sikaKennedy sisisigxina sokuguquka kohlobo lwe-X oluchaphazelekayo oluchaphazela i-androgen receptor. Ingxaki leyo ibangela ubuthathaka obuhamba phambili kunye nentlungu yeemisipha eziseduze naso. Ubuso, umhlathi kunye nolwimi zibandakanyeka. Ngenxa yokuba iX-axhunyiwe, isifo sikaKennedy sichaphazela kakhulu abantu. Abasetyhini kunye nokuguquka kwemfuyo bayabathwali, abanama-50 ekhulwini amathuba okudlulisa umtya kubantwana babo. Abasetyhini kunye nokuguqulwa kwamanzi bangaphinda baxhamle kwiimpawu ezincinci, ezinjengeenkomfa zamanwe, endaweni yobuthakathaka obuninzi.
Ngenxa yokuba isifo sichaphazela i-androgen receptor (i-receptor apho i-estrogen kunye ne-testosterone ifakwe), amadoda anesifo abuye ahlupheke kwiimpawu ezifana ne- gynecomastia (ukukhulisa isifuba), i-atrophy ye-testicular kunye ne-erectile disysction. Ukuphila kwabantu abanesifo sikaKennedy kuvame ukuqhelekileyo, nangona ubuthathaka babo beqhubeka befuna isitulo sabakhubazekile.
I-Spinal Muscular Atrophy
I-atrophy ye-spinal muscular isifo esizuze njengefa esichaphazela kakhulu abantwana. Yibangelwa yimpembelelo kwi-gene NOMSNA kwaye ilifa kwi-autosomal pattern. Ngenxa yolu geni olungalunganga, akukho neprotheni eyenziwe yintsimi eyenziwe, kwaye oku kuholele ekuhlahleni kwee-motor neurons eziphantsi. Oku kubangela ubuthathaka kunye nokuphazamiseka kwemizimba.
Kukho iintlobo ezintathu eziphambili ze-SMA, nganye ibandakanya abantwana ngexesha elihlukile.
- Uhlobo lwe-SMA 1, olubizwa nangokuthi isifo sikaWerdnig-Hoffman, luyabonakala ngexesha lokuba umntwana uneminyaka engathandathu ubudala. Umntwana uya kuba ne-hypotonia (imisipha ye-floppy) kwaye akayi kuhamba ngokukhawuleza. Abayi kukwazi ukuhlala bodwa ngexesha elilindelekileyo. Ngenxa yobunzima be-airway kunye nokugcina amandla anele ukuphefumula, uninzi lwaba bantwana bafa baneminyaka emibini.
- Uhlobo lwe-SMA II luqala kancinci kamva, lubonakala phakathi kweminyaka eyi-6 ukuya kwezi-18. Aba bantwana abayi kukwazi ukuma okanye bahambe ngaphandle koncediso, kwaye baya kuba nobunzima bokuphefumula. Nangona kunjalo, abantwana abane-SMA hlobo II bahlala ixesha elide kunabo bahlala no-Werdnig-Hoffman, ngamanye amaxesha bahlala ebuntwaneni abadala.
- Uhlobo lwe-SMA IIII lubizwa ngokuba yi-Kugelberg-Welander isifo, kubonakala phakathi kweminyaka emi-2 ne-17. Abantwana abanalesi sifo banokuba nobunzima bokusebenza okanye ukunyuka kwamanyathelo. Basenokuba neengxaki zangemva , ezifana ne- scoliosis . Nangona kunjalo, abantwana abanalesi sifo banokuphila ngokuqhelekileyo.
Ukuxilongwa kunye noTyango
Akukho nonyango olusebenzayo kuyo nayiphi na yezifo ze-neuron. Ulwaphulo lwezokwelapha lujolise ekulawuleni iimpawu zesifo ngokusemandleni akho. Nangona kunjalo, ukuze ukwazi ukuba yiziphi iimpawu zokulindeleka, kwakunye nokulawula ezinye izifo eziphathekayo, kubalulekile ukuba ufumane unyango olufanelekileyo.
Ukusebenzisa uvavanyo lwabo lomzimba kunye nezinye iindlela ezifana ne- electromyography , izifundo zophando lwe-nerve, kunye nokuhlolwa kofuzo xa kufanelekile, i-neurologists inokukunceda ukuchaza ukuchonga okufanelekileyo. Ukufumana i-diagnostic eyiyo kuvumela i-neurologist yakho ukuba ikwazi ukulawula iimpawu zakho ngokusemandleni akho kunye nokukulindela nokulungiselela zonke iingxaki ezilindelekileyo.
Ukujamelana
Ekuqaleni, sathi "inhlanhla" izifo ze-neuron ziqhelekanga. Oku kungalungile ngaphandle kokuba wena okanye othandekayo ahlakulele enye yale miqathango. Emva koko, ngaphezu kokubandezeleka kweempawu zezi zifo, unokufumanisa ukuba kukho uphando olungaphantsi kunye nenkxaso engaphantsi kunokuba ungathemba. Nangona ezi zifo ziqhelekanga, amanyathelo afana ne- Orphan Drug Act ayalela ingqwalaselo engakumbi kwiimeko eziqhelekileyo kodwa ezingekho phantsi kwezinto ezingabalulekanga.
Unokuziva wedwa xa ufumene ukuba unesifo se-neuron. Ngokungafani namaqela amakhulu "abameli beengcingo zesifuba" apho, asiboni amaqela amakhulu, umzekelo, abaqhubi beepalli eziqhubekayo. Nangona kunjalo ukuqonda kukhula, kwaye ubuncinane kwi-ALS, inkxaso.
Abantu abanezifo ze-neuron zifuna ukuxhaswa njengalezo ziimeko eziqhelekileyo. Ngelixa ungeke ube neqela loxhaso kuluntu lwakho, kukho inkxaso yoluntu kwi-intanethi apho abantu abaneemeko ezithile ze-neuron "badibana" kwaye banxibelelana nabanye abajongene neminye imingeni efanayo. Nangona singenayo "ipilisi" okanye utyando lokunyangwa kwesi sifo, kukho into enokuyenza ukuze kuncede abantu baphile kakuhle ngesifo, kwaye uphando lwangoku lunikeza ithemba lokuba phambili kuya kwenziwa kwikamva.
Imithombo
- Garg, N., Ipaki, S., Vucic, S. et al. Ukuhlukana kweMoto ye-Neuron Syndromes ephantsi. I-Journal ye-Neurology, i-Neurosurgery kunye ne-Psychiatry . 2016 Disemba 21. (Epub ngaphambi kokuprinta).
- Kasper, uDennis L .., u-Anthony S. Fauci, noStephen L .. Hauser. Iinqununu zeHarrison zeMithi yoPhakathi. ENew York: iMac Graw-Hill Education, 2015. Phrinta.
- Ng, L., Khan, F., Young, C., noM M. Galea. Iimpawu zonyango kwi-Amyotrophic Lateral Sclerosis / i-Motor Neuron. I-Cochrane Database yeeNkqubo eziPhezulu . 2017. 1: CD0111776.
- Roper. I-Adams kunye neMigaqo kaVictor ye-Neurology, 10e. Np: McGraw-Hill, 2014. Print.