{"id":7101,"date":"2023-12-17T23:35:06","date_gmt":"2023-12-17T22:35:06","guid":{"rendered":"https:\/\/veterinarska-stanica-journal.hr\/?post_type=article&p=7101"},"modified":"2023-12-17T23:35:06","modified_gmt":"2023-12-17T22:35:06","slug":"chiari-like-malformacija","status":"publish","type":"article","link":"https:\/\/journal.h3s.org\/?article=chiari-like-malformacija","title":{"rendered":"“Chiari-like<\/em>” malformacija"},"content":{"rendered":"

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E. Pongrac<\/strong>*, A. Rai\u0107<\/strong>, L. E\u0107imovi\u0107<\/strong>, I. Ki\u0161<\/strong>, M. Efendi\u0107<\/strong>, G. Jurki\u0107 Krsteska<\/strong> i M. Brklja\u010di\u0107<\/strong><\/p>\n

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Elizabeta PONGRAC<\/strong>*, dr. med. vet. (dopisni autor, e-mail: epongrac@vef.hr), Anja RAI\u0106<\/strong>, dr. med. vet., Luka E\u0106IMOVI\u0106<\/strong>, dr. med. vet., dr. sc. Ivana KI\u0160<\/strong>, dr. med. vet., izvanredna profesorica, Ma\u0161a EFENDI\u0106<\/strong>, dr. med. vet., asistentica, dr.sc. Gabrijela JURKI\u0106 KRSTESKA<\/strong>, dr. med. vet., dr. sc. Mirna BRKLJA\u010cI\u0106<\/strong>, dr. med. vet., izvanredna profesorica, Veterinarski fakultet Sveu\u010dili\u0161ta u Zagrebu, Hrvatska<\/div>\n
\"\"<\/a>https:\/\/doi.org\/10.46419\/vs.55.4.9<\/a><\/div>\n<\/p>\n

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Sa\u017eetak<\/a>Uvod<\/a>Patofiziologija<\/a>Klini\u010dki znaci<\/a>Dijagnostika<\/a>Lije\u010denje<\/a>Zahvala<\/a>Literatura<\/a>Abstract<\/a><\/span><\/div>\n<\/div>\n

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Sa\u017eetak<\/h2>\n
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\u201eChiari-like<\/em>\u201c malformacija (CLM) je priro\u0111ena anomalija lubanje koja mo\u017ee rezultirati hernijacijom maloga mozga kroz foramen magnum<\/em>. Kao posljedica CLM-a javlja se siringomijelija koju obilje\u017eavaju novonastale \u0161upljine u le\u0111noj mo\u017edini. \u201eChiari-like<\/em>” malformacija naj\u010de\u0161\u0107e se javlja kod kavalirskog \u0161panijela kralja Charlesa u dobi od 3. do 6. godine \u017eivota, a manifestira se klini\u010dkim znakovima od strane \u017eiv\u010danog sustava od kojih je naj\u010de\u0161\u0107i neuropatska bol. Za postavljanje definitivne dijagnoze potrebna je magnetska rezonanca (MR). Uvijek se prvo pristupa konzervativnom lije\u010denju, a ukoliko konzervativno lije\u010denje ne polu\u010di u\u010dinak ili kada se klini\u010dki simptomi pogor\u0161aju preporu\u010da se kirur\u0161ko lije\u010denje. Konzervativna terapija naj\u010de\u0161\u0107e uklju\u010duje lijekove koji smanjuju neuropatsku bol.<\/p>\n

Klju\u010dne rije\u010di:<\/strong> \u201echiari-like\u201c malformacija, siringomijelija, kavalirski \u0161panijel kralja Charlesa, neuropatska bol<\/em><\/p><\/blockquote>\n

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Uvod<\/h2>\n
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\u201eChiari-like<\/em>\u201c malformacija (CLM) je bolest koja se sve \u010de\u0161\u0107e prepoznaje kod pasa. Radi se o slo\u017eenoj razvojnoj malformaciji lubanje i kranio-cervikalnih kralje\u0161aka koju karakterizira konformacijska promjena pri kojoj dolazi do hernijacije (Rushbridge, 2020.). Naime, kranijalna i kaudalna fosa su premale za volumen mozga koji bi u njih trebao stati \u0161to rezultira hernijacijom maloga mozga kroz foramen magnum<\/em> (Dewey i Costa, 2016.).
\nU korelaciji s CLM-om javlja se siringomijelija (SM), odnosno stvaranje \u0161upljina (syrinx<\/em>) koje se pune likvorom unutar parenhima le\u0111ne mo\u017edine, a nastaju zbog kompresije mo\u017edanog debla i le\u0111ne mo\u017edine te zbog opstrukcije kanala le\u0111ne mo\u017edine (Taylor, 2020.). Naj\u010de\u0161\u0107e se javlja kod malih i kod brahicefali\u010dnih pasmina pasa (kavalirskog \u0161panijela kralja Charlesa, njema\u010dkog \u0161pica, patuljastih pudli, \u010divava, francuskih buldoga, mopsova, briselskih grifona) (Dewey i Costa, 2016.).
\nVe\u0107ina pasa s CLM-om po\u010dinje pokazivati klini\u010dke simptome u rasponu od 3. do 6. godine \u017eivota (Carnes, 2019.).<\/p>\n

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Patofiziologija<\/h2>\n
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Chiari<\/em> malformacije spadaju u skupinu poreme\u0107aja koji zahva\u0107aju mo\u017edano deblo, mali mozak i le\u0111nu mo\u017edinu kod ljudi, a sli\u010dno stanje opisano je i kod pasa te nazvano \u201eChiari-like<\/em>\u201c malformacija (CLM) i siringomijelija (De Lahunta i sur., 2015.a,b). To je kompleksni poreme\u0107aj lubanje i kraniocervikalnih spojeva koji se naj\u010de\u0161\u0107e javlja kod pasa s brahicefali\u010dnim tipovima lubanja (Rushbridge, 2018.). U normalnim uvjetima cerebrospinalni likvor cirkulira iz ventrikularnog sustava u subarahnoidni prostor. Svaka opstrukcija normalnog protoka mo\u017ee dovesti do siringomijelije (Loughin, 2015.). Baza lubanje je prvi dio lubanje koji se razvija u embrionalnom razvoju. Do pro\u0161irenja baze lubanje dolazi zbog primarnog rasta hrskavice i ekspanzije sinhondroza, odnosno hrskavi\u010dnih spojeva izme\u0111u kostiju. Rast je neravnomjeran i prilago\u0111ava se razvoju mozga (McGeady i sur., 2014.). Okcipitalna i sfenoidna kost se sporije razvijaju, a sinhondroza izme\u0111u njih nakon ro\u0111enja u po\u010detku nije oko\u0161tana te se zatvori do 4. mjeseca starosti kod ve\u0107ine pasa. Dokazano je da sinhondroza kod kavalirskog \u0161panijela kralja Charlesa oko\u0161ta puno ranije nego kod drugih brahicefali\u010dnih pasa.
\nMe\u0111utim, \u010dak do 95% kavalirskih \u0161panijela kralja Charlesa mo\u017ee imati ovu malformaciju bez da ikada razviju klini\u010dke simptome siringomijelije (Gon\u00e7alves, 2011.).
\nKod brahicefali\u010dnih pasa sinhondroza oko\u0161ta tako\u0111er prije u odnosu na ostale pse (Knowler i sur., 2014.). Na slikama 1. i 2. prikazana je razlika izme\u0111u okcipitalne regije (mo\u017edanog debla) kod zdravog psa i kod psa s CLM-om.<\/p>\n

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Slika 1<\/strong>. Prikaz okcipitalne regije (mo\u017edanog debla) kod zdravog pasa (Izvor: A. \u0160abanovi\u0107).<\/figcaption><\/figure>\n
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Slika 2<\/strong>. Prikaz okcipitalne regije (mo\u017edanog debla) kod psa s \u201eChiari-like<\/em>\u201c malformacijom (Izvor: A. \u0160abanovi\u0107).<\/figcaption><\/figure>\n

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Klini\u010dki znaci<\/h2>\n
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Klini\u010dki znaci koji se javljaju mogu biti vezani uz neuropatsku bol, bolnost u vratu, mo\u017edano deblo, mali mozak i vestibularnu disfunkciju. Kod ljudi (Hechler i Moore, 2018.) je naj\u010de\u0161\u0107i simptom bol koja se manifestira na razne na\u010dine (kao glavobolja, bol u ekstremitetima i me\u0111ulopati\u010dnom podru\u010dju, poja\u010dana osjetljivost na dodir, trigeminalna bol i sl.). Kod pasa vlasnici opisuju promjene u pona\u0161anju koje mogu ukazivati na neuropatsku bol koja se mo\u017ee manifestirati parestezijom (poreme\u0107eni osjet) ili alodinijom (bol evocirana dodirom) \u0161to su naj\u010de\u0161\u0107e manifestacije neuropatske boli kod ljudi. Me\u0111utim, vlasnici tako\u0111er \u010desto prijavljuju da psi spavaju s glavom na povi\u0161enom mjestu (Loughin, 2015.). \u010cesti klini\u010dki simptom zbog kojeg vlasnici potra\u017ee veterinarsku pomo\u0107 je fantomski svrbe\u017e. Me\u0111utim, s obzirom da psi koji naj\u010de\u0161\u0107e boluju od CLM-a imaju \u010deste upale u\u0161iju i druge dermatolo\u0161ke bolesti potrebno je diferencijalno dijagnosti\u010dki isklju\u010diti pravi pruritus. Mogu se javiti i \u010de\u0161anje o razne predmete ili pod, nepodno\u0161enje tjelesne aktivnosti i nepodno\u0161enje no\u0161enja ogrlice (Hechler i Moore, 2018.).
\nOd neurolo\u0161kih simptoma mogu se javiti deficiti kranijalnih \u017eivaca (naj\u010de\u0161\u0107e facijalnog \u017eivca), vestibularni sindrom, epilepsijski napadaji, deficiti propriocepcije, tremor glave, atrofija temporalnog mi\u0161i\u0107a i multifokalni znakovi sredi\u0161njeg \u017eiv\u010danog sustava. Kao posljedica SM-a mo\u017ee se javiti skolioza \u0161to je posljedica o\u0161te\u0107enja donjih motornih neurona (Dewey i Costa, 2016., Sanders, 2016.). Graham i sur. (2012.) opisali su samo jedan slu\u010daj disfagije kod CLM-a kod pasa \u0161to je ina\u010de \u010dest simptom kod ljudi.<\/p>\n

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Dijagnostika<\/h2>\n
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Dijagnoza CLM-a i SM-a se temelji na magnetskoj rezonanci (MRI) mozga i produ\u017eene mo\u017edine (Knowler i sur., 2014.).
\nNa MRI-u se mogu uo\u010diti kaudalna cerebelarna hernija, kompresija okcipitalne displazije, ventrikulomegalija\/hidrocefalus i SM. Vrlo \u010desti nalaz je primarni otitis media<\/em> koji je prikazan na MRI-u na slici 6.<\/p>\n

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Slika 6<\/strong>. Otitis media<\/em> (Ljubazno\u0161\u0107u Martina Drinkovi\u0107a, dr. med. spec. klini\u010dke radiologije).<\/figcaption><\/figure>\n

SM se mo\u017ee uo\u010diti na jednom ili na vi\u0161e mjesta (Loughin, 2016.). SM je prikazan na MRI-u na slici 4.<\/p>\n

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Slika 4<\/strong>. Cervikalna siringohidromijelija (Ljubazno\u0161\u0107u Martina Drinkovi\u0107a, dr. med. spec. klini\u010dke radiologije).<\/figcaption><\/figure>\n

Loderstedt i sur, (2011.) opisali su C1-C4 i T2-L2 kao predilekcijska mjesta za stvaranje SM-a, ali ipak se preporu\u010duje snimanje cijele kralje\u017enice.<\/p>\n

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Slika 3<\/strong>. Asimetri\u010dna lateralna ventrikulomegalija (Ljubazno\u0161\u0107u Martina Drinkovi\u0107a, dr. med. spec. klini\u010dke radiologije).<\/figcaption><\/figure>\n

Na slici 3. vidljiva je asimetri\u010dna lateralna ventrikulomegalija dok je na slici 5. vidljiva hernijacija kroz foramen magnum<\/em>.<\/p>\n

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Slika 5<\/strong>. Cerebralna hernijacija kroz foramen magnum<\/em> (Ljubazno\u0161\u0107u Martina Drinkovi\u0107a, dr. med. spec. klini\u010dke radiologije).<\/figcaption><\/figure>\n

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Lije\u010denje<\/h2>\n
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Lije\u010denje se bazira na ubla\u017eavanju neuropatske boli i na redukciji proizvodnje cerebrospinalnog likvora (Plessas i sur., 2015.). Najbolji pristup lije\u010denju neuropatske boli je multimodalna terapija (Stalin, 2015.). U tabeli 1. prikazani su naju\u010dinkovitiji lijekovi kod CLM-a.<\/p>\n

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Tabela 1<\/strong>. Naj\u010de\u0161\u0107i lijekovi koji se koriste kod CLM-a (Carnes, 2019., Hechler i Moore, 2018.).<\/figcaption><\/figure>\n

Gabapentin i topiramat su lijekovi izbora za neuropatsku bol kod ljudi. Gabapentin se u veterinarskoj medicini koristi svakodnevno dok se topiramat i dalje ne koristi \u010desto iako je poznat njegov utjecaj na smanjeno stvaranje cerebrospinalnog likvora \u0161to je pogodno kod lije\u010denja siringomijelije (Plessas i sur., 2015.). Stalin (2015.) opisuje bolji u\u010dinak gabapentina od topiramata kod neuropatske boli.<\/p>\n

Tricikli\u010dni antidepresanti (amitriptilin) su se pokazali u\u010dinkoviti kod lije\u010denja neuropatske boli u ljudi. U veterinarskoj medicini ne postoje istra\u017eivanja provedena na amitriptilinu i njegovom utjecaju na neuropatsku bol, ve\u0107 se na osnovi istra\u017eivanja iz humane medicine koristi amitriptilin koji polu\u010duje dobre rezultate (Hechler i Moore, 2018.). N-metil-D-aspartat (NMDA) receptor antagonisti (amantadin) se koriste za lije\u010denje neuropatske boli i boli povezane s osteoartritisom u humanoj medicini.<\/p>\n

Smatra se da NMDA receptori imaju utjecaj kod alodinije pa se amantadin koristi kao antagonist koji smanjuje osjetljivost na bol (Collins i sur., 2010.). Nema dovoljno istra\u017eivanja u veterinarskoj medicini o utjecaju amantadina na neuropatsku bol, ali smatra se da u kombinaciji s opiodima, nesteroidnim protuupalnim lijekovima (NSPUL) i gabapentinom pouspje\u0161uje njihov u\u010dinak (Moore, 2016.).
\nLascelles i sur. (2008.) opisali su pozitivan u\u010dinak amantadina na bol kod osteoartritisa u pasa. Iako se opioidi koriste za kroni\u010dnu bol u ljudi, njihovo kori\u0161tenje kroz du\u017ei vremenski period u veterinarskoj medicini je kontroverzno jer ne postoji dovoljno istra\u017eivanja. Prema Hechler i Moore (2018.) tramadol nije indiciran za lije\u010denje CLM-a kod ve\u0107ine pacijenata.
\nOmeprazol, inhibitor protonske pumpe, smanjuje proizvodnju likvora redukcijom NA-K protonske pumpe u koroidnom pleksusu posljedi\u010dno \u010demu se smanjuje pritisak u le\u0111noj mo\u017edini te se usporava stvaranje syrinksa<\/em> i progresija simptoma (Lindvall-Axelsson i sur., 1992.). NSPUL se \u010desto propisuju kod CLM-a iako su istra\u017eivanja pokazala da nemaju utjecaj na pobolj\u0161anje simptoma jer COX inhibitori nemaju utjecaj na neuropatsku bol kod CLM-a zbog kroniciteta (Park i sur., 2009.).<\/p>\n

Kortikosteroidi su u\u010dinkovitiji jer inhibiraju COX-2 i fosfolipazu A2 \u0161to sprje\u010dava otpu\u0161tanje medijatora upale te utje\u010de na smanjenje proizvodnje likvora, no obzirom na nuspojave, preporu\u010da ih se koristiti samo ukoliko pacijent ne reagira na druge lijekove (Rushbridge i sur., 2000.). Prema Rushbridge (2018.) prvi lijekovi izbora su NSPUL i gabapentin, iako (Park i sur., 2009.) navode da NSPUL nemaju utjecaj na pobolj\u0161anje simptoma.
\nRushbridge (2018.) preporu\u010da uvo\u0111enje pregabalina i kortikosteroida, a potom i amantadin kao zadnju mogu\u0107nost konzervativnog lije\u010denja, prije kirur\u0161kog zahvata ako pacijent i nakon uvo\u0111enja tih lijekova pokazuje znakove bolnosti.
\nAko i nakon terapije, \u017eivotinja ima progresiju klini\u010dkih znakova preporu\u010da se kirur\u0161ki zahvat dekompresije foramen magnum<\/em>-a uz okcipitalnu kraniotomiju s dorzalnom laminektomijom prvog vratnog kralje\u0161ka (Dewey i sur., 2005.) koja je prikazana na slici 7. Pokazalo se da kirur\u0161ka dekompresija ubla\u017eava simptome kod 80% pasa (Loughin, 2015.).<\/p>\n

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Slika 7<\/strong>. (A) prikaz regije okcipitalne kosti i dorzalnog pogleda na C1 kralje\u017eak koji se mi\u010de zbog dekompresije foramen magnum<\/em>-a, (B) prikaz dekompresije foramen magnum<\/em>-a (Izvor: A. \u0160abanovi\u0107).<\/figcaption><\/figure>\n

Plessas i sur. (2012.) proveli su istra\u017eivanje na 48 pasa koji su imali CLM sa ili bez SM-a pri \u010demu je zabilje\u017eena progresiju simptoma u 75 % pasa. Me\u0111utim 75 % od tih pasa je i dalje imalo zadovoljavaju\u0107u kvalitetu \u017eivota dok je 15 % pasa eutanazirano zbog progresije klini\u010dkih simptoma.<\/p>\n

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Zahvala<\/h2>\n
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Zahvaljujem Alenu \u0160abanovi\u0107u na ustupanju crte\u017ea za ovaj rad. Isto tako, zahvaljujem Martinu Drinkovi\u0107u, dr. med. spec. klini\u010dke radiologije na ustupanju slika magnetske rezonance.<\/p>\n

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\nLiteratura<\/strong> [… prika\u017ei]<\/span><\/a><\/span><\/p>\n

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\n<\/em><\/p>\n<\/div>\n

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Chiari-like malformation<\/h2>\n
\n
Elizabeta PONGRAC<\/strong>, DVM, Anja RAI\u0106<\/strong>, DVM, Luka E\u0106IMOVI\u0106<\/strong>, DVM, Ivana KI\u0160<\/strong>, DVM, PhD, Associate Professor, Ma\u0161a EFENDI\u0106<\/strong>, DVM, Assistant, Mirna BRKLJA\u010cI\u0106<\/strong>, DVM, PhD, Associate Professor, Faculty of Veterinary Medicine University of Zagreb, Croatia<\/div>\n
\n

Chiari-like malformation (CLM) is a hereditary defect characterised by a conformational change that can lead to herniation of the cerebellum through the foramen magnum<\/em>. CLM can result in the development of syringomyelia, a condition associated with the filling of spinal cord cavities with fluid. CLM has the most often been described in the Cavalier King Charles Spaniel and occurs most often from the third to the sixth year of life. Magnetic resonance imaging (MRI) is necessary for its diagnosis. Clinical symptoms include the neurological system and one of the most common symptoms is neuropathic pain that can manifest in different ways. Conservative treatment is always the first choice and only when conservative treatment fails or when clinical symptoms worsen is surgical treatment is recommended. Conservative therapy usually includes drugs that reduces neuropathic pain.<\/p>\n

Key words:<\/strong> chiari-like malformation; syringomyelia; Cavalier King Charles Spaniel; neuropathic pain<\/em><\/p><\/blockquote>\n","protected":false},"excerpt":{"rendered":"

E. Pongrac*, A. Rai\u0107, L. E\u0107imovi\u0107, I. Ki\u0161, M. Efendi\u0107, G. Jurki\u0107 Krsteska i M. Brklja\u010di\u0107 Elizabeta PONGRAC*, dr. med.<\/p>\n","protected":false},"author":8,"featured_media":0,"menu_order":5,"comment_status":"closed","ping_status":"open","template":"","format":"standard","meta":{"footnotes":""},"categories":[28],"tags":[2181,2179,2182,2180],"issuem_issue":[2128],"ppma_author":[2176,2177,2178,141,968,959,708],"class_list":["post-7101","article","type-article","status-publish","format-standard","hentry","category-review-articles","tag-cavalier-king-charles-spaniel","tag-chiari-like-malformation","tag-neuropathic-pain","tag-syringomyelia","issuem_issue-veterinarska-stanica-55-4"],"yoast_head":"\n"Chiari-like" malformacija - CROATIAN VETERINARY JOURNAL<\/title>\n<meta name=\"description\" content=\"Chiari like malformation (CLM) is a hereditary defect characterised by a conformational change that can lead to herniation of the cerebellum through the foramen magnum.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/journal.h3s.org\/?article=chiari-like-malformacija\" \/>\n<meta property=\"og:locale\" content=\"en_GB\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\""Chiari-like" malformacija - 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