{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T06:50:07Z","timestamp":1756191007480},"reference-count":78,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2002,2,1]],"date-time":"2002-02-01T00:00:00Z","timestamp":1012521600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Curr Treat Options Neurol"],"published-print":{"date-parts":[[2002,2]]},"DOI":"10.1007\/s11940-002-0001-9","type":"journal-article","created":{"date-parts":[[2007,6,4]],"date-time":"2007-06-04T17:28:42Z","timestamp":1180978122000},"page":"3-17","source":"Crossref","is-referenced-by-count":14,"title":["Progressive myoclonic epilepsies"],"prefix":"10.1007","volume":"4","author":[{"given":"Basim M.","family":"Uthman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Reichl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"issue":"5","key":"1_CR1","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1056\/NEJM198607313150506","volume":"315","author":"SF Berkovic","year":"1986","unstructured":"Berkovic SF, Andermann F, Carpenter S, Wolfe LS: Progressive myoclonus epilepsies: specific causes and diagnosis. N Engl J Med 1986, 315(5):296\u2013305.","journal-title":"N Engl J Med"},{"issue":"5","key":"1_CR2","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1097\/00006205-199305000-00012","volume":"18","author":"E Tate","year":"1993","unstructured":"Tate E: The clinical challenge of progressive myoclonus epilepsy. Nurse Pract 1993, 18(5):25\u201328.","journal-title":"Nurse Pract"},{"key":"1_CR3","first-page":"467","volume-title":"The Treatment of Epilepsy: Principles and Practice","author":"AV Delgado-Escueta","year":"1997","unstructured":"Delgado-Escueta AV, Serratosa JM, Medina MT: Myoclonic seizures and progressive myoclonus epilepsy syndromes. In The Treatment of Epilepsy: Principles and Practice, edn 2. Edited by Wyllie E. Williams & Wilkins: Baltimore; 1997:467\u2013483.","edition":"edn 2"},{"key":"1_CR4","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1093\/hmg\/4.4.709","volume":"4","author":"K Yamakawa","year":"1995","unstructured":"Yamakawa K, Mitchell S, Hubert R, et al.: Isolation and characterization of a candidate gene for progressive myoclonus epilepsy on 21q22.3. Hum Mol Genet 1995, 4:709\u2013716.","journal-title":"Hum Mol Genet"},{"key":"1_CR5","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1093\/hmg\/4.9.1657","volume":"4","author":"JM Serratosa","year":"1995","unstructured":"Serratosa JM, Delgado-Escueta AV, Posada I, et al.: The gene for progressive myoclonus epilepsy of the Lafora type maps to chromosome 6q. Hum Mol Genet 1995, 4:1657\u20131663.","journal-title":"Hum Mol Genet"},{"key":"1_CR6","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1001\/archneur.1983.04050090059009","volume":"40","author":"JJ Ohisalo","year":"1983","unstructured":"Ohisalo JJ, Murros K, Fredholm BB, Hare TA: Concentrations of g-aminobutyric acid and adenosine in the CSF in progressive myoclonus epilepsy without Lafora bodies. Arch Neurol 1983, 40:623\u2013625.","journal-title":"Arch Neurol"},{"key":"1_CR7","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1111\/j.1600-0404.1980.tb03002.x","volume":"62","author":"E Leino","year":"1980","unstructured":"Leino E, MacDonald E, Airaksinen MM, Riekinen PJ: Homovanilic acid and 5-hydroxyindolacetic acid levels in cerebrospinal fluid of patients with progressive myoclonus epilepsy. Acta Neurol Scand 1980, 62:41\u201354.","journal-title":"Acta Neurol Scand"},{"issue":"4","key":"1_CR8","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1007\/BF02335732","volume":"8","author":"M Iivanainen","year":"1987","unstructured":"Iivanainen M, Eldrige R: Effect of phenytoin on the mental and physical function of patients with Baltic myoclonus epilepsy. Ital J Neurol Sci 1987, 8(4):313\u2013317.","journal-title":"Ital J Neurol Sci"},{"key":"1_CR9","unstructured":"Halliday AM: Cerebral evoked potentials in familial progressive myoclonic epilepsy. J Royal Coll Phys Lond 1:123\u2013134."},{"key":"1_CR10","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1093\/brain\/101.3.447","volume":"101","author":"H Shibasaki","year":"1978","unstructured":"Shibasaki H, Yamashita Y, Kuroiwa Y: Myoclonusrelated cortical spikes and high amplitude somatosensory evoked potentials. Brain 1978, 101:447\u2013460.","journal-title":"Brain"},{"key":"1_CR11","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1093\/brain\/108.1.225","volume":"108","author":"H Shibasaki","year":"1985","unstructured":"Shibasaki H, Yamashita Y, Neshige R, et al.: Pathogenesis of giant somatosensory evoked potentials in progressive myoclonic epilepsy. Brain 1985, 108:225\u2013240.","journal-title":"Brain"},{"key":"1_CR12","first-page":"119","volume-title":"Advances in Neurology, vol 43: Myoclonus","author":"S Bressman","year":"1986","unstructured":"Bressman S, Fahn S, Eisenberg M, et al.: Biotinresponsive encephalopathy with myoclonus, ataxia, and seizures. In Advances in Neurology, vol 43: Myoclonus. Edited by Fahn S. New York: Raven Press; 1986:119\u2013125."},{"issue":"11","key":"1_CR13","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1056\/NEJM198303173081107","volume":"308","author":"JG Thoene","year":"1983","unstructured":"Thoene JG, Lemons R, Baker H: Impaired intestinal absorption of biotin in juvenile multiple carboxylase deficiency. N Engl J Med 1983, 308(11):639\u2013642.","journal-title":"N Engl J Med"},{"key":"1_CR14","doi-asserted-by":"crossref","unstructured":"Wolf B, Heard GS, McVoy JRS, Grier RE: Biotinidase deficiency. Ann NY Acad Sci 1985, 252\u2013262.","DOI":"10.1111\/j.1749-6632.1985.tb18443.x"},{"key":"1_CR15","first-page":"307","volume":"2","author":"RM Wilder","year":"1921","unstructured":"Wilder RM: The effects of ketonemia on the course of epilepsy. Mayo Clin Bull 1921, 2:307\u2013308.","journal-title":"Mayo Clin Bull"},{"key":"1_CR16","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1111\/j.1528-1157.1978.tb04503.x","volume":"19","author":"NM Davidian","year":"1978","unstructured":"Davidian NM, Butler TM, Poole DT: The effect of ketosis induced by medium chain triglycerides on intracellular pH of mouse brain. Epilepsia 1978, 19:396\u2013378.","journal-title":"Epilepsia"},{"key":"1_CR17","volume-title":"Comprehensive Management of Epilepsy in Infancy, Childhood, and Adolescence","author":"S Livingston","year":"1972","unstructured":"Livingston S: Comprehensive Management of Epilepsy in Infancy, Childhood, and Adolescence, edn 1. Springfield: Charles C. Thomas Publishers; 1972.","edition":"edn 1"},{"key":"1_CR18","first-page":"937","volume-title":"The Treatment of Epilepsy: Principles and Practice","author":"BM Uthman","year":"1997","unstructured":"Uthman BM, Beydoun A: Less commonly used antiepileptic therapies. In The Treatment of Epilepsy: Principles and Practice, edn 2. Edited by Wyllie E. Baltimore: Williams & Wilkins; 1997:937\u2013953.","edition":"edn 2"},{"key":"1_CR19","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1001\/archpedi.153.9.946","volume":"153","author":"JM Freeman","year":"1999","unstructured":"Freeman JM, Viling EPG: Seizures decrease rapidly after fasting. Arch Pediatr Adoesc Med 1999, 153:946\u2013949. A recent trial in 17 children evaluating the decrease in atonic and myoclonic seizures during the use of a ketogenic diet. To provide an objective method of counting seizures, an ambulatory 24-hour EEG was used in this study.","journal-title":"Arch Pediatr Adoesc Med"},{"key":"1_CR20","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1111\/j.1528-1157.1985.tb05658.x","volume":"26","author":"AWK Chan","year":"1985","unstructured":"Chan AWK: Alcohol and epilepsy. Epilepsia 1985, 26:323\u2013333.","journal-title":"Epilepsia"},{"key":"1_CR21","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1002\/mds.870060219","volume":"6","author":"S Jain","year":"1991","unstructured":"Jain S, Jain M: Action myoclonus (Lance-Adams syndrome) secondary to strangulation with dramatic response to alcohol. Mov Disord 1991, 6:183.","journal-title":"Mov Disord"},{"issue":"9","key":"1_CR22","doi-asserted-by":"crossref","first-page":"1412","DOI":"10.1212\/WNL.40.9.1412","volume":"40","author":"P Genton","year":"1990","unstructured":"Genton P, Guerrini R: Antimyoclonic effects of alcohol in progressive myoclonus epilepsy. Neurology 1990, 40(9):1412\u20131416.","journal-title":"Neurology"},{"key":"1_CR23","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1136\/jnnp.54.10.905","volume":"54","author":"CS Lu","year":"1991","unstructured":"Lu CS, Chu NS: Effects of alcohol on myoclonus and somatosensory evoked potentials in dyssinergia cerebellaris.J Neurol Neurosurg Psychiatry 1991, 54:905\u2013908.","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"1_CR24","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1046\/j.1440-1819.1999.00608.x","volume":"53","author":"MC Hsiao","year":"1999","unstructured":"Hsiao MC, Liu CY, Yang YY, et al.: Progressive myoclonus epilepsies syndrome (Ramsay Hunt syndrome) with mental disorder: report of two cases. Psych Clin Neurosci 1999, 53:575\u2013578.","journal-title":"Psych Clin Neurosci"},{"key":"1_CR25","first-page":"824","volume-title":"The Treatment of Epilepsy: Principles and Practice","author":"JC Dean","year":"1997","unstructured":"Dean JC: Valproate. In The Treatment of Epilepsy: Principles and Practice, edn 2. Edited by Wyllie E. Williams & Wilkins: Baltimore; 1997: 824\u2013832.","edition":"edn 2"},{"key":"1_CR26","first-page":"581","volume-title":"Antiepileptic Drugs","author":"R Fariello","year":"1995","unstructured":"Fariello R, Varasi M, Smith MC: Valproate: mechanism of action. In Antiepileptic Drugs, edn 4. Edited by Levy R, Mattson RH, Meldrum BS, et al. New York: Raven Press; 1995:581\u2013588.","edition":"edn 4"},{"issue":"4","key":"1_CR27","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1001\/archneur.1982.00510160042008","volume":"39","author":"M Iivanainen","year":"1982","unstructured":"Iivanainen M, Himberg JJ: Valproate and clonazepam in the treatment of severe progressive myoclonus epilepsy. Arch Neurol 1982, 39(4):236\u2013238.","journal-title":"Arch Neurol"},{"issue":"2","key":"1_CR28","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0920-1211(97)00069-7","volume":"29","author":"M Kyllerman","year":"1998","unstructured":"Kyllerman M, Ben-Menachem E: Zonisamide for progressive myoclonus epilepsy: long term observations in seven patients. Epilepsy Res 1998, 29(2):109\u2013114. A recent article describing the long term results of treating 7 PME patients (six Unverricht-Lundborg disease and one with Lafora\u2019s) with a new AED, zonisamide. The authors are particularly impressed with the dramatic effect of zonisamide on myoclonus in 5 of the 7 patients.","journal-title":"Epilepsy Res"},{"key":"1_CR29","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1097\/00007691-198612000-00006","volume":"8","author":"MRF Gatta De","year":"1986","unstructured":"De Gatta MRF, Gonzales ACA, Sanchez MJG, et al.: Effect of sodium valproate on phenobarbital serum levels in children and adults. Ther Drug Monit 1986, 8:416\u2013420.","journal-title":"Ther Drug Monit"},{"key":"1_CR30","first-page":"161","volume":"20","author":"RR Clancy","year":"1990","unstructured":"Clancy RR: Valproate: an update - the challenge of modern pediatric seizure management. Curr Probl Pediatr 1990, 20:161\u2013233.","journal-title":"Curr Probl Pediatr"},{"key":"1_CR31","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1136\/bmj.2.5919.584","volume":"2","author":"PM Jeavons","year":"1974","unstructured":"Jeavons PM, Clark JE: Sodium valproate in the treatment of epilepsy. Br Med J 1974, 2:584\u2013586.","journal-title":"Br Med J"},{"issue":"suppl 4","key":"1_CR32","first-page":"72","volume":"36","author":"JC Dean","year":"1995","unstructured":"Dean JC, Penry JK: Weight gain patterns in patients with epilepsy: comparison of antiepileptic drugs. Epilepsia 1995, 36(suppl 4):72.","journal-title":"Epilepsia"},{"key":"1_CR33","first-page":"721","volume-title":"Anitepileptic Drugs","author":"W Haefely","year":"1989","unstructured":"Haefely W: Benzodiazepines: mechanism of action. In Anitepileptic Drugs, edn 3. Edited by Levy RH, Dreifuss FE, Mattson RH, et al. New York: Raven Press; 1989:721\u2013734.","edition":"edn 3"},{"key":"1_CR34","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1136\/jnnp.40.6.538","volume":"40","author":"RN Nanda","year":"1977","unstructured":"Nanda RN, Johnson RH, Koegh HJ, et al.: Treatment of epilepsy with clonazepam and its effect on other anticonvulsants. J Neurol Neurosurg Psychiatry 1977, 40:538\u2013543.","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"5","key":"1_CR35","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1111\/j.1528-1157.1989.tb05479.x","volume":"30","author":"T Obeid","year":"1989","unstructured":"Obeid T, Panayiotopoulos CP: Clonazepam in juvenile myoclonic epilepsy. Epilepsia 1989, 30(5):603\u2013606.","journal-title":"Epilepsia"},{"key":"1_CR36","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1056\/NEJM197810122991505","volume":"299","author":"TR Browne","year":"1978","unstructured":"Browne TR: Clonazepam. N Engl J Med 1978, 299:812\u2013816.","journal-title":"N Engl J Med"},{"key":"1_CR37","first-page":"845","volume-title":"The Treatment of Epilepsy: Principles and Practice","author":"BFD Bourgeois","year":"1997","unstructured":"Bourgeois BFD: Phenobarbital and primidone. In The Treatment of Epilepsy: Principles and Practice, edn 2. Edited by Wyllie E. Baltimore: Williams & Wilkins; 1997:845\u2013855.","edition":"edn 2"},{"key":"1_CR38","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1007\/978-1-4615-3328-3_10","volume-title":"Ion Channels","author":"RL MacDonald","year":"1992","unstructured":"MacDonald RL, Twyman RE: Kinetic properties and regulation of GABA receptor channels. In Ion Channels. Edited by Narahshi R. New York: Plenum Press; 1992:315\u2013343."},{"key":"1_CR39","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/0006-8993(82)90042-7","volume":"236","author":"EJ Heyer","year":"1982","unstructured":"Heyer EJ, MacDonald RL: Barbiturate reduction of calcium-dependent action potentials: correlation with anesthetic action. Brain Res 1982, 236:157\u2013171.","journal-title":"Brain Res"},{"key":"1_CR40","doi-asserted-by":"crossref","first-page":"824","DOI":"10.1542\/peds.68.6.824","volume":"68","author":"AG Davis","year":"1981","unstructured":"Davis AG, Mutchie KD, Thompson JA, Myers GG: Once-daily dosing with phenobarbital in children with seizure disorders. Pediatrics 1981, 68:824\u2013827.","journal-title":"Pediatrics"},{"key":"1_CR41","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/0006-8993(93)91660-K","volume":"612","author":"G Lees","year":"1993","unstructured":"Lees G, Leach MJ: Studies on the mechanism of action of the novel anticonvulsant lamotrigine (Lamictal) using primary neuroglial cultures from rat cortex. Brain Res 1993, 612:190\u2013199.","journal-title":"Brain Res"},{"issue":"2","key":"1_CR42","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/S0920-1211(97)00080-6","volume":"29","author":"SJ Wallace","year":"1998","unstructured":"Wallace SJ: Myoclonus and epilepsy in childhood: a review of treatment with valproate, ethosiximide, lamotrigine, and zonisamide. Epilepsy Res 1998, 29(2):147\u2013154. An article reviewing 4 AEDs (two classical and two new drugs) in the treatment of myoclonus epilepsy (PME and other conditions) in pediatric patients.","journal-title":"Epilepsy Res"},{"issue":"suppl 3","key":"1_CR43","first-page":"S264","volume":"36","author":"P Santavuori","year":"1995","unstructured":"Santavuori P, Himberg JJ, Ahola A, et al.: Lamotrigine therapy in infantile ceroid lipofuscinosis. Epilepsia 1995, 36(suppl 3):S264.","journal-title":"Epilepsia"},{"issue":"5","key":"1_CR44","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1111\/j.1528-1157.1998.tb01413.x","volume":"39","author":"R Guerrini","year":"1998","unstructured":"Guerrini R, Dravet C, Genton P, et al.: Lamotrigine and seizure aggravation in severe myoclonic epilepsy. Epilepsia 1998, 39(5):508\u2013512.","journal-title":"Epilepsia"},{"issue":"suppl 3","key":"1_CR45","first-page":"82","volume":"32","author":"GD Anderson","year":"1991","unstructured":"Anderson GD, Gidal BE, Levy RH, et al.: Effect of lamotrigine on the pharmacokinetics and biotransformation of valproate. Epilepsia 1991, 32(suppl 3):82.","journal-title":"Epilepsia"},{"key":"1_CR46","first-page":"920","volume-title":"The Treatment of Epilepsy: Principles and Practice","author":"R Fisher","year":"1997","unstructured":"Fisher R: Newer antiepileptic drugs. In The Treatment of Epilepsy: Principles and Practice, edn 2. Edited by Wyllie E. Baltimore: Williams & Wilkins; 1997:920\u2013930.","edition":"edn 2"},{"key":"1_CR47","first-page":"123","volume":"34","author":"D Coulter","year":"1993","unstructured":"Coulter D, Sombati S, DeLorenzo RJ: Selective effects of topiramate on sustained repetetive findings and spontaneous bursting in cultured hippocampal neurons. Epilepsia 1993, 34:123.","journal-title":"Epilepsia"},{"issue":"3","key":"1_CR48","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/S1090-3798(99)90097-1","volume":"3","author":"P Uldall","year":"1999","unstructured":"Uldall P, Bucholt JM: Clinical experiences with topiramate in children with intractable epilepsy. Euro J Ped Neurol 1999, 3(3):105\u2013111. A recent open, add-on trial with topiramate in 39, mostly mentally retarded children with intractable epilepsy syndromes. One of the two PME patients included in the study showed dramatic clinical improvements.","journal-title":"Euro J Ped Neurol"},{"issue":"suppl 5","key":"1_CR49","doi-asserted-by":"crossref","first-page":"S23","DOI":"10.1111\/j.1528-1157.1999.tb00916.x","volume":"40","author":"IE Leppik","year":"1999","unstructured":"Leppik IE: Zonisamide. Epilepsia 1999, 40(suppl 5):S23-S29. A very recent review of the new AED zonisamide, with special interest in regards to pharmacologic basics and clinical use of this drug, including drug interactions and adverse events. The potency of zonisamide in the treatment of PME is outlined.","journal-title":"Epilepsia"},{"key":"1_CR50","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/0920-1211(92)90087-A","volume":"12","author":"S Suzuki","year":"1992","unstructured":"Suzuki S, Kawakami K, Nishimura S, et al.: Zonisamide blocks T-type calcium channel in cultured neurons of rat cerebral cortex. Epilepsy Res 1992, 12:21\u201327.","journal-title":"Epilepsy Res"},{"key":"1_CR51","first-page":"640","volume":"42","author":"T Mimaki","year":"1998","unstructured":"Mimaki T, Suzuki S, Tagawa T, et al.: Zonisamide binding in rat brain. Jpn J Psychiatry Neurol 1998, 42:640\u2013642.","journal-title":"Jpn J Psychiatry Neurol"},{"key":"1_CR52","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1212\/WNL.38.6.928","volume":"38","author":"TR Henry","year":"1988","unstructured":"Henry TR, Leppik IE, Gumnit RJ, Jacobs M: Progressive myoclonus epilepsy treated with zonisamide. Neurology 1988, 38:928\u2013931.","journal-title":"Neurology"},{"key":"1_CR53","unstructured":"NDA 20-789; Zonegram (zonisamide) Capsules 100mg [FDA approved labeling text]. San Francisco, CA: Elan Pharmaceuticals Inc; 2000."},{"key":"1_CR54","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1248\/bpb1978.15.631","volume":"15","author":"M Kimura","year":"1992","unstructured":"Kimura M, Tanaka N, Kimura Y, et al.: Pharmacokinetic interaction of zonisamide in rats: effect of other antiepiletics on zonisamide. J Pharmacobio Dynam 1992, 15:631\u2013639.","journal-title":"J Pharmacobio Dynam"},{"key":"1_CR55","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1097\/00007691-198609000-00010","volume":"8","author":"LM Ojemann","year":"1986","unstructured":"Ojemann LM, Shastri RA, Wilensky AJ, et al.: Comparative pharmacokinetics of zonisamide in epileptic patients on carbamazepine or phenytoin monotherapy. Ther Drug Monit 1986, 8:293\u2013296.","journal-title":"Ther Drug Monit"},{"key":"1_CR56","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/S0006-2952(96)00463-7","volume":"53","author":"WE Mueller","year":"1997","unstructured":"Mueller WE, Koch S, Scheuer K, et al.: Effects of piracetam on membrane fluidity in the aged mouse, rat, and human brain. Biochem Pharmaco 1997, 53:135\u2013140.","journal-title":"Biochem Pharmaco"},{"key":"1_CR57","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1016\/0006-2952(95)00225-O","volume":"50","author":"J Peuvot","year":"1995","unstructured":"Peuvot J, Schanck A, Deleers M, Brasseur R: Piracetam induced changes to membran physical properties. Biochem Pharmacol 1995, 50:1129\u20131134.","journal-title":"Biochem Pharmacol"},{"key":"1_CR58","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1016\/S0140-6736(79)90689-5","volume":"8145","author":"RL Bick","year":"1979","unstructured":"Bick RL: In vivo platelet inhibition by piracetam [letter]. Lancet 1979, 8145:752\u2013753.","journal-title":"Lancet"},{"key":"1_CR59","first-page":"247","volume-title":"Alzheimer\u2019s Disease","author":"G Pepeu","year":"1990","unstructured":"Pepeu G, Spignoli G: Neurochemichal actions of \u2018nootropic drugs\u2019. In Alzheimer\u2019s Disease. Edited by Wirtman RJ, Corkin S, Growdon JH, Ritter-Walker E. New York: Raven; 1990:247\u2013252."},{"issue":"1","key":"1_CR60","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1097\/00002826-198602000-00006","volume":"9","author":"JA Obeso","year":"1986","unstructured":"Obeso JA, Artieda J, Luquin MR, et al.: Antimyoclonic action of piracetam. Clin Neuropharmacology 1986, 9(1):58\u201364.","journal-title":"Clin Neuropharmacology"},{"issue":"6","key":"1_CR61","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1002\/mds.870110615","volume":"11","author":"A Ikeda","year":"1996","unstructured":"Ikeda A, Shibasaki H, Tashiro K, et al.: Clinical trial of piracetam in patients with myoclonus: nationwide multiinstitution study in Japan. Mov Disord 1996, 11(6):691\u2013700.","journal-title":"Mov Disord"},{"key":"1_CR62","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1136\/jnnp.64.3.344","volume":"64","author":"M Koskiniemi","year":"1998","unstructured":"Koskiniemi M, Van Vleymen B, Hakamies L, et al.: Piracetam relieves symptoms in progressive myoclonus epilepsy: a multicentre, randomised, double blind, crossover study comparing the efficacy and safety of three dosages of oral piracetam vs. placebo. J Neurol Neurosurg Psychiatry 1998, 64:344\u2013348. A recent multicenter, randomized, double-blind, crossover trial demonstrating a dose-effect relationship between piracetam and its beneficial effect on 20 patients with the classical Unverricht-Lundborg disease. The ratings of stimulus sensitivity, functional disability, handwriting, and global assessment were included in measuring outcome.","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"6","key":"1_CR63","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1159\/000116700","volume":"31","author":"W Paulus","year":"1991","unstructured":"Paulus W, Ried S, Stodieck SR, Schmidt D: Abolition of photoparoxysmal response in progressive myoclonus epilepsy. Eur.Neurol 1991, 31(6):388\u2013390.","journal-title":"Eur.Neurol"},{"key":"1_CR64","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1528-1157.1980.tb04082.x","volume":"21","author":"M Koskiniemi","year":"1980","unstructured":"Koskiniemi M, Hyyppa M, Sainio K, et al.: Transient effect of L-tryptophan in progressive myoclonus epilepsy without Lafora bodies: clinical and electrophysiological study. Epilepsia 1980, 21:351\u2013357.","journal-title":"Epilepsia"},{"issue":"2","key":"1_CR65","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1056\/NEJM197701132960203","volume":"296","author":"MH Woert Van","year":"1977","unstructured":"Van Woert MH, Rosenbaum D, Howieson J, Bowers MB Jr: Long-term therapy of myoclonus and other neurologic disorders with L-5-hydroxytryptophan and carbidopa. N Engl J Med 1977, 296(2):70\u201375.","journal-title":"N Engl J Med"},{"issue":"8","key":"1_CR66","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1111\/j.1528-1157.1995.tb01615.x","volume":"36","author":"MR Pranzatelli","year":"1995","unstructured":"Pranzatelli MR, Tate E, Huang Y, et al.: Neuropharmacology of progressive myoclonus epilepsy: response to 5-hydroxy-L-tryptophan. Epilepsia 1995, 36 (8):783\u2013791.","journal-title":"Epilepsia"},{"key":"1_CR67","first-page":"1960","volume":"36","author":"M Brownstein","year":"1977","unstructured":"Brownstein M: Neurotransmitter and hypothalamic hormones in the central nervous system. Fed Proc 1977, 36:1960\u20131963.","journal-title":"Fed Proc"},{"key":"1_CR68","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1016\/S0140-6736(80)90963-0","volume":"1","author":"I Sobue","year":"1980","unstructured":"Sobue I, Yamamota H, Kanagawa M, et al.: Effects of thyrotropin releasing hormone on ataxia of spincerebellar degeneration. Lancet 1980, 1:418\u2013419.","journal-title":"Lancet"},{"issue":"5","key":"1_CR69","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/S0887-8994(97)00230-0","volume":"18","author":"C Tanaka","year":"1998","unstructured":"Tanaka C, Maegaki Y, Koeda T, et al.: Successful treatment of progressive myoclonus epilepsy with TRH. Pediatr Neurol 1998, 18(5):442\u2013444.","journal-title":"Pediatr Neurol"},{"key":"1_CR70","doi-asserted-by":"crossref","first-page":"201","DOI":"10.2739\/kurumemedj.28.201","volume":"28","author":"K Inanaga","year":"1981","unstructured":"Inanaga K, Inoue Y: Effect of thyrotropin releasing hormone analog in a patient with myoclonus epilepsy. Kurume Med J 1981, 28:201\u2013210.","journal-title":"Kurume Med J"},{"key":"1_CR71","first-page":"82","volume":"28","author":"AJ Prange","year":"1972","unstructured":"Prange AJ, Wilson IC: Thyrotropin releasing hormone for the immediate relief of depression. Psychopharmacology 1972, 28(suppl):82\u201387.","journal-title":"Psychopharmacology"},{"issue":"suppl 2","key":"1_CR72","first-page":"14","volume":"34","author":"RW Hurd","year":"1993","unstructured":"Hurd RW, Wilder BJ, Perchalski RJ: Increased extracellular superoxide dismutase in Baltic myoclonus. Epilepsia 1993, 34(suppl 2):14\u201315.","journal-title":"Epilepsia"},{"issue":"5","key":"1_CR73","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1212\/WNL.47.5.1264","volume":"47","author":"RW Hurd","year":"1996","unstructured":"Hurd RW, Wilder BJ, Helveston WR, Uthman BM: Treatment of four siblings with progressive myoclonus epilepsy of the Unverricht Lundborg type with N acetylcysteine. Neurology 1996 Nov; 47 (5): 1264\u20131268. A brief report describing the beneficial effect of N-acetylcysteine (NAC) in four siblings with PME of the Unverricht-Lundborg type. Doses of NAC and supplemental vitamins and trace metals are mentioned.","journal-title":"Neurology"},{"issue":"2","key":"1_CR74","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1212\/WNL.52.2.426","volume":"52","author":"LM Selwa","year":"1999","unstructured":"Selwa LM: N-acetylcysteine for Unverricht-Lundborg disease. Neurology 1999, 52(2):426\u2013427.","journal-title":"Neurology"},{"issue":"1","key":"1_CR75","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1177\/088307389501000118","volume":"10","author":"Y Awaad","year":"1995","unstructured":"Awaad Y, Fish I: Baclofen in the treatment of polymyoclonus in a patient with Unverricht Lundborg disease. J Child Neurol 1995, 10(1):68\u201370.","journal-title":"J Child Neurol"},{"key":"1_CR76","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/med.2610050103","volume":"5","author":"G Bartholini","year":"1985","unstructured":"Bartholini G: GABA receptor agonist: Pharmacological spectrum and therapeutic actions. Med Res Rev 1985, 5:55\u201375.","journal-title":"Med Res Rev"},{"key":"1_CR77","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/0014-2999(76)90091-1","volume":"36","author":"R Horowski","year":"1976","unstructured":"Horowski R, Wachtel H: Direct dopaminergic action of lisuride hydrogen maleate, an ergot derivate in mice. Eur J Pharmacol 1976, 36:373\u2013383.","journal-title":"Eur J Pharmacol"},{"key":"1_CR78","first-page":"191","volume-title":"Myoclonus: Advances in Neurology","author":"JA Obeso","year":"1986","unstructured":"Obeso JA, Rothwell JC, Quinn NP, et al.: Lisuride in the treatment of myoclonus. In Myoclonus: Advances in Neurology, vol 43. Edited by Fahn S, Marsden CD, Van Woert MH. New York: Raven Press; 1986:191\u2013196."}],"container-title":["Current Treatment Options in Neurology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11940-002-0001-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s11940-002-0001-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11940-002-0001-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,14]],"date-time":"2024-02-14T19:40:09Z","timestamp":1707939609000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s11940-002-0001-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,2]]},"references-count":78,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2002,2]]}},"alternative-id":["1"],"URL":"https:\/\/doi.org\/10.1007\/s11940-002-0001-9","relation":{},"ISSN":["1092-8480","1534-3138"],"issn-type":[{"value":"1092-8480","type":"print"},{"value":"1534-3138","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,2]]}}}