Longitudinal EEG Studies in a Kindred with Lafora Disease
dc.contributor.author | Yen, C. | en_US |
dc.contributor.author | Beydoun, Ahmad A. | en_US |
dc.contributor.author | Drury, Ivo | en_US |
dc.date.accessioned | 2010-04-01T14:58:41Z | |
dc.date.available | 2010-04-01T14:58:41Z | |
dc.date.issued | 1991-12 | en_US |
dc.identifier.citation | Yen, C.; Beydoun, A.; Drury, I. (1991). "Longitudinal EEG Studies in a Kindred with Lafora Disease." Epilepsia 32(6): 895-899. <http://hdl.handle.net/2027.42/65436> | en_US |
dc.identifier.issn | 0013-9580 | en_US |
dc.identifier.issn | 1528-1167 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/65436 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=1743163&dopt=citation | en_US |
dc.description.abstract | We reviewed 18 EEG studies in four members of a family with the Lafora form of progressive myoclonic epilepsy. Each patient was the product of a consan-guinous marriage and presented as a teenager with progressive seizures, myoclonus, dementia, and ataxia, and had biopsy proven disease. The EEG early in Lafora disease has spike-wave activity resembling that seen in a primary generalized epilepsy; the background slowing is more typical of a secondary generalized epilepsy. With disease progression, there is increased epileptiform activity, and a striking change in the spike-wave complexes, with a marked increase in frequency up to 6–12 Hz, and many more short duration poly spike components. Unlike some other forms of secondarily generalized epilepsy, the EEG in Lafora disease is distinguished by an increased frequency of the spike-wave complexes with disease progression. RESUME Les auteurs ont revu 18 enregistrements EEG pratiquÉs chez 4 membres d'une famille prÉsentant une maladie de Lafora. Chaque patient Était issu d'un manage consanguin et a prÉsentÉ dans adolescence association progressive de crises, d'un myoclonus, d'une dÉmence et d'une ataxie; le diagnostic de la maladie a ÉtÉ confirmÉ par une biopsie. EEG prÉsente, À la phase initiale de la maladie de Lafora, un aspect comparable À celui de Épilepsie gÉnÉralisÉe idiopathique, mais le ralentissement de activitÉ de fond Évoque davantage EEG des Épilepsies gÉnÉi-alisÉes symptomatiques (EGS). Pendant la progression de la maladie, les auteurs ont constatÉ une augmentation de activitÉÉp-ileptique EEG, avec modification importante de la morphologie des PO, qui ont augmentÉ de frÉquence, jusqu'À 6–12 c/s, avec composantes accrues en Polypointes rapides. Contrairement À d'autres formes d'EGS, EEG de la maladie de Lafora se distingue par une augmentation de frÉquence des PO au cours de Évolution. RESUMEN Se revisan 18 estudios de EEG en 4 miembros de una familia con la forma de Lafora de una epilepsÍa mioclÓnica progresiva. Cada paciente fue producto de un matrimonio consanguÍneo y en su adolescencia presentaron ataques progresivos, mioclonÍas, demencia y ataxia, y se les practiceÓ una biopsia que confirmÓ la enfermedad. Los EEGs practicados precozmente en la enfer-medad de Lafora tienen una actividad de punta-onda que semeja la que se observa en epilepsÍa generalizada primaria y la lentifi-caciÓn de la actividad de fondo es mÁs tÍpica de epilepsÍa generalizada sencundaria. A medida que la enfermedad progresa se observÓ un incremento de la actividad epileptiforme y un cambio sorprendente en los complejos punta-onda con marcado incremento de la frecuencia hasta 6–12 Hz. y muchos mÁs compo-nentes de polipuntas de breve duraciÓn. Contrariamente a otras formas de epilepsÍa generalizada secundaria el EEG en la enfermedad de Lafora se caracteriza por un incremento de la frecuencia de los complejos punta-onda a medida que la enfermedad progresa. ZUSAMMENFASSUNG Wir beurteilten 18 EEG-Untersuchungen bei 4 Mitgliedern einer Familie mit der Lafora-Form der progressiven Myoklonus-Epilepsie. Jeder Patient entstammte einer konsanguinen Ehe und wurde als Teenager mit progressiven AnfÄllen, Myoklonus, De-menz und Ataxie auffÄllig; die Diagnose wurde durch Biopsie bestÄtigt. Das EEG im frÜhen Verlauf einer Lafora-Erkrankung zeigt Spike Wave AktivitÄt, die der bei primÄr generalisierter Epilepsie Ähnelt, wÄhrend die HintergrundaktivitÄt mehr fÜr eine sekundÄr generaliserte Epilepsie typisch ist. Mit fortschreitender Erkrankung tritt vermehrt epileptische AktivitÄt und ein deutli-cher Wechsel der Spike Wave Komplexe mit starkem Ansteigen der Frequenz auf 6–12 Hz und vermehrten kurzdauernden Polyspike-Komponenten auf. Im Gegensatz zu anderen Formen sekundÄr generalisierter Epilepsien zeigt das EEG bei der Lafora-Erkrankung mit Erkrankungsfortgang eine zunehmende Frequenz der Spike Wave Komplexe. | en_US |
dc.format.extent | 460274 bytes | |
dc.format.extent | 3110 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.publisher | Blackwell Publishing Ltd | en_US |
dc.rights | 1991 International League Against Epilepsy | en_US |
dc.subject.other | Lafora Disease | en_US |
dc.subject.other | Seizures | en_US |
dc.subject.other | Electroencephalography | en_US |
dc.subject.other | Genetics | en_US |
dc.title | Longitudinal EEG Studies in a Kindred with Lafora Disease | en_US |
dc.type | Article | en_US |
dc.rights.robots | IndexNoFollow | en_US |
dc.subject.hlbsecondlevel | Medicine (General) | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Department of Neurology, University of Michigan, Ann Arbor, Michigan, U.S.A. | en_US |
dc.identifier.pmid | 1743163 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/65436/1/j.1528-1157.1991.tb05548.x.pdf | |
dc.identifier.doi | 10.1111/j.1528-1157.1991.tb05548.x | en_US |
dc.identifier.source | Epilepsia | en_US |
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dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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