. "Die konzentrischen Sklerose Bal\u00F3 ist eine seltene akut-entz\u00FCndliche Entmarkungserkrankung des zentralen Nervensystems (ZNS). Sie wird synonym auch als Bal\u00F3-Krankheit (lat. Morbus Bal\u00F3), Bal\u00F3s konzentrische Sklerose oder als Encephalitis periaxialis concentrica bezeichnet. Der Name der Erkrankung geht auf die Erstbeschreibung des ungarischen Pathologen im Jahr 1928 zur\u00FCck. Charakteristisch f\u00FCr die Erkrankung sind die L\u00E4sionen in der wei\u00DFen Substanz. Diese sind gekennzeichnet durch ringf\u00F6rmige, wechselnd st\u00E4rker und weniger stark entmarkte wei\u00DFe Substanz. Dieser Aufbau der L\u00E4sion ergibt in der Bildgebung mittels Magnetresonanztomografie das Bild schie\u00DFscheibenartiger L\u00E4sionen. Anders als bei der klassischen Multiple Sklerose (MS) finden sich bei den meisten Patienten mit konzentrischer Sklerose keine liquorspezifischen oligoklonalen Banden (ca. 34 %; im Vergleich zu >=98 % bei MS). Die Erkrankung ist durch einen akuten und oft monophasischen Verlauf gekennzeichnet. Die auftretenden Symptome sind abh\u00E4ngig von der betroffenen Hirnregion. Betroffen sind vor allem junge Erwachsene. Vor dem Einsatz der heutigen MRT-Technik konnte die Diagnose nur postmortem durch histologische Untersuchungen gestellt werden; in den damals beschriebenen F\u00E4llen kam es meist innerhalb weniger Wochen bis Monate zum Tod. Dank fr\u00FCherer Diagnose anhand der charakteristischen Bal\u00F3-L\u00E4sionen im MRT-Bild wurden im letzten Jahrzehnt h\u00E4ufiger auch F\u00E4lle mit milden Verl\u00E4ufen beschrieben und wurde eine fr\u00FChere Behandlung mit dann oft gutem Outcome m\u00F6glich. Wie bei der multiplen Sklerose wird zun\u00E4chst eine Therapie mit hochdosierten therapeutischen Glucocorticoiden empfohlen. Allerdings ist die Wirksamkeit bei konzentrischer Sklerose weniger gut belegt. Es gibt einzelne Fallbeschreibungen f\u00FCr eine gute Wirksamkeit von Plasmapherese und Immunsuppressiva wie Mitoxantron. In ca. 50 % der F\u00E4lle sind im MRT-Bild auch typische L\u00E4sionen der Multiplen Sklerose erkennbar. Bei einigen dieser Patienten gleicht der weitere Verlauf einer normalen Multiplen Sklerose und zur Schubprophylaxe werden von einigen \u00C4rzten MS-Medikamente eingesetzt."@de . ""@en . . "Stwardnienie koncentryczne Bal\u00F3 (\u0142ac. sclerosis concentrica, ang. Balo concentric sclerosis) \u2013 rzadki wariant neuropatologiczny stwardnienia rozsianego. Chorob\u0119 opisa\u0142 jako pierwszy w\u0119gierski patolog J\u00F3zsef Bal\u00F3 w 1927 roku. W 1933 roku Julius Hallervorden i Hugo Spatz przedstawili opisy neuropatologii choroby i jako pierwsi zauwa\u017Cyli podobie\u0144stwo uformowania ognisk demielinizacyjnych do figur Liesganga. W 2007 roku przedstawiono model matematyczny podaj\u0105cy mo\u017Cliwy mechanizm tworzenia si\u0119 tych niezwyk\u0142ych zmian."@pl . . . . . . . . . . . . "341.1"^^ . "8723023"^^ . . . "A esclerose conc\u00EAntrica de Bal\u00F3 \u00E9 uma doen\u00E7a rara, pertencente ao grupo das doen\u00E7as desmielinizantes inflamat\u00F3rias, na qual ocorre desmieliniza\u00E7\u00E3o aguda fulminante, cujas les\u00F5es apresentam um padr\u00E3o caracter\u00EDstico de an\u00E9is conc\u00EAntricos alternando entre \u00E1reas s\u00E3s e \u00E1reas afectadas."@pt . . . . . . . . "Balo concentric sclerosis"@en . . "Sclerosi concentrica di Bal\u00F3"@it . . "A esclerose conc\u00EAntrica de Bal\u00F3 \u00E9 uma doen\u00E7a rara, pertencente ao grupo das doen\u00E7as desmielinizantes inflamat\u00F3rias, na qual ocorre desmieliniza\u00E7\u00E3o aguda fulminante, cujas les\u00F5es apresentam um padr\u00E3o caracter\u00EDstico de an\u00E9is conc\u00EAntricos alternando entre \u00E1reas s\u00E3s e \u00E1reas afectadas."@pt . . "Bal\u00F3's concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. It was described by J\u00F3zsef M\u00E1ty\u00E1s Bal\u00F3 who initially named it \"leuko-encephalitis periaxialis concentrica\" from the previous definition, and it is currently considered one of the borderline forms of multiple sclerosis. Bal\u00F3's concentric sclerosis is a demyelinating disease similar to standard multiple sclerosis, but with the particularity that the demyelinated tissues form concentric layers. Scientists used to believe that the prognosis was similar to Marburg multiple sclerosis, but now they know that patients can survive, or even have spontaneous remission and asymptomatic cases. The concentric ring appearance is not specific to Bal\u00F3's MS. Concentric lesions have also been reported in patients with neuromyelitis optica, standard MS, progressive multifocal leukoencephalopathy, cerebral autosomal dominant arteriopathy with subcortical infarcts, leukoencephalopathy, concomitant active hepatitis C and human herpes virus 6."@en . "341.1" . . . . "Enfermedad de Bal\u00F3"@es . . "11849"^^ . . . . . . . . . "(Bal\u00F3ova) koncentrick\u00E1 skler\u00F3za je jednou z hrani\u010Dn\u00EDch forem roztrou\u0161en\u00E9 skler\u00F3zy (RS). Jedn\u00E1 se o demyeliniza\u010Dn\u00ED onemocn\u011Bn\u00ED podobn\u00E9 klasick\u00E9 RS, kter\u00E9 se v\u0161ak li\u0161\u00ED v tom, \u017Ee demyelinizovan\u00E9 tk\u00E1n\u011B tvo\u0159\u00ED soust\u0159edn\u00E9 (koncentrick\u00E9) vrstvy. D\u0159\u00EDve se l\u00E9ka\u0159i domn\u00EDvali, \u017Ee u Bal\u00F3ovy koncentrick\u00E9 skler\u00F3zy je progn\u00F3za podobn\u00E1 Margbursk\u00E9 roztrou\u0161en\u00E9 skler\u00F3ze, ale dnes jsou zn\u00E1my p\u0159\u00EDpady, kdy pacienti s touto nemoc\u00ED p\u0159e\u017Eili, \u010Di takov\u00E9 p\u0159\u00EDpady, kdy u nich dokonce nastala spont\u00E1nn\u00ED remise, p\u0159\u00EDpadn\u011B asymptomatick\u00E9 p\u0159\u00EDpady. B\u011B\u017En\u00FDm klinick\u00E9m projevem je prim\u00E1rn\u011B progresivn\u00ED forma RS, v\u0161ak vyskytly se ji\u017E i p\u0159\u00EDpady relaps-remitentn\u00ED formy. Jednou z teori\u00ED l\u00E9\u010Dby je nasazen\u00ED , nicm\u00E9n\u011B pro toto existuj\u00ED pouze neofici\u00E1ln\u00ED d\u016Fkazy a mimo to je tyto z\u00E1v\u011Bry t\u011B\u017Ek\u00E9 p\u0159ijmout, nebo\u0165 existuj\u00ED p\u0159\u00EDpady, kdy se pacienti spont\u00E1nn\u011B uzdravili, a\u0165 ji\u017E u nich byla nasazena l\u00E9\u010Dba steroidy, \u010Di nikoliv."@cs . 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"23391"^^ . . . . . . "G37.5" . "Stwardnienie koncentryczne Bal\u00F3"@pl . . . . . "Typical aspects of Bal\u00F3's concentric sclerosis. Original case of Bal\u00F3; several anastomoses are located in the lower half of the lesion. Lesion centered by a veinule showing ring fragmentation in a constrained area. Lesion. Progress of the pathologic process from a center located in a constrained area, showing formation of bands. Loyez staining ; scale bars: 1 cm."@en . . "Bal\u00F3's concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. It was described by J\u00F3zsef M\u00E1ty\u00E1s Bal\u00F3 who initially named it \"leuko-encephalitis periaxialis concentrica\" from the previous definition, and it is currently considered one of the borderline forms of multiple sclerosis."@en . . . . 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"\u062A\u0635\u0644\u0628 \u0628\u0627\u0644\u0648 \u0627\u0644\u0645\u0631\u0643\u0632"@ar . . "G37.5"@en . . "Esclerose conc\u00EAntrica de Bal\u00F3"@pt . "Bal\u00F3's concentric sclerosis"@en . . . . . . . "Bal\u00F3's concentric sclerosis"@en . "Konzentrische Sklerose Bal\u00F3"@de . . . . . "Koncentrick\u00E1 skler\u00F3za"@cs . . 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"La sclerosi concentrica di Bal\u00F3 \u00E8 uno dei disturbi borderline della sclerosi multipla. Queste patologie sono un insieme di malattie tra cui la Malattia di Devic o neuromielite ottica, la sclerosi maligna di Marburg o sclerosi multipla tumefattiva e la sclerosi mielinoclastica diffusa. \u00C8 una condizione rara e relativamente acuta. Gli attacchi possono procedere rapidamente per settimane o mesi, a volte senza remissione, come la variante di Marburg, con possibili esiti letali o grave disabilit\u00E0. Tuttavia, la maggior parte dei casi ha un decorso pi\u00F9 benigno e autolimitante, con remissione spontanea. Si tratta di una malattia demielinizzante simile alla classica sclerosi multipla, ma con la particolarit\u00E0 che i tessuti demielinizzati formano strati concentrici. I ricercatori ritengono che, sebbene inizialmente la diagnosi venisse fatta unicamente post-mortem, la prognosi sia simile alla sclerosi maligna di Marburg, e si \u00E8 visto che in entrambi i casi i pazienti possono sopravvivere, restare stabili con le terapie pi\u00F9 efficaci attualmente usate per la sclerosi multipla, e ci sono stati casi asintomatici."@it . "1122144407"^^ . "La sclerosi concentrica di Bal\u00F3 \u00E8 uno dei disturbi borderline della sclerosi multipla. Queste patologie sono un insieme di malattie tra cui la Malattia di Devic o neuromielite ottica, la sclerosi maligna di Marburg o sclerosi multipla tumefattiva e la sclerosi mielinoclastica diffusa."@it . . . "D002549"@en . . "11849" . . "La enfermedad de Bal\u00F3 o esclerosis conc\u00E9ntrica de Bal\u00F3 es una enfermedad neurol\u00F3gica rara, caracterizada por la desmielinizaci\u00F3n (p\u00E9rdida r\u00E1pida, y progresiva de la envoltura de mielina, que es la cubierta grasa que rodea las fibras nerviosas) en el cerebro.\u200B Se caracteriza por lesiones conc\u00E9ntricas con grandes olas conc\u00E9ntricas de desmielinizaci\u00F3n y . Las resonancias muestran realce en anillo con Di-s, aumento de la se\u00F1al en FLAIR, lesiones en anillo mediante difusi\u00F3n ponderada de im\u00E1genes, y los cambios relacionados en coeficiente de difusi\u00F3n. Hay p\u00E9rdida de la glicoprote\u00EDna asociada a la mielina y apoptosis de oligodendrocitos. Las \u00E1reas desmielinizadas son ricas en \u00F3xido n\u00EDtrico sintetasa (iNOS). Un acondicionado hip\u00F3xico puede explicar los anillos, donde la hipoxia subletal ofrece resistencia a las lesiones m\u00E1s tarde. Inducible por hipoxia de factor-1-alpha (HIF) se incrementa en el borde exterior de tejido conservado (Stadelmann et al 2005, Normal Appearing White Matter). Esta enfermedad afecta t\u00EDpicamente a ni\u00F1os, aunque algunos casos se han descrito en adultos. Los s\u00EDntomas incluyen par\u00E1lisis progresiva, espasmos involuntarios del m\u00FAsculo, y otros problemas neurol\u00F3gicos. Los s\u00EDntomas de la enfermedad de Balo var\u00EDan seg\u00FAn las \u00E1reas del cerebro que se afecten. Las lesiones desmielinizantes cerebrales se pueden localizar en cualquier parte del cerebro (es decir, cerebro, cerebelo, o tronco cerebral). Las lesiones consisten en placas irregulares de desmielinizaci\u00F3n que se extienden en una serie de c\u00EDrculos conc\u00E9ntricos. Los s\u00EDntomas de la enfermedad de Bal\u00F3 pueden amenazar gravemente la vida, al progresar en unas semanas, o evolucionar m\u00E1s lentamente durante un tiempo de 2 a 3 a\u00F1os."@es . . . . . 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"Stwardnienie koncentryczne Bal\u00F3 (\u0142ac. sclerosis concentrica, ang. Balo concentric sclerosis) \u2013 rzadki wariant neuropatologiczny stwardnienia rozsianego. Chorob\u0119 opisa\u0142 jako pierwszy w\u0119gierski patolog J\u00F3zsef Bal\u00F3 w 1927 roku. W 1933 roku Julius Hallervorden i Hugo Spatz przedstawili opisy neuropatologii choroby i jako pierwsi zauwa\u017Cyli podobie\u0144stwo uformowania ognisk demielinizacyjnych do figur Liesganga. W 2007 roku przedstawiono model matematyczny podaj\u0105cy mo\u017Cliwy mechanizm tworzenia si\u0119 tych niezwyk\u0142ych zmian."@pl . . "(Bal\u00F3ova) koncentrick\u00E1 skler\u00F3za je jednou z hrani\u010Dn\u00EDch forem roztrou\u0161en\u00E9 skler\u00F3zy (RS). Jedn\u00E1 se o demyeliniza\u010Dn\u00ED onemocn\u011Bn\u00ED podobn\u00E9 klasick\u00E9 RS, kter\u00E9 se v\u0161ak li\u0161\u00ED v tom, \u017Ee demyelinizovan\u00E9 tk\u00E1n\u011B tvo\u0159\u00ED soust\u0159edn\u00E9 (koncentrick\u00E9) vrstvy. D\u0159\u00EDve se l\u00E9ka\u0159i domn\u00EDvali, \u017Ee u Bal\u00F3ovy koncentrick\u00E9 skler\u00F3zy je progn\u00F3za podobn\u00E1 Margbursk\u00E9 roztrou\u0161en\u00E9 skler\u00F3ze, ale dnes jsou zn\u00E1my p\u0159\u00EDpady, kdy pacienti s touto nemoc\u00ED p\u0159e\u017Eili, \u010Di takov\u00E9 p\u0159\u00EDpady, kdy u nich dokonce nastala spont\u00E1nn\u00ED remise, p\u0159\u00EDpadn\u011B asymptomatick\u00E9 p\u0159\u00EDpady."@cs . "La enfermedad de Bal\u00F3 o esclerosis conc\u00E9ntrica de Bal\u00F3 es una enfermedad neurol\u00F3gica rara, caracterizada por la desmielinizaci\u00F3n (p\u00E9rdida r\u00E1pida, y progresiva de la envoltura de mielina, que es la cubierta grasa que rodea las fibras nerviosas) en el cerebro.\u200B Un acondicionado hip\u00F3xico puede explicar los anillos, donde la hipoxia subletal ofrece resistencia a las lesiones m\u00E1s tarde. Inducible por hipoxia de factor-1-alpha (HIF) se incrementa en el borde exterior de tejido conservado (Stadelmann et al 2005, Normal Appearing White Matter)."@es . . . "\u041A\u043E\u043D\u0446\u0435\u043D\u0442\u0440\u0438\u0447\u0435\u0441\u043A\u0438\u0439 \u0441\u043A\u043B\u0435\u0440\u043E\u0437 \u0411\u0430\u043B\u043E"@ru . "D002549" . . . "Die konzentrischen Sklerose Bal\u00F3 ist eine seltene akut-entz\u00FCndliche Entmarkungserkrankung des zentralen Nervensystems (ZNS). Sie wird synonym auch als Bal\u00F3-Krankheit (lat. Morbus Bal\u00F3), Bal\u00F3s konzentrische Sklerose oder als Encephalitis periaxialis concentrica bezeichnet. Der Name der Erkrankung geht auf die Erstbeschreibung des ungarischen Pathologen im Jahr 1928 zur\u00FCck."@de . . . .