Progressive Myoclonic Epilepsies (Progressive Myoclonic Epilepsy)

226  relevant articles (4 outcomes, 20 trials/studies) found for this Disease

Progressive Myoclonic Epilepsy; Epilepsy, Progressive Myoclonic; Dentatorubral-Pallidoluysian Atrophy; Myoclonic Epilepsies, Progressive; Atypical Inclusion-Body Disease; Familial Progressive Myoclonic Epilepsy; Progressive Myoclonus Epilepsies; Action Myoclonus Renal Failure Syndrome; Atrophies, Dentatorubral-Pallidoluysian; Atrophy, Dentatorubral-Pallidoluysian; Atypical Inclusion Body Disease; Atypical Inclusion-Body Diseases; Biotin Responsive Encephalopathy; Biotin-Responsive Encephalopathies; Dentatorubral Pallidoluysian Atrophy; Dentatorubral-Pallidoluysian Atrophies; Disease, Atypical Inclusion-Body; Diseases, Atypical Inclusion-Body; Encephalopathies, Biotin-Responsive; Encephalopathy, Biotin-Responsive; Epilepsies, Progressive Myoclonic; Epilepsies, Progressive Myoclonus; Epilepsy, Progressive Myoclonus; Inclusion-Body Disease, Atypical; Inclusion-Body Diseases, Atypical; May White Syndrome; May-White Syndromes; Myoclonic Epilepsy, Progressive; Myoclonus Epilepsies, Progressive; Myoclonus Epilepsy, Progressive; Progressive Myoclonus Epilepsy; Syndrome, May-White; Syndromes, May-White; Action Myoclonus-Renal Failure Syndrome; Biotin-Responsive Encephalopathy; May-White Syndrome

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Seizures (Seizure)
2.	Neurodegenerative Diseases (Neurodegenerative Disease)
3.	Huntington Disease (Huntington's Disease)
4.	Spinocerebellar Ataxias (Spinocerebellar Ataxia)
5.	Myoclonus (Nocturnal Myoclonus)
47 Diseases and 105 more articles analyzed in the Research Interface, order at left...

Experts

1.	Minassian, Berge A: 4 articles (09/2005 - 01/2002)
2.	Vaarmann, Annika: 2 articles (10/2006 - 01/2005)
3.	Sato, Toshiya: 2 articles (08/2006 - 11/2002)
4.	Yamada, Mitsunori: 2 articles (08/2006 - 11/2002)
5.	Tsuji, Shoji: 2 articles (08/2006 - 11/2002)
6.	Takahashi, Hitoshi: 2 articles (08/2006 - 11/2002)
7.	Lohi, Hannes: 2 articles (09/2005 - 06/2004)
8.	Chan, Elayne M: 2 articles (09/2005 - 06/2004)
9.	Ianzano, Leonarda: 2 articles (09/2005 - 06/2004)
10.	Scherer, Stephen W: 2 articles (09/2005 - 06/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Progressive Myoclonic Epilepsies:

1.	Serotonin (5 Hydroxytryptamine)IBA 08/01/1995 - "Low concentrations of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid (CSF) of patients with progressive myoclonus epilepsy (PME) suggest hypofunctional serotonergic neurotransmission" 05/01/1996 - "Serotonergic drugs that work through mechanisms different than L-5-HTP may be more effective in correcting the abnormal serotonergic neurotransmission suggested by reduction of serotonin metabolites in cerebrospinal fluid in patients with progressive myoclonus epilepsy." 07/01/1980 - "The possibility of disturbed dopamine and serotonin metabolism in the progressive myoclonus epilepsy (PME) occurring in Finland (a type of PME without Lafora bodies) was examined" 01/01/1975 - "The tryptophan and 5-hydroxytryptamine metabolism deserves further investigation in attempting to discover the aetiology of progressive myoclonus epilepsy." 05/01/1996 - "To study the role of serotonin in the ataxia of progressive myoclonus epilepsy, eight patients received oral 5-hydroxy-L-tryptophan (L-5-HTP) or placebo plus carbidopa for 1 month in a controlled, double-blinded, dose-ranging, cross-over, add-on study" Get all the results and reference details, order at left...
2.	zonisamide (Zonegran)FDA LinkGeneric 08/01/2001 - "Zonisamide has a special place in the progressive myoclonus epilepsies" 01/01/1998 - "Zonisamide for progressive myoclonus epilepsy: long-term observations in seven patients." 06/01/1988 - "Progressive myoclonus epilepsy treated with zonisamide." 03/01/2008 - "PURPOSE: To examine the safety and efficacy of zonisamide in treating myoclonic seizures associated with progressive myoclonic epilepsy (PME), in an open-label setting" 03/01/2008 - "Zonisamide for the treatment of myoclonic seizures in progressive myoclonic epilepsy: an open-label study." Get all the results and reference details, order at left...
3.	etiracetam (levetiracetam)FDA Link 04/22/2003 - "Efficacy of levetiracetam in a patient with Unverricht-Lundborg progressive myoclonic epilepsy." 02/24/2004 - "Levetiracetam in progressive myoclonic epilepsy: an exploratory study in 9 patients." 06/01/2007 - "Levetiracetam appears to be a useful antimyoclonic agent in cases of progressive myoclonic epilepsy and should be considered for adjunctive therapy." 06/01/2007 - "Levetiracetam in three cases of progressive myoclonus epilepsy." 02/24/2004 - "The authors conducted an open study of levetiracetam as add-on therapy in nine patients with well-defined progressive myoclonic epilepsies and refractory myoclonus" Get all the results and reference details, order at left...
4.	Serotonin AgentsIBA 05/01/1996 - "Serotonergic drugs that work through mechanisms different than L-5-HTP may be more effective in correcting the abnormal serotonergic neurotransmission suggested by reduction of serotonin metabolites in cerebrospinal fluid in patients with progressive myoclonus epilepsy." Get all the results and reference details, order at left...
5.	Chloral HydrateIBA 11/01/2001 - "Because chloral hydrate helped to improve quality of life, it should be made available to patients with progressive myoclonus epilepsy as adjunctive therapy" 11/01/2001 - "Chloral hydrate for progressive myoclonus epilepsy: a new look at an old drug." Get all the results and reference details, order at left...
6.	DNA (Deoxyribonucleic Acid)IBA 01/01/2001 - "We applied whole-scalp magnetoencephalography to study activation sequences of the somatosensory cortical network in 7 patients with Unverricht-Lundborg-type progressive myoclonus epilepsy diagnostically verified by DNA analysis" 06/01/1993 - "Studies of mitochondria DNA in progressive myoclonus epilepsy (PME) and a case of atypical MELAS." 01/01/2003 - "Deoxyribonucleic acid analysis for the dentatorubral pallidoluysian atrophy gene was performed, and the CAG trinucleotide repeat numbers in this patient were 61/15" 11/01/1998 - "Genetic analysis including predictive DNA testing in a Japanese family with dentatorubral-pallidoluysian atrophy]" 09/01/1996 - "Therefore, children with progressive myoclonus epilepsy and the above MEP findings are likely candidates for childhood-onset DRPLA and should undergo DNA analysis for DRPLA." Get all the results and reference details, order at left...
7.	Carbidopa (Lodosyn)FDA LinkGeneric 08/01/1981 - "L-tryptophan-carbidopa trial in patients with long-standing progressive myoclonus epilepsy." 06/01/1981 - "Open trial with levodopa-carbidopa combination to patients with long-standing progressive myoclonus epilepsy." 05/01/1996 - "To study the role of serotonin in the ataxia of progressive myoclonus epilepsy, eight patients received oral 5-hydroxy-L-tryptophan (L-5-HTP) or placebo plus carbidopa for 1 month in a controlled, double-blinded, dose-ranging, cross-over, add-on study" 08/01/1981 - "Eleven patients with long-standing progressive myoclonus epilepsy, PME, and age- and sex-matched epileptic controls received L-tryptophan (L-Trp) 100 mg/kg body weight combined with carbidopa in addition to their usual anticonvulsant regimen" Get all the results and reference details, order at left...
8.	Tryptophan (L-Tryptophan)FDA Link 08/01/1980 - "A double-blind crossover trial with 2 g L-tryptophan and placebo was carried out with five familial and two sporadic patients with progressive myoclonus epilepsy (PME) without Lafora bodies" 08/01/1980 - "Transient effect of L-tryptophan in progressive myoclonus epilepsy without Lafora bodies: clinical and electrophysiological study." 01/01/1996 - "A periodic tryptophan protein 2 gene homologue (PWP2H) in the candidate region of progressive myoclonus epilepsy on 21q22.3." 07/01/1980 - "L-Tryptophan was administered orally to 7 patients with progressive myoclonus epilepsy (PME) without Lafora bodies and 7 controls" 07/01/1980 - "Deficient intestinal absorption of L-tryptophan in progressive myoclonus epilepsy without Lafora bodies." Get all the results and reference details, order at left...
9.	tributyl phosphate (TBP)IBA 08/01/2001 - "In this immunohistochemical study, we showed recruitment of ataxin-2, ataxin-3 and TATA box binding protein (TBP) into NIIs of the pontine neurons of spinocerebellar ataxia type (SCA) 1, SCA2, SCA3 and dentatorubral-pallidoluysian atrophy brains" 05/01/2008 - "BACKGROUND AND PURPOSE: The aim of this study was to perform DNA analysis in patients with clinical diagnosis of Huntington's disease (HD) after molecular exclusion of HD and further molecular examinations for other neurodegenerative diseases such as Huntington's disease-like 2 (HDL-2; gene JPH3), dentatorubral pallidoluysian atrophy (DRPLA; gene ATN1) and spinocerebellar ataxia type 17 (SCA17; gene TBP)" 12/01/2000 - "It has been demonstrated that the inclusions in dentatorubral-pallidoluysian atrophy (DRPLA) and Machado-Joseph disease (MJD) were immunopositive for several transcription factors such as TATA-binding protein (TBP), TBP-associated factor (TAF(II)130), Sp1, cAMP-responsive element-binding protein (CREB) and CREB-binding protein, suggesting that neuronal degeneration in polyglutamine diseases may result from nuclear depletion of transcription factors containing the glutamine-rich domain" Get all the results and reference details, order at left...
10.	polyglutamineIBA 08/01/2006 - "Polyglutamine disease: recent advances in the neuropathology of dentatorubral-pallidoluysian atrophy." 04/01/2005 - "The majority of these diseases result from expanded polyglutamine tracts in the encoded protein as seen in SCA1, SCA2, SCA3, SCA6, SCA7 and Dentatorubral-Pallidoluysian Atrophy (DRPLA)" 01/01/2005 - "We became aware of a subtle sequence difference in DRPLA, a polyglutamine disease gene for dentatorubral pallidoluysian atrophy" 11/01/2002 - "These findings suggest that glial cells in dentatorubral-pallidoluysian atrophy also are involved in the polyglutamine pathogenesis." 11/01/2002 - "Oligodendrocytic polyglutamine pathology in dentatorubral-pallidoluysian atrophy." Get all the results and reference details, order at left...
131 Drugs and Important Bio-Agents and 222 more articles analyzed in the Research Interface, order at left...

Therapies and Procedures

1.	Lasers (Laser) 11/01/2000 - "Employing a laser-captured microdissection (LCM), we have investigated the somatic instability of CAG repeats in the variable brain cell lineage in three patients with dentatorubral pallidoluysian atrophy (DRPLA)" 11/01/2000 - "Differential somatic CAG repeat instability in variable brain cell lineage in dentatorubral pallidoluysian atrophy (DRPLA): a laser-captured microdissection (LCM)-based analysis." 03/01/1999 - "Genotyping of the dentatorubral-pallidoluysian atrophy (DRPLA) locus in six patient samples, representing four normal individuals and two DRPLA patients, was successfully obtained using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF-MS)" Get all the results and reference details, order at left...
2.	Drug Therapy (Chemotherapy) 10/01/2007 - "Here, the case of a 39-year-old male with progressive myoclonic epilepsy is reported who remained severely impaired despite VNS and combined antiepileptic drug therapy" 06/01/2007 - "METHODS: We recorded the clinical course of the disease and the response to drug therapy, whereas carefully excluding known causes of progressive myoclonic epilepsy" Get all the results and reference details, order at left...
3.	Pallidotomy 11/25/2003 - "Pallidotomy for severe generalized chorea of juvenile-onset dentatorubral-pallidoluysian atrophy." Get all the results and reference details, order at left...
4.	Deep Brain Stimulation 10/01/2007 - "Chronic high-frequency deep brain stimulation of the STN/SNr for progressive myoclonic epilepsy." Get all the results and reference details, order at left...
5.	Electrodes (Electrode) 02/01/2001 - "Using a Coulochem electrode array system, we investigated the developmental changes of monoamine-related substances in patients with dentatorubral-pallidoluysian atrophy" Get all the results and reference details, order at left...
5 Therapies and Procedures and 6 more articles analyzed in the Research Interface, order at left...