{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T03:14:07Z","timestamp":1761016447655},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2005,6,9]],"date-time":"2005-06-09T00:00:00Z","timestamp":1118275200000},"content-version":"vor","delay-in-days":3657,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1995,6,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The relationship between free radicals and scavenger enzymes, and the disorders called the neuronal ceroid\u2010lipofuscinoses, has long been an argumentative one. Recent evidence would seem to support the fact that such a relationship might exist but that it is indirect. The relationship does not seem due to an inborn error of free radical scavenger enzyme metabolism. Anticonvulsants play a role, as they influence free radical generating systems. At this juncture, no one has studied the relationship of anticonvulsant therapy, neuronal ceroid\u2010lipofuscinosis, and the free radical\u2010scavenging enzyme system, and their interplay.<\/jats:p><jats:p>We have studied a large number of patients with epilepsy who are on either monotherapeutic or polytherapeutic regimens of most of the common anticonvulsants. We have found excessive free radical production in many of these patients, ranging from minor effects in the simpler anticonvulsants when used monotherapeutically, to more complex changes in polytherapeutic combinations. Likewise, we have found subtle and inconsistent findings in the free radical\u2010scavenging enzyme system in a variety of examples of neuronal ceroid\u2010lipofuscinosis.<\/jats:p><jats:p>When refractory seizure disorders stimulate the vigorous use of polytherapy with a variety of free radical\u2010facilitating anticonvulsants, free radical production becomes deleterious. Likewise, in certain types of neuronal ceroid\u2010lipofuscinosis, polypharmacy with anticonvulsants, by enhancing the production of free radicals or suppressing scavenging enzymes, tends to be deleterious and induces a worsening in the disease process. \u00a9 1995 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320570222","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T18:01:48Z","timestamp":1118512908000},"page":"225-228","source":"Crossref","is-referenced-by-count":53,"title":["Free radicals, anticonvulsants, and the neuronal ceroid\u2010lipofuscinoses"],"prefix":"10.1002","volume":"57","author":[{"given":"Paul","family":"Maertens","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Dyken","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William","family":"Graf","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charles","family":"Pippenger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Chronister","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arvind","family":"Shah","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,9]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1974.00490320032004"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(81)90373-8"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/94.4.745"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(85)13062-4"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(82)90492-5"},{"key":"e_1_2_1_7_1","first-page":"348","article-title":"Assays for differentiation of gluta\u2010thione\u2010S\u2010transferases","volume":"77","author":"Habig WH","year":"1981","journal-title":"Methods Enzymol"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1159\/000114834"},{"key":"e_1_2_1_9_1","doi-asserted-by":"crossref","first-page":"6049","DOI":"10.1016\/S0021-9258(18)63504-5","article-title":"Superoxide dismutase: an enzymatic function for erythocuprin (hemocuprein)","volume":"244","author":"McCord JM","year":"1969","journal-title":"J Biol Chem"},{"key":"e_1_2_1_10_1","volume-title":"Principles of Biostatistics.","author":"Pagano M","year":"1983"},{"key":"e_1_2_1_11_1","first-page":"158","article-title":"Studies on the quantitative and qualitative characterization of erythrocyte glutathione peroxidase","volume":"70","author":"Paglia DE","year":"1967","journal-title":"J Lab Clin Med"},{"key":"e_1_2_1_12_1","first-page":"75","volume-title":"Idiosyncratic Reaction to Valproate. 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