Mawallafi: Gregory Harris
Ranar Halitta: 10 Afrilu 2021
Sabuntawa: 22 Yuni 2024
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[Neurology] Myoclonus in Creutzfeldt - Jacob Disease
Video: [Neurology] Myoclonus in Creutzfeldt - Jacob Disease

Cutar Creutzfeldt-Jakob (CJD) wani nau'i ne na lalacewar kwakwalwa wanda ke haifar da raguwar saurin motsi da asarar aikin tunani.

CJD ya samo asali ne daga furotin da ake kira prion. Prion yana haifar da sunadarai na al'ada suyi ninki ba daidai ba.Wannan yana shafar sauran sunadaran ikon aiki.

CJD yana da wuya. Akwai nau'ikan da yawa. Nau'ukan gargajiya na CJD sune:

  • CJD mai rikitarwa yana ɗaukar mafi yawan lokuta. Yana faruwa ba tare da sanannen dalili ba. Matsakaicin shekarun da ya fara shine 65.
  • CJD na Iyali yana faruwa ne lokacin da mutum ya gaji prion mara kyau daga iyaye (wannan nau'in CJD ba safai ba).
  • CJD da aka samo ya haɗa da bambancin CJD (vCJD), nau'in da ke da alaƙa da cutar saniya. Iatrogenic CJD shima nau'i ne na cutar. IJErogenic CJD wani lokaci ana wucewa ta hanyar karɓar kayan jini, dasawa, ko gurɓatattun kayan aikin tiyata.

Bambancin CJD yana faruwa ne ta hanyar cin naman ƙwayar cuta. Kamuwa da cuta da ke haifar da cutar a cikin shanu an yi imanin cewa daidai ne wanda ke haifar da vCJD a cikin mutane.


Bambance-bambancen CJD yana haifar da ƙasa da kashi 1 cikin 100 na duk shari'ar CJD. Yana da lahani ga matasa. Kasa da mutane 200 a duniya sun kamu da wannan cutar. Kusan dukkan shari'o'in sun faru ne a Ingila da Faransa.

CJD na iya kasancewa da alaƙa da wasu cututtukan da yawa waɗanda prions suka haifar, gami da:

  • Cutar da cutar ta yau da kullun (wanda aka samo a cikin barewa)
  • Kuru (galibi mata ne a New Guinea waɗanda suka ci kwakwalwar dangin da suka mutu a matsayin wani ɓangare na bikin binne gawa)
  • Scrapie (an samo a cikin tumaki)
  • Sauran cututtukan mutane da ba a gado sosai, kamar su Gerstmann-Straussler-Scheinker da kuma rashin barci na dangi

Alamomin CJD na iya haɗawa da ɗayan masu zuwa:

  • Rashin hankali wanda ke saurin lalacewa cikin weeksan makonni ko watanni
  • Rashin gani (wani lokacin)
  • Canje-canje a cikin tafiya (tafiya)
  • Rikicewa, rikicewa
  • Mafarki (gani ko jin abubuwan da basa nan)
  • Rashin daidaituwa (misali, tuntuɓe da faɗuwa)
  • Musarfin tsoka, juyawa
  • Jin juyayi, tsalle
  • Yanayin mutum yana canzawa
  • Bacci
  • Kwatsam motsi ko kamawa
  • Matsalar magana

Farkon cutar, tsarin juyayi da binciken hankali zai nuna ƙwaƙwalwar ajiya da matsalolin tunani. Daga baya a cikin cutar, gwajin tsarin motsa jiki (gwaji don gwada juzu'in tsoka, ƙarfi, daidaitawa, da sauran ayyukan jiki) na iya nuna:


  • Abubuwa na yau da kullun ko karuwar martani na yau da kullun
  • Inara yawan sautin tsoka
  • Tsokar tsoka da spasms
  • Responsearamar amsa mai ƙarfi
  • Rauni da asarar tsoka (ɓarnar tsoka)

Akwai asarar daidaituwa da canje-canje a cikin cerebellum. Wannan shine yankin kwakwalwar da ke sarrafa daidaituwa.

Binciken ido yana nuna wuraren makantar da mutum ba zai iya lura da shi ba.

Gwaje-gwajen da aka yi amfani dasu don tantance wannan yanayin na iya haɗawa da:

  • Gwajin jini don kawar da wasu nau'ikan cututtukan ƙwaƙwalwa da kuma neman alamomin da wasu lokuta ke faruwa tare da cutar
  • CT scan na kwakwalwa
  • Kayan lantarki (EEG)
  • MRI na kwakwalwa
  • Spinal tap don gwada don furotin da ake kira 14-3-3

Ba za a iya tabbatar da cutar ba tare da nazarin halittar ƙwaƙwalwa ko kuma bincikar kansa ba. A yau, da wuya a yi gwajin ƙwayoyin cuta don bincika wannan cutar.

Babu sanannen magani ga wannan yanayin. Anyi kokarin gwada magunguna daban daban dan rage cutar. Wadannan sun hada da maganin rigakafi, magunguna don farfadiya, masu rage jini, magungunan kashe ciki, da kuma interferon. Amma babu wanda ke aiki da kyau.


Manufar magani ita ce samar da yanayi mai aminci, sarrafa zafin rai ko tada hankali, da kuma biyan bukatun mutum. Wannan na iya buƙatar saka idanu da taimako a cikin gida ko a wurin kulawa. Nasihun dangi na iya taimakawa dangi wajen jurewa da canje-canjen da ake bukata na kula da gida.

Mutanen da ke da wannan yanayin na iya buƙatar taimako don sarrafa rashin yarda ko halaye masu haɗari. Wannan ya haɗa da ba da ladabi ga halaye masu kyau da kuma watsi da halaye marasa kyau (idan yana da aminci). Hakanan suna iya buƙatar taimako don fuskantar al'amuran su. Wani lokaci, ana buƙatar magunguna don taimakawa wajen magance zalunci.

Mutanen da ke tare da CJD da dangin su na iya buƙatar neman shawara ta doka da wuri a cikin rikicewar cutar. Umarnin ci gaba, ikon lauya, da sauran ayyukan shari'a na iya sauƙaƙa yanke shawara game da kula da mutumin da ke tare da CJD.

Sakamakon CJD ba shi da kyau. Mutanen da ke da CJD kwatsam ba za su iya kula da kansu ba a cikin watanni 6 ko afterasa bayan bayyanar cututtuka ta fara.

Rikicin na mutuwa ne cikin kankanin lokaci, galibi cikin watanni 8. Mutanen da ke da bambancin CJD suna ƙara yin rauni a hankali, amma yanayin har yanzu yana mutuwa. Wasu 'yan mutane suna rayuwa har tsawon shekara 1 ko 2. Dalilin mutuwa galibi kamuwa da cuta ne, gazawar zuciya, ko gazawar numfashi.

Aikin CJD shine:

  • Kamuwa da cutar
  • Tsananin rashin abinci mai gina jiki
  • Rashin hankali a wasu lokuta
  • Rashin ikon hulɗa da wasu
  • Rashin iya aiki ko kula da kai
  • Mutuwa

CJD ba gaggawa ta gaggawa ba ce. Koyaya, yin bincike da magani tun da wuri na iya sauƙaƙa alamun cutar da sauƙi, ba marasa lafiya lokaci don yin umarni na gaba da shirya don ƙarshen rayuwa, da ba iyalai ƙarin lokaci don daidaita yanayin.

Ya kamata a cire kayan aikin likitanci waɗanda zasu iya gurɓata daga sabis kuma zubar dasu. Mutanen da aka sani suna da CJD bai kamata su ba da gudummawar cornea ko sauran kayan jikin ba.

Yawancin ƙasashe yanzu suna da ƙa'idodin ƙa'idodi don kula da shanun da ke fama da cutar don guje wa watsa CJD ga mutane.

Maganin yaduwar cutar sankara; vCJD; CJD; Yakubu-Creutzfeldt cuta

  • Creutzfeldt-Jakob cuta
  • Tsarin juyayi na tsakiya da tsarin juyayi na gefe

Bosque PJ, Tyler KL. Prions da prion cuta na tsarin mai juyayi na tsakiya (cututtukan neurodegenerative mai yaduwa). A cikin: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, da Ka'idojin Bennett da Aiwatar da Cututtukan Cutar. 9th ed. Philadelphia, PA: Elsevier; 2020: babi na 179.

Geschwind MD. Cututtukan prion. A cikin: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology a cikin Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016: babi na 94.

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