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Progressive Myoclonic Epilepsies
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Legros, Benjamin, Zupanc, Mary M.L.
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Add-on therapy with topiramate in progressive myoclonic epilepsy
We evaluated the clinical responses to add-on therapy with topiramate in eight patients with progressive myoclonic epilepsy (PME). Severe myoclonic seizures disturbing daily activities were persistent despite adequate trials of various combinations of antiepileptic drugs in all patients.
Ebru, Aykutlu +5 more
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The progressive myoclonic epilepsies
Progressive myoclonic epilepsies are a group of disorders characterised by a relentlessly progressive disease course until death; treatment-resistant epilepsy is just a part of the phenotype. This umbrella term encompasses many diverse conditions, ranging from Lafora body disease to Gaucher's disease.
Naveed, Malek +2 more
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Startle Response in Progressive Myoclonic Epilepsy
Cortical reflex myoclonus is a typical feature of progressive myoclonic epilepsy (PME) in which it is accompanied by other types of mostly drug-resistant seizures and progressive neurological signs. Although PME is characterized by cortical hyperexcitability, studies have demonstrated atrophy and degenerative changes in the brainstem in various types ...
Kiziltan, Meral E. +6 more
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Progressive Myoclonic Epilepsies
Journal of Child Neurology, 2002The progressive myoclonic epilepsies are a rare but extremely debilitating group of disorders that are difficult to diagnose and even harder to treat. They represent a heterogeneous subgroup of those with secondary generalized epilepsy. Efficacy of treatment is often measured in terms of slowing a patient's inevitable decline.
Basim M., Uthman, Andreas, Reichl
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Neurophysiology of Juvenile and Progressive Myoclonic Epilepsy
Journal of Clinical Neurophysiology, 2022Summary: Myoclonus can be epileptic or nonepileptic. Epileptic myoclonus has been defined in clinical, neurophysiological, and neuroanatomical terms. Juvenile myoclonic epilepsy (JME) is typically considered to be an adolescent-onset idiopathic generalized epilepsy with a combination of myoclonic, generalized tonic–clonic, and absence ...
Jayant N, Acharya, Vinita J, Acharya
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Progressive Myoclonic Epilepsy
2012Myoclonic seizures are epileptic seizures in which the main manifestation is myoclonus. Myoclonus is accompanied by a generalized epileptiform discharge but the myoclonus may be generalized, segmental, or focal. Progressive myoclonic epilepsies (PMEs) are an unusual and heterogeneous group of epilepsies with debilitating progression, resistance to ...
Roongroj Bhidayasiri, Daniel Tarsy
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[Progressive myoclonic epilepsy].
Ugeskrift for laeger, 1996Progressive myoclonic epilepsy (PME) is a syndrome characterized by myoclonias, epilepsy, progressive dementia and other neurological deficits. PME may be caused by various, rare, incompletely elucidated genetic diseases, and is characterized by age at onset, duration, clinical and pathoanatomical abnormalities. There is ethnic and geographic variation
T T, Sørensen +2 more
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Progressive Myoclonic Epilepsy
2013A 19-year-old, right-handed female of mixed Caucasian and Mediterranean decent presented to an epilepsy clinic with a diagnosis of “seizure disorder.” At 14 years of age she experienced her first generalized tonic–clonic seizure. Subsequently, she developed multifocal myoclonus and was diagnosed with the Juvenile Myoclonic Epilepsy (JME) syndrome ...
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Levetiracetam in progressive myoclonic epilepsy
Neurology, 2004The authors conducted an open study of levetiracetam as add-on therapy in nine patients with well-defined progressive myoclonic epilepsies and refractory myoclonus. Myoclonus was evaluated semiquantitatively (territory, intensity, daily living activities). Five patients had improvement of their myoclonus score.
C, Crest +4 more
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