. "I" . "Vyb\u00EDhal, V\u00E1clav" . . "272616" . . . "Lhermitte-Duclos Disease - a Case Report"@en . "Morbus Lhermitte-Duclos - kazuistika" . "Lhermitte-Duclos Disease - a Case Report"@en . . "5" . "Dysplastick\u00FD gangliocytom moze\u010Dku (morbus Lhermitte-Duclos) pat\u0159\u00ED mezi vz\u00E1cn\u00E9 benign\u00ED l\u00E9ze. Podle posledn\u00ED klasifikace WHO z roku 2007 je onemocn\u011Bn\u00ED pova\u017Eov\u00E1no za hamartom nebo n\u00E1dor (WHO gradus I). Na z\u00E1klad\u011B genetick\u00FDch studi\u00ED se \u0159ada autor\u016F v posledn\u00ED dob\u011B p\u0159ikl\u00E1n\u00ED k hamartogenn\u00EDmu p\u016Fvodu. Klinicky se onemocn\u011Bn\u00ED projevuje cerebel\u00E1rn\u00EDmi p\u0159\u00EDznaky, event. p\u0159\u00EDznaky z komprese mozkov\u00E9ho kmene a hlavov\u00FDch nerv\u016F. M\u016F\u017Ee se tak\u00E9 manifestovat syndromem nitrolebn\u00ED hypertenze. Z\u00E1kladn\u00ED zobrazovac\u00ED metoda je magnetick\u00E1 rezonance, na kter\u00E9 lze spat\u0159it typick\u00FD obraz onemocn\u011Bn\u00ED. Dysplastick\u00FD gangliocytom moze\u010Dku m\u016F\u017Ee b\u00FDt sou\u010D\u00E1st\u00ED syndromu Cowdenov\u00E9. Jedn\u00E1 se o autozom\u00E1ln\u011B dominantn\u00ED onemocn\u011Bn\u00ED, jeho\u017E hlavn\u00ED p\u0159\u00ED\u010Dinou je mutace genu PTEN. Jeho charakteristick\u00FDm projevem je v\u00FDskyt mnoho\u010Detn\u00FDch hamartom\u016F a vysok\u00E9 riziko vzniku syst\u00E9mov\u00FDch malignit. Nejefektivn\u011Bj\u0161\u00ED l\u00E9\u010Dba je radik\u00E1ln\u00ED chirurgick\u00E1 resekce, kter\u00E1 ale nemus\u00ED b\u00FDt v\u017Edy mo\u017En\u00E1. Nezbytn\u00E9 je proveden\u00ED genetick\u00E9ho vy\u0161et\u0159en\u00ED k vylou\u010Den\u00ED syndromu Cowdenov\u00E9." . "\u010Cesk\u00E1 a Slovensk\u00E1 Neurologie a Neurochirurgie" . "73" . . . . "Vidl\u00E1k, Milan" . "Fadrus, Pavel" . "Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare benign lesion. According to the the most recent WHO classification (2007), the disease is considered a hamartoma or tumour (WHO grade 1). A number of authors incline to the opinion that it is of hamartogenous origin, basing their view on the results of recent genetic studies. Clinical symptoms of the disease include cerebellar symptoms and signs of compression of the brain stem and cranial nerves, while the disease may also be associated with intracranial hypertension syndrome. Magnetic resonance examination is the best diagnostic imaging technique, since as it reveals a unique pattern for the disorder. Dysplastic gangliocytoma of the cerebellum may be associated with Cowden disease. This is an autosomal dominant condition that results mainly from a mutation of the PTEN gene. Multiple hamartomas are specific to Cowden disease, which is associated with high risk of systemic malignancies."@en . . "Dysplastick\u00FD gangliocytom moze\u010Dku (morbus Lhermitte-Duclos) pat\u0159\u00ED mezi vz\u00E1cn\u00E9 benign\u00ED l\u00E9ze. Podle posledn\u00ED klasifikace WHO z roku 2007 je onemocn\u011Bn\u00ED pova\u017Eov\u00E1no za hamartom nebo n\u00E1dor (WHO gradus I). Na z\u00E1klad\u011B genetick\u00FDch studi\u00ED se \u0159ada autor\u016F v posledn\u00ED dob\u011B p\u0159ikl\u00E1n\u00ED k hamartogenn\u00EDmu p\u016Fvodu. Klinicky se onemocn\u011Bn\u00ED projevuje cerebel\u00E1rn\u00EDmi p\u0159\u00EDznaky, event. p\u0159\u00EDznaky z komprese mozkov\u00E9ho kmene a hlavov\u00FDch nerv\u016F. M\u016F\u017Ee se tak\u00E9 manifestovat syndromem nitrolebn\u00ED hypertenze. Z\u00E1kladn\u00ED zobrazovac\u00ED metoda je magnetick\u00E1 rezonance, na kter\u00E9 lze spat\u0159it typick\u00FD obraz onemocn\u011Bn\u00ED. Dysplastick\u00FD gangliocytom moze\u010Dku m\u016F\u017Ee b\u00FDt sou\u010D\u00E1st\u00ED syndromu Cowdenov\u00E9. Jedn\u00E1 se o autozom\u00E1ln\u011B dominantn\u00ED onemocn\u011Bn\u00ED, jeho\u017E hlavn\u00ED p\u0159\u00ED\u010Dinou je mutace genu PTEN. Jeho charakteristick\u00FDm projevem je v\u00FDskyt mnoho\u010Detn\u00FDch hamartom\u016F a vysok\u00E9 riziko vzniku syst\u00E9mov\u00FDch malignit. Nejefektivn\u011Bj\u0161\u00ED l\u00E9\u010Dba je radik\u00E1ln\u00ED chirurgick\u00E1 resekce, kter\u00E1 ale nemus\u00ED b\u00FDt v\u017Edy mo\u017En\u00E1. Nezbytn\u00E9 je proveden\u00ED genetick\u00E9ho vy\u0161et\u0159en\u00ED k vylou\u010Den\u00ED syndromu Cowdenov\u00E9."@cs . "RIV/00216224:14110/10:00058941" . "6"^^ . "5"^^ . "RIV/00216224:14110/10:00058941!RIV13-MSM-14110___" . "1210-7859" . "000283640800017" . . "Duba, Milo\u0161" . "6"^^ . . "14110" . "[18519751BD0F]" . "Lhermitte-Duclos disease; dysplastic gangliocytoma of the cerebellum; Cowden syndrome"@en . . "CZ - \u010Cesk\u00E1 republika" . "Morbus Lhermitte-Duclos - kazuistika" . . . . . . "\u0160prl\u00E1kov\u00E1-Pukov\u00E1, Andrea" . "Morbus Lhermitte-Duclos - kazuistika"@cs . . . "Morbus Lhermitte-Duclos - kazuistika"@cs . "K\u0159en, Leo\u0161" .