7 research outputs found
NEURORADIOLOGICAL CHANGES IN NEUROCUTANEOUS DISEASES: II TUBEROUS SCLEROSIS (TS), STURGE ā WEBER (S-W) SYNDROME, HIPPEL-LINDAU (H-L) SYNDROME
Intrakranijalne lezije u TS-u obilježene su nazoÄnoÅ”Äu kortikalnih i subkortikalnih hamartoma, subependimalnih periventrikularnih nodula, giant-cell astrocitoma u subependimalnom podruÄju, radijarnih heterotopija unutar bijele tvari mozga koje se Å”ire od komora prema subkortikalnom podruÄju. Kortikalni i subkortikalni hamartomi mogu biti multipli, ali i solitarni, najÄeÅ”Äe lokalizirani u caput nuclei caudati te uz postraniÄne komore. Mogu degenerirati u giant-cell astrocitome. CT znaci TS-a na nativnim presjecima su tipiÄne multiple toÄkaste kalcifikacije u subependimalnom periventrikularnom podruÄju bez imbibicije nakon primjene kontrastnog sredstva. PostraniÄne komore su Äesto neÅ”to Å”ire, a takoÄer i sulkusi na konveksitetu. U parenhimu se mogu naÄi hipodenzne ili ÄeÅ”Äe hiperdenzne lezije koje ne djeluju kompresivno. Hipodenzne lezije se ne imbibiraju, dok se hiperdenzne mogu eventualno blago imbibirati nakon aplikacije kontrastnog sredstva. MRI znaci hamartoma su hipointezivne do izointenzivne lezije na T1 i hiperintenzivne na T2-mjerenoj slici.NajÄeÅ”Äe se nalaze u frontalnom podruÄju ili u cerebelumu i mogu se imbibirati nakon primjene kontrasta. Subependimalni noduli se ili ne pojaÄavaju ili se razliÄito imbibiraju kontrastom.
KlasiÄne neuroradioloÅ”ke promjene u S-W sindromu su fuziformne, tubularne i serpiginozne kalcifikacije koje zahvaÄaju korteks mozga, obiÄno unilateralno okcipitalni ili okcipito-parijetalni režanj, iako mogu biti zahvaÄene i obje hemisfere. ObiÄno je prisutna i hemiatrofija. Na CT-u kalcifikacije su vidljive i na nativnim presjecima, a nakon primjene kontrasta dolazi do njihove imbibicije zbog pialne angiomatoze. Na MRI-u kalcifikacije su vidljive kao hipointenzivne promjene na svim sekvencama. GRE sekvence su osobito osjetljive na detekciju sitnih kalcifikata. Atrofija mozga je znaÄajna. Leptomeninge su zadebljane s izrazitom imbibicijom. Bijela tvar može imati poviÅ”en signal na T2 i FLAIR- slici zbog prisutne glioze.
TipiÄna promjena SŽS-a u H-L sindromu je hemangioblastom koji se prikazuje kao cistiÄni nodus, lokaliziran u cerebelumu, meduli oblongata i meduli spinalis. Metoda izbora u dijagnozi hemangioblastoma je MRI .Muralni nodus je na T1 izointenzivan ili hipointenzivan , a na T2-mjerenoj slici hiperintenzivan. Nakon kontrasta nodul se intenzivno imbibira, a rubovi ciste se mogu blago imbirati ili se uopÄe ne imbibiraju.U 20% -40% sluÄajeva hemangiblastom je solidna lezija koja se izrazito imbibira. CT znaci - infratentorijalno smjeÅ”ten hiperdenzan nodus na nativnom skenu koji se izrazito dobro imbibira nakon primjene kontrasta, uz uvijek nazoÄnu oÅ”tro demarkiranu hipodenznu leziju koja se ne imbibira.Intracranial lesions in TS are characterized by the presence of cortical and subcortical hamartomas, subependimal periventricul nodules, giant-cell astrocitomas in subependimal areas, radial heterotopias inside the white matter of brain spreading from ventricles towards the subcortical area. Cortical and subcortical hamartomas can be multiple, but also solitary, frequently localized in the caput nuclei caudati and near the lateral ventricles. They can degenerate into giant-cell astrocitomas. CT signs of TS on native slices are typical multiple punctiform calcifications in the subependimal periventricular area without imbibition after the application of contrast. Lateral ventricles are often wider, as are the sulci on convexity. In the parenchyma there are hypo or hyperdense lesions with no compressive appearance. Hypodense lesions do not have imbibition, while hyperdense lesions can possibly have slight imbibition after contrast. MRI signs of hamartomas are hypo or isointensive lesions on T1 and hyperintensive on T2-weighted imaging. They are mostly localized in the frontal area or in the cerebellum and they can have imbibition after contrast. Subependimal nodules can have diverse imbibition after contrast.
Classical neuroradiological changes in S-W syndrome are fusiform, tubular and serpentine calcifications that affect the brain cortex, usually unilateral occipital or occipito-parietal lobe, although both hemispheres can be affected. Usually there is hemyatrophy. On CT, the calcifications are also visible on the native images, and after the application of contrast there is imbibition because of pial angiomatosis. On MRI, the calcifications are visible as hypointensive changes on all sequences. GRE sequences are particularly sensitive to the detection of tiny calcifications. Brain atrophy is significant. Leptomeningas are thickened with expressed imbibition. White matter can have high signal on T2-weighted and FLAIR images because of the presence of gliosis.
A typical change of CNS in H-L syndrome is hemangioblastoma that presents itself as a cystic nodule localized in the cerebellum, medulla oblongata and medulla spinalis. MRI is the method of choice in diagnosis of hemangioblastoma. The mural nodule on T1 is iso- or hypointensive and on T2-weighted image it is hyperintensive. After the contrast, the nodule has intensive imbibition and the edges of the cyst can have slight imbibition or no imbibition at all. In 20% -40% of cases, the haemangioblastoma is a solid lesion with marked imbibition. CT signs ā the hyperdense nodule with intratentorial localization on the native scan that has markedly good imbibition after contrast and always with a sharply limited hypodense lesion without imbibition
NEURORADIOLOGICAL CHANGES IN NEUROCUTANEOUS DISEASES: I. NEUROFIBROMATOSIS
U neuroradioloÅ”koj procjeni brojnih neurokutanih sindroma (fakomatoza) danas se rutinski primjenjuje kompjutorizirana tomografija (CT) i magnetska rezonancija (MRI). Od neoplastiÄkih lezija u neurofibromatozi tip 1 (NF1) najÄeÅ”Äi su gliomi optiÄkog živca, drugi gliomi mozga i produljene moždine, pleksiformni neurofibromi i neurofibrosarkom. NeneoplastiÄke lezije su hiperplastiÄna ili displastiÄna glialna proliferacija - hamartomi, heterotopije i podruÄja dismijelinizacije. CT znaci optiÄkih glioma su hiperdenzne vretenaste tvorbe vezane za optiÄki živac, od kojeg se ne mogu izdiferencirati, s blagom imbibicijom nakon primjene kontrasta i bez kompresivnog uÄinka. Ako su zahvaÄeni hijazma, hipotalamus, talamus, bazalni gangliji, kompresivni uÄinak može biti nazoÄan dajuÄi sliku pravih tumora, a imbibicija može biti jaÄe izražena. Na nativnoj slici ovi tumori su hiperdenzni. MRI daje poviÅ”en signal nodoznog izgleda u gore navedenim lokalizacijama u T2 slici i FLAIR tehnici te hipointenzivnog ili isointenzivnog signala na T1. Hamartomi se vide kao poviÅ”en signal na T2 i mogu biti blago poviÅ”enog signala na T1 bez znakova kompresije.. U cca 43%-93% bolesnika s NF-om1 nalaze se na MRI-u tzv. UBO lezije ( od engl. Unidentified Bright Object) -. hiperinetenzivni signali u FLAIR i T2- mjerenoj slici iskljuÄivo u bijeloj tvari bez imbibicije nakon kontrasta.
AkustiÄki neurinomi u sklopu neurofibromatoze tip 2 (NF2) pokazuju iste CT znaÄajke kao i neurinomi u bilo kojoj drugoj lokalizaciji. Na MRI slici vestibularni neurinomi su hipointenzivni ili izointenzivni na T1 i hiperintenzivni na T2-mjerenoj slici. Heterogeno se imbibiraju zbog prisutnih cistiÄnih i degenerativnih promjena unutar tumora i po tome ih lako razlikujemo od meningeoma koji pokazuju jednoliÄnu imbibiciju. Meningeomi mogu imati kalcifikacije ili prominentne krvne žile te zadebljanje dure koji su Äest znak meningeoma. Oni su izointenzivni sa sivom tvari mozga na T2 i blago hipointenzivni na T1-mjerenoj slici MRI mozga.In current neurological evaluation of numerous neurocutaneous syndromes (facomatosis) there is routine utilization of computerized tomopgraphy (CT) and magnetic resonance imaging (MRI). From neoplastic lesions in neurofibromatosis type I (NF1) the most common are optic nerve gliomas, other gliomas of the brain and medula oblongata, plexiform neurofibromas and neurofibrosarcomas. Non-neoplastic lesions are hyperplastic or dysplastic glial proliferation ā hamartomas, heterotopias and areas of dysmyelinization. CT signs of optic gliomas are hyperdense spindle-shaped masses attached to the optic nerve, from which they cannot be distinguished, with slight imbibition of contrast and without compression signs. When there is involvement of the hiasma, hypotalamus, thalamus or basal ganglia, the compressive effect can be evident, giving us the impression of real tumors and imbibition can be also accentuated. On native imaging these tumors are hyperdense. MRI reveals a high signal of nodosal appearance in the above mentioned localizations in T2 and FLAIR imaging and hypo or isointensive signal on T1. Hamartomas can be seen as higher signals on T2, slightly higher signals on T1 without signs of compression. In about 43%-93% of patients with NF1 there are so called UBO (Unidentified Bright Objects) lesions on MRI- hyperintensive signals on FLAIR and T2 weighted pictures, exclusively in the white matter without imbibition after contract.
Acustic neurinomas in neurofibromatosis type 2 (NF2) show the same features on CT as neurinomas in any other localization. Vestibular neurinomas on MRI are hypo or isointensive on T1 and hyperintensive on T2-weighted pictures. Heterogenous imbibition is present because of cystic and degenerative changes inside the tumor and they can be easily distinguished from meningeomas showing homogenous imbibition. Meningeomas can have calcifications or prominent blood vessels and dura thickening, as common signs of meningeomas. They are isointensive with the grey matter of the brain on T2 and slightly hypointensive on T1-weighted brain MRI
Blaži kognitivni poremeÄaj kao kliniÄka manifestacija neurosarkoidoze lijevka hipofize: prikaz sluÄaja
A case is presented of a 59-year-old male patient with a 5-year history of sarcoidosis. In the last half a year, deterioration of his intellectual abilities was noticed. Psychological testing detected a mild cognitive disorder. Laboratory diagnostics found a decreased level of testosterone and magnetic resonance imaging showed pituitary stalk neurosarcoidosis without any other pathomorphological substrate of cognitive impairment. This case indicates that neurosarcoidosis should be considered as a possible cause of mild cognitive disorder and, consequently, included in the International Classification of Mental and Behavioural Disorders.Prikazuje se sluÄaj 59-godiÅ”njeg bolesnika s 5-godiÅ”njom povijeÅ”Äu bolesti sarkoidoze. U posljednjih pola godine primjetno je bilo slabljenje njegovih intelektualnih sposobnosti. PsiholoÅ”kim testiranjem otkriven je blaži spoznajni poremeÄaj. Laboratorijskom dijagnostikom utvrÄena je snižena razina testosterona, dok je magnetska rezonancija pokazala neurosarkoidozu lijevka hipofize bez ikakvog drugog patomorfoloÅ”kog supstrata kognitivnog poremeÄaja. Ovaj sluÄaj pokazuje da neurosarkoidozu treba uzeti u obzir kao moguÄi uzrok blažeg spoznajnog poremeÄaja, pa bi je trebalo uvrstiti u MeÄunarodnu klasifikaciju psihiÄkih bolesti i bolesti ponaÅ”anja
Hemangioblastoma of the cerebellum : the correlative pathology with the computed tomography
U radu je morfoloÅ”ki prikazano 28 sluÄajeva hemangioblastoma malog mozga, koji su analizirani metodom kompjutorizirane tomografije na nativnim presjecima, kao i nakon aplikacije kontrastnog sredstva, u svim sluÄajevima uz primjenu kontrastnog sredstva tijekom pregleda. Analiziran je smjeÅ”Ā taj muralnog nodusa i cistiÄnog dijela tumora u odnosu na tkivo malog mozga. Kompjutoriziranom tomografijom utvrÄene su morfoloÅ”ke kvalitete stijenke tumorske Å”upljine i solidnog dijela tumora, a u odnosu na neke druge tumore. Pojedini dijelovi tumorskog tkiva potvrÄeni su patohistoloÅ”ki. NaglaĀ Å”ena je važnost moguÄnosti vizualizacije morfoloÅ”Ā kih tkivnih osobitosti metodom kompjutorizirane tomografije.Cerebral hemangioblastoma has been morphologically presented in 28 cases. The method of analysis was the computed thomography on native crossections before and after application of contrast, and with the application of contrast during the examination in all cases. The position of mural nodus and cystic part of the tumor in relation to the tissue of cerebellum has been analyzed. The morphologic qualities of the tumor paries and the solid part of the tumor were established, also in comparison to some other tumors. Some parts, of the tumor tissue were pathohistologically verified. The importance of the possibility of visualisation of the morphologic tissue specificities by the computed tomography is pointed out
Neuroimaging in brain development malformations EUROIMAGING IN BRAIN DEVELOPMENT MALFORMATIONS
Prikazani su rezultati studije ocjene vrijednosti ultrazvuka, kompjutorizirane tomografije i magnetne rezonancije u dijagostificiranju poremeÄaja prouzroÄenih anomalijama u razvoju mozga. Studijom je obuhvaÄeno 70 djece s cerebralnim anomalijama dijagnosticiranim primjenom UZV, CT i MR u razdoblju od 1984. do 1995. godine, koja su kliniÄki obraÄivana u Klinici za djeÄje bolesti Zagreb. U radu je primijenjena modificirana klasifikacija anomalija mozga prema De Myeru. Navedena klasifikacija polazi od stupnja razvoja u kojem anomalija nastaje, pa govorimo o cerebralnim anomalijama uslijed poremeÄaja citogeneze, histiogeneze ili organogeneze. UZV je osnovna metoda u dijagnostici intrakranijskih anomalija i mora biti primijenjen kao "screening" metoda. S obzirom da znaÄajan broj anomalija mozga može ostati neprepoznat ukoliko se primjenjuje samo UZV, potrebno je, ponekad, primijeniti i CT ili MR, a pogotovo nakon zatvaranja fontanele djeteta. Primjenom kombinacije radioloÅ”kih metoda uz kliniÄke i laboratorijske nalaze, u moguÄnosti smo utvrditi konaÄnu dijagnozu anomalija mozga u velikom broju djece, a Å”to je vrlo znaÄajno s kliniÄkog, prognostiÄkog i genetskog stajaliÅ”ta.The paper presents the results of the study of the value of ultrasound (US), computed tomography (CT) and magnetic resonance (MR) in the diagnostics of brain development malformations, In the period from 1984 to 1995 US, CT and MR examinations were performed on 70 children with cerebral development malformations who were treated at the Children\u27s Hospital in Zagreb. In our study we applied the classification of cerebral malformations by De Myer. This Classification is based on the stage in the foetal development in which malformation occurs: disorders of cytogenesis, disorders of histogenesis or organogenesis. US is the basic imaging method in the diagnostics of cerebral malformations and must be used as a screening method. However, CT and MR, have to be used sometimes especially after the closure of a child\u27s fontanelle, because some of the malformations can be misdiagnosed if US is applied alone. The combination of imaging methods, clinical and laboratory findings enables us to set the definite diagnosis in children with cerebral malformations. This is very important from clinical, genetic and prognostic points of view
Doprinos transkranijske dupleks Dopplerove sonografije dijagnostici stenoze velikih moždanih arterija u djeteta
The contribution of pulsating duplex Doppler ultrasonography to the diagnosis of middle (MCA) and anterior (ACA) cerebral artery obstruction in one patient is reported. A 10year-old boy was admitted to the hospital for pulsating headaches (especially pronounced on physical training). He had no neurologic disabilities. His EEG and brain CT scan were normal, and so were his funduscopic examination, lumbar puncture, and laboratory tests. Transcranial color duplex Doppler ultrasonography showed very high velocities in both ACA and right MCA as a sign of suspected stenosis or spasm. Bilateral subtraction cerebral angiography performed after several months of recurrent headaches and unchanged Doppler ultrasonography findings produced an image of high degree stenosis of A1 segment of both ACA and right MCA, with signs of āsteal syndromeā through the posterior cerebral circulation. MRI performed one year later, after episodes of transient ischemic attacks, showed ischemic infarction in the right temporo-occipital region. The etiology of stenosis was supposed to include vasculopathy, i.e. early stage of moyamoya syndrome. Other vasculopathies were excluded by laboratory tests and clinical elaboration. It is concluded that transcranial Doppler ultrasonography is a very helpful method for detection and follow-up of the degree of stenosis of great cerebral arteries in children, and that it correlates well with cerebral angiography, yet it is not useful in diagnosing the etiology of stenosis.Prikazan je sluÄaj 10-godiÅ”njeg djeÄaka koji je primljen na Kliniku zbog pulsirajuÄih glavobolja koje su se najÄeÅ”Äe javljale za vrijeme tjelesnog napora. DjeÄak je bio urednog somatskog i neuroloÅ”kog statusa. Njegov EEG i CT mozga bili su uredni, kao i pregled oÄnog dna, likvora i laboratorijske pretrage. Transkranijski obojeni dupleks Doppler pokazao je izrazito velike brzine u objema prednjim moždanim arterijama (ACA) i u desnoj srednjoj moždanoj arteriji (MCA), Å”to je moglo odgovarati stenozi krvnih aila. Subtrakcijska cerebralna angiografija uÄinjena je nakon nekoliko mjeseci opetovanih glavobolja i nepromijenjenog Doplerskog nalaza. Pokazala je veÄi stupanj stenoze prednjeg segmenta obiju ACA i poÄetnog dijela desne MCA, sa znacima āsindroma kraÄeā kroz stražnju moždanu cirkulaciju. MRI (uÄinjena godinu dana kasnije, nakon ponavljanih epizoda prolaznih ishemijskih napadaja) pokazala je ishemijski infarkt temporookcipitalno desno. Etiologija bolesti ostala je otvorenom. Pretpostavljeno je da se radi o vaskulopatiji, tj. ranom stadiju bolesti moyamoya. Ostale vaskulopatije iskljuÄene su laboratorijskim i kliniÄkim ispitivanjem. ZakljuÄuje se kako je transkranijski obojeni dupleks Doppler vrlo dobra metoda za otkrivanje i praÄenje stupnja stenoze moždanih arterija u djece i dobro korelira s cerebralnom angiografijom, ali joÅ” ne pomaže u otkrivanju etiologije stenoze