Perampanel is an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antagonist that has been marked as an antiepileptic drug for partial-onset and primary generalized tonic-clonic seizures. There have been some recent reports of perampanel being effective against cortical myoclonus by Lafora disease and Unverricht-Lundborg disease. We herein report a 49-year-old man who presented with myoclonus due to Lance-Adams syndrome (LAS) after cardiopulmonary arrest caused by a severe bronchial asthma attack. Perampanel was very effective against myoclonus induced by LAS even in the chronic state, over 10 years after the remote onset. Perampanel should be considered for the treatment of extremely refractory myoclonus due to LAS.
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Myoclonus After Cardiac Arrest: Need for Standardization-A Systematic Review and Research Proposal on Terminology.
Crit Care Med
November 2024
Department of Neurology, Neurocritical Care and Neurorehabilitation, Christian Doppler University Hospital, Paracelsus Medical University, Member of the European Reference Network EpiCARE, Salzburg, Austria.
Objectives: Although myoclonus less than or equal to 72 hours after cardiac arrest (CA) is often viewed as a single entity, there is considerable heterogeneity in its clinical and electrophysiology characteristics, and its strength of association with outcome. We reviewed definitions, electroencephalogram, and outcome of myoclonus post-CA to assess the need for consensus and the potential role of electroencephalogram for further research.
Data Sources: PubMed, Embase, and Cochrane databases.
The Use of Perampanel in the Treatment of Lance-Adams Syndrome.
J Epilepsy Res
December 2024
Department of Neurology, Comprehensive Epilepsy Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Lance Adams syndrome (LAS) is characterized by chronic action or intention myoclonus resulting from cerebral hypoxia. Perampanel, a non-competitive antagonist of aamino-3-hydroxy-5methyl-4 isooxazoleproprionic acid glutamate receptor, has demonstrated some efficacy in myoclonic epilepsy and other types of myoclonus. We report significant benefit in a patient with LAS treated with add on perampanel and provide a review of the relevant literature.
View Article and Find Full Text PDFRepetitive muscle silent periods in acute post-anoxic brain injury: A novel phenotype of negative myoclonus.
Clin Neurophysiol
January 2025
Epilepsy Center, Neurological Institute, University Hospitals Cleveland Medical Center, Cleveland, OH, USA; Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA. Electronic address:
Article Synopsis
- A study reports a new type of negative myoclonus observed in three patients with acute post-anoxic brain injury (PABI).
- The researchers analyzed EEG and muscle recordings, revealing a pattern of whole body tonic posturing accompanied by brief periods of muscle relaxation in two out of the three patients.
- The findings suggest that this phenomenon can be identified using sEMG and may involve cortical or reticular brain regions, contributing to our understanding of brain recovery after oxygen deprivation.
Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome.
Neurology
December 2024
From the Paris Brain Institute (G.V., E.A., P.B., V.N.), ICM, Inserm, CNRS, Sorbonne University; AP-HP (G.V., V.N.), EEG Unit, Department of Neurophysiology, Pitié-Salpêtrière Hospital; AP-HP (E.A.), Neurophysiology of Movement Disorders Unit, Department of Neurophysiology, Saint-Antoine and Pitié-Salpêtrière Hospital; AP-HP (M.A.D.R.Q., V.N.), Epilepsy Unit, Department of Neurology, Reference Center of Rare Epilepsies, ERN-EpiCare, Pitié-Salpêtrière Hospital; AP-HP (D.V.C., A.K.), Department of Nuclear Medicine, Pitié-Salpêtrière Hospital, APHP Sorbonne University; Sorbonne University (A.K.), Inserm, CNRS, Laboratoire D'Imagerie Biomédicale, LIB, Paris, France.
Background And Objectives: Lance-Adams syndrome (LAS), or chronic posthypoxic myoclonus, is a long-term disabling neurologic disorder occurring in survivors of anoxia. The cortical or subcortical origin of this myoclonus is unclear. We aimed to identify the neuroanatomical origin of myoclonus in LAS.
View Article and Find Full Text PDFObjective: Lance-Adams syndrome is a rare and debilitating disorder characterized by successful cardiopulmonary resuscitation resulting in myoclonus activity. Alcohol withdrawal seizures from alcohol use disorder may further exacerbate Lance-Adams syndrome. We aim to present a case of Lance-Adams syndrome complicated by alcohol withdrawal seizures and successfully treated with a combination of valproate, clonazepam, and gabapentin.
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