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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.

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Treatment of refractory post-hypoxic myoclonus and focal epilepsy with subthalamic nuclei deep brain stimulation.

Parkinsonism Relat Disord

October 2024

Department of Neurology, University of Texas Health Science Center at Houston, McGovern Medical School, 6431 Fannin St, Houston, TX, 77030, USA. Electronic address:

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Lance-Adams syndrome (LAS), or chronic post-hypoxic myoclonus, is a myoclonic disorder following acute cerebral hypoxia after successful cardiopulmonary resuscitation (CPR). LAS is distinct from acute post-hypoxic myoclonus (acute PHM), presenting with myoclonic jerks and cerebellar ataxia after regaining consciousness. However, the overlap at the onset complicates differentiation and may lead to the withdrawal of life-sustaining measures, especially in sedated ICU patients.

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We discuss two recent cases from our hospital in which two patients with ESKD receiving periodical hemodialysis (HD) and SarS-Cov-2 infection suffered movement disorders, being the onset related to the HD sessions in both. First case is a 78 year-old woman who is admitted with generalized myoclonic status epilepticus and second case is a 46 year-old male who starts repeatedly suffering myoclonus during his hemodialysis sessions on day +10 after testing positive (asymptomatic infection). There are two main hypotheses when it comes to myoclonus and CNS disorders in COVID19, post-hypoxic origin and inmunomediated postinfectious origin.

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Background: Lance-Adams Syndrome (LAS) is a rare complication of successful cardiopulmonary resuscitation (CPR). It is a form of posthypoxic myoclonus characterized by action or intention myoclonus developing days to months after an hypoxic insult to the brain. LAS, especially early in a patient's clinical course, can be challenging to diagnose.

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