Oʻtkir osti sklerozlashgan panensefalit

Oʻtkir osti sklerozlashgan panensefalit (SSPE) — Dawson kasalligi deb ham ataladigan bu kasallik qizamiq virusi bilan kasallanish natijasida kelib chiqadigan progressiv miya yallig'lanishining noyob shakli hisoblanadi. Kasallik koʻproq bolalar, oʻsmirlar va yoshlarga kuzatiladi. Taxminlarga koʻra, qizamiq bilan kasallangan har 10 000 kishidan 2 nafarida oxir-oqibat SSPE kuzatilish ehtimoli yuoqri^[1]. Biroq, 2016-yilgi tadqiqotga koʻra, 15 oygacha boʻlgan emlanmagan chaqaloqlarda bu koʻrsatkich nisbatan yuqori yaʼni 609 ta chaqaloqdan tasida SSSPE kuzatilgan^[2]^[3]. Bugungi kunga qadar SSPEning hech qanday davosi mavjud emas va bu holat kasallik deyarli har doim oʻlimga bilan yakunlanadi. SSPEni qizamiq virusi sabab boʻlishi mumkin boʻlgan oʻtkir tarqalgan ensefalomiyelit bilan adashtirmaslik kerak, sababi ensefalomiyelitning davomiyligi boshqacha^[4]. SSPE vujudga kelishiga vaksina shtammlari emas, balki yovvoyi turdagi virus sababchi boʻladi^[5]^[6].

Simptomlari

SSPEning paydo boʻlish ehtimoli qizamiq infeksiyasi tarixi bilan boshlanishi mumkin, keyin oʻrtacha 7 yil davom etadigan asimptomatik davr boshlanadi, lekin bu davr 1 oydan 27 yilgacha davom etishi mumkin. Asimptomatik davrdan soʻng, xulq-atvorning oʻzgarishi, intellektual muammolar, miyoklonik buzilishlar, koʻrlik, ataksiya va oxir-oqibat oʻlim bilan yakunlanadigan progressiv nevrologik buzilish sodir boʻladi^[7]^[8].

Rivojlanish bosqichlari

Kasallik simptomlari quyidagi 4 ta bosqichda kuzatiladi:^[9]^[10]:

1-bosqich: shaxsiyat oʻzgarishi, kayfiyat oʻzgarishi yoki tushkunlik boʻlishi mumkin. Isitma, bosh og'rig'i va xotira yoʻqolishi ham boʻlishi mumkin. Ushbu bosqich 6 oygacha davom etishi mumkin.
2-bosqich: Bu bosqichda titrash, mushaklarning spazmlari, tutqanoqlar, koʻrishning yoʻqolishi va demensiya kuzatiishi mumkin.
3-bosqich: Doimiy harakatlarda buzilishlar kuzatiladi. Ushbu bosqichda asoratlar koʻrlik yoki oʻlimga olib kelishi mumkin.
4-bosqich: falajdan oldingi yoki doimiy vegetativ holatga progressiv ongni yoʻqotish oxirgi bosqichda sodir boʻladi, bu davrda nafas olish, yurak urishi va qon bosimilarida oʻzgarishlar kuzatiladi. O'lim odatda isitma, yurak yetishmovchiligi yoki miyaning avtonom nerv tizimini nazorat qila olmasligi natijasida yuzaga keladi.

Patogenezi

Koʻp miqdordagi nukleokapsidlar neyronlar va glial hujayralarda ishlab chiqariladi. Bu hujayralarda immunitet reaksiyasini qoʻzgatuvchi virus genlari aktiv faoliyati susayadi^[11]. Natijada, M oqsili kabi elementlar ishlab chiqarilmaydi va virus immunitet reaktsiyasini qoʻzgʻatmasdan doimiy ravishda inson tanasida yashash imkoniyatiga ega boʻladi. Oxir-oqibat infektsiya SSPEga olib keladi^[12].

Diagnoz

Merck qoʻllanmasiga koʻra^[8]:

SSPE demensiya va nerv-mushak tizimida tutqanoq kuzatilish xususiyati bilan kasallangan kichik yoshli bemorlarda uchrashi gumon qilinadi. EEG, KT yoki MRT, CSF tekshiruvi va qizamiqning serologik tekshiruvi oʻtkaziladi. EEG tekshiruvi davomida sinxron ravishda yuzaga keladigan yuqori kuchlanishli difazik toʻlqinlar bilan davriy komplekslarni koʻrsatadi. KT yoki MRT kortikal atrofiya yoki oq moddaning shikastlanishi odatda normal bosimni, hujayralar miqdorini va umumiy protein miqdorini koʻrsatishi mumkin. Ammo CSFda globulin deyarli har doim yuqori boʻladi. Qon zardobi va globulin kasallik rivojlanishi bilan mavjud Virusga qarshi IgG koʻpayishiga olib keladi, agar test natijalari aniq boʻlmasa, miya biopsiyasi kerak boʻlishi mumkin.

Davolash

Kasallikning hech qanday davosi yoʻq^[13]. Agar tashxis 1-bosqichda qoʻyilgan boʻlsa, kasallikni ogʻiz orqali izoprinozin va intraventrikulyar interferon alfa bilan davolash mumkin, ammo bu dorilarga javob rekasiyasi bemorlarda turlicha va shu kunga qadar yagona qabul qilingan davolash usuli hisoblanadi^[14]. 2-bosqich boshlanganidan keyin kasallik har doim oʻlim bilan yakunlanadi^[8].

Prognoz

Kasallikning klassik koʻrinishida oʻlim 1-3 yil ichida sodir boʻladi^[15], ammo bu holat kasallikning kechishiga qarab tezroq yoki sekinroq rivojlanishi ham mumkin. Kasallikning oʻtkir fulminant SSPE holatlarida ahvoning tezroq yomonlashuvi, tashxisdan keyin 3 oy ichida oʻlimga kuzatilgan holatlar mavjud^[16]^[17].

Epidemiologiyasi

SSPE kamdan-kam uchraydigan kasallik, ammo Osiyo va Yaqin Sharqda nisbatan yuqori kasallanish hali ham mavjud. Xususan 2024-yilda Oʻzbekistonda SSPE bilan kasallangnalar soni 100 nafarga yaqinlashgan va bir nechta oʻlim kuzatilgan^[18]^[19]. Qizamiqga qarshi vaksina joriy etilganidan beri kasallanish dunyo boʻyicha sezilarli darajada kamaygan.

Manbalar

↑ "Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized.". The Journal of Infectious Diseases 192 (10): 1686–1693. 2005. doi:10.1086/497169. PMID 16235165.
↑ "Subacute Sclerosing Panencephalitis: The Devastating Measles Complication Is More Common Than We Think.". Open Forum Infectious Diseases 3. 2016. doi:10.1093/ofid/ofw194.81.
↑ Sun, Lena. „New data shows a deadly measles complication is more common than thought“ (2016-yil 28-oktyabr). Qaraldi: 2016-yil 28-oktyabr.
↑ "Measles-induced encephalitis". QJM 108 (3): 177–182. 2015. doi:10.1093/qjmed/hcu113. PMID 24865261.
↑ Jafri, Sidra K; Kumar, Raman; Ibrahim, Shahnaz H (2018-06-26). "Subacute sclerosing panencephalitis – current perspectives". Pediatric Health, Medicine and Therapeutics 9: 67–71. doi:10.2147/PHMT.S126293. ISSN 1179-9927. PMID 29985487. PMC 6027681. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=6027681.
↑ Campbell, H; Andrews, N; Brown, K E; Miller, E (2007). "Review of the effect of measles vaccination on the epidemiology of SSPE". International Journal of Epidemiology 36 (6): 1334–1348. doi:10.1093/ije/dym207. PMID 18037676. https://archive.org/details/sim_international-journal-of-epidemiology_2007-12_36_6/page/1334.
↑ „CDC pinkbook“ (2019-yil 29-mart).
↑ ^8,0 ^8,1 ^8,2 „merckmanuals.com“.
↑ „medline.gov“.
↑ National Institute of Neurological Disorders and Stroke (NINDS). „Subacute Sclerosing Panencephalitis Information Page | National Institute of Neurological Disorders and Stroke“. ninds.nih.gov (2019-yil 27-mart). — „The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brainʼs inability to continue controlling the autonomic nervous system.“. Qaraldi: 2021-yil 21-noyabr.
↑ Jawetz. Melnick & Adelberg's Medical Microbiology. Lange, 2010 — 586-bet. ISBN 978-0-07-174271-9.
↑ Carter, M. J.; Willcocks, M. M.; Ter Meulen, V. (1983). "Defective translation of measles virus matrix protein in a subacute sclerosing panencephalitis cell line.". Nature 305 (5930): 153–5. doi:10.1038/305153a0. PMID 6888557. PMC 7094927. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=7094927.
↑ Rocke, Zoe; Belyayeva, Mariya (2022), „Subacute Sclerosing Panencephalitis“, StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 32809508, qaraldi: 2023-03-10
↑
- Gascon G, Yamanis S, Crowell J, et al. Combined oralisoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993; 15:346-55.
- Anlar B, Yalaz K, Oktem F (1997). "Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon". Neurology 48 (2): 526–8. doi:10.1212/wnl.48.2.526. PMID 9040751. https://archive.org/details/sim_neurology_1997-02_48_2/page/526.
- Cianchetti C, Marrosu MG, Muntoni F (1998). "Intraventricularalpha-interferon in subacute sclerosing panencephalitis". Neurology 50 (1): 315–16. doi:10.1212/wnl.50.1.315. PMID 9443512. https://archive.org/details/sim_neurology_1998-01_50_1/page/315.
↑ „SSPE information page www.ninds.nih.gov“.
↑ Risk WS, Haddad FS (1979). "The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East". Arch Neurol 56 (10): 610–14. doi:10.1001/archneur.1979.00500460044004. PMID 485888.
↑ Garg, R K (1 February 2002). "Subacute sclerosing panencephalitis". Postgraduate Medical Journal 78 (916): 63–70. doi:10.1136/pmj.78.916.63. PMID 11807185. PMC 1742261. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1742261.
↑ „Samarqandda 5 yashar bola kamyob kasallikdan vafot etdi“.
↑ „Ўзбекистонда бир ҳафта ичида учинчи бола камёб касалликдан вафот этди“.

[Bellini2005-1] "Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized.". The Journal of Infectious Diseases 192 (10): 1686–1693. 2005. doi:10.1086/497169. PMID 16235165.

[Wendorf2016-2] "Subacute Sclerosing Panencephalitis: The Devastating Measles Complication Is More Common Than We Think.". Open Forum Infectious Diseases 3. 2016. doi:10.1093/ofid/ofw194.81.

[newdata-3] Sun, Lena. „New data shows a deadly measles complication is more common than thought“ (2016-yil 28-oktyabr). Qaraldi: 2016-yil 28-oktyabr.

[4] "Measles-induced encephalitis". QJM 108 (3): 177–182. 2015. doi:10.1093/qjmed/hcu113. PMID 24865261.

[5] Jafri, Sidra K; Kumar, Raman; Ibrahim, Shahnaz H (2018-06-26). "Subacute sclerosing panencephalitis – current perspectives". Pediatric Health, Medicine and Therapeutics 9: 67–71. doi:10.2147/PHMT.S126293. ISSN 1179-9927. PMID 29985487. PMC 6027681. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=6027681.

[6] Campbell, H; Andrews, N; Brown, K E; Miller, E (2007). "Review of the effect of measles vaccination on the epidemiology of SSPE". International Journal of Epidemiology 36 (6): 1334–1348. doi:10.1093/ije/dym207. PMID 18037676. https://archive.org/details/sim_international-journal-of-epidemiology_2007-12_36_6/page/1334.

[pinkbook-7] „CDC pinkbook“ (2019-yil 29-mart).

[merckmanual-8] 8,0 ^8,1 ^8,2 „merckmanuals.com“.

[medline-9] „medline.gov“.

[NINDS-10] National Institute of Neurological Disorders and Stroke (NINDS). „Subacute Sclerosing Panencephalitis Information Page | National Institute of Neurological Disorders and Stroke“. ninds.nih.gov (2019-yil 27-mart). — „The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brainʼs inability to continue controlling the autonomic nervous system.“. Qaraldi: 2021-yil 21-noyabr.

[11] Jawetz. Melnick & Adelberg's Medical Microbiology. Lange, 2010 — 586-bet. ISBN 978-0-07-174271-9.

[12] Carter, M. J.; Willcocks, M. M.; Ter Meulen, V. (1983). "Defective translation of measles virus matrix protein in a subacute sclerosing panencephalitis cell line.". Nature 305 (5930): 153–5. doi:10.1038/305153a0. PMID 6888557. PMC 7094927. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=7094927.

[13] Rocke, Zoe; Belyayeva, Mariya (2022), „Subacute Sclerosing Panencephalitis“, StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 32809508, qaraldi: 2023-03-10

[14] 
Gascon G, Yamanis S, Crowell J, et al. Combined oralisoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993; 15:346-55.

Anlar B, Yalaz K, Oktem F (1997). "Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon". Neurology 48 (2): 526–8. doi:10.1212/wnl.48.2.526. PMID 9040751. https://archive.org/details/sim_neurology_1997-02_48_2/page/526.

Cianchetti C, Marrosu MG, Muntoni F (1998). "Intraventricularalpha-interferon in subacute sclerosing panencephalitis". Neurology 50 (1): 315–16. doi:10.1212/wnl.50.1.315. PMID 9443512. https://archive.org/details/sim_neurology_1998-01_50_1/page/315.

[15] Gascon G, Yamanis S, Crowell J, et al. Combined oralisoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis. Brain Dev. 1993; 15:346-55.

[16] Anlar B, Yalaz K, Oktem F (1997). "Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon". Neurology 48 (2): 526–8. doi:10.1212/wnl.48.2.526. PMID 9040751. https://archive.org/details/sim_neurology_1997-02_48_2/page/526.

[17] Cianchetti C, Marrosu MG, Muntoni F (1998). "Intraventricularalpha-interferon in subacute sclerosing panencephalitis". Neurology 50 (1): 315–16. doi:10.1212/wnl.50.1.315. PMID 9443512. https://archive.org/details/sim_neurology_1998-01_50_1/page/315.

[NIH-15] „SSPE information page www.ninds.nih.gov“.

[16] Risk WS, Haddad FS (1979). "The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East". Arch Neurol 56 (10): 610–14. doi:10.1001/archneur.1979.00500460044004. PMID 485888.

[Garg2001-17] Garg, R K (1 February 2002). "Subacute sclerosing panencephalitis". Postgraduate Medical Journal 78 (916): 63–70. doi:10.1136/pmj.78.916.63. PMID 11807185. PMC 1742261. //www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1742261.

[18] „Samarqandda 5 yashar bola kamyob kasallikdan vafot etdi“.

[19] „Ўзбекистонда бир ҳафта ичида учинчи бола камёб касалликдан вафот этди“.

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