lance adams syndrome

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...

Emergency clinicians often resuscitate cardiac arrest patients, and after acute resuscitation, clinicians face multiple decisions regarding disposition. Recent evidence suggests that out-of-hospital cardiac arrest patients with return of spontaneous circulation have higher odds of survival to hospital discharge, long-term survival, and improved functional outcomes when treated at centers that can provide advanced multidisciplinary care. For community clinicians, a high volume cardiac arrest center may be hours away...

Post-hypoxic myoclonus (PHM) and Lance-Adams syndrome (LAS) are rare conditions following cardiopulmonary resuscitation. The aim of this study was to identify functional activity in the cerebral cortex after a hypoxic event and to investigate alterations that could be modulated by deep brain stimulation (DBS). A voxel-based subtraction analysis of serial positron emission tomography (PET) scans was performed in a 34-year-old woman with chronic medically refractory PHM that improved with bilateral globus pallidus internus (Gpi) DBS implanted three years after the hypoxic event...

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BACKGROUND: The Lance-Adams syndrome (LAS) is a myoclonus syndrome caused by hypoxic-ischemic encephalopathy. LAS cases could be refractory to first-line medications, and the neuronal mechanism underlying LAS pathology remains unknown. OBJECTIVES: To describe a patient with LAS who underwent bilateral globus pallidus internus (GPi) stimulation and discuss the pathophysiology of LAS with intraoperative electrophysiological findings. PATIENTS: A 79-year-old woman presented with a history of cardiopulmonary arrest due to internal carotid artery rupture following carotid endarterectomy after successful cardiopulmonary resuscitation...

Barth syndrome is a mitochondrial myopathy resulting from mutations in the tafazzin ( TAZ ) gene encoding a phospholipid transacylase required for cardiolipin remodeling. Cardiolipin is a phospholipid of the inner mitochondrial membrane essential for the function of numerous mitochondrial proteins and processes. However, it is unclear how tafazzin deficiency impacts cardiac mitochondrial metabolism. To address this question while avoiding confounding effects of cardiomyopathy on mitochondrial phenotype, we utilized Taz -shRNA knockdown ( TazKD ) mice, which exhibit defective cardiolipin remodeling and respiratory supercomplex instability characteristic of human Barth syndrome but normal cardiac function into adulthood...

No abstract text is available yet for this article.

Lance-Adams syndrome (LAS) is chronic post-hypoxic myoclonus that is often associated with sudden lapses in muscle tone (negative myoclonus) in the legs, causing a disabling "bouncy gait." Given its relative rarity, there are no controlled treatment studies of LAS. The majority of cases require polypharmacy management, with an incomplete response. "Bouncy gait," in particular, is notoriously medication-refractory. Here, we report a patient with long-standing LAS who improved markedly when low-dose perampanel was added to his existing treatment regime consisting of clonazepam, levetiracetam, sodium valproate, and acetazolamide...

Background: Treatment of posthypoxic myoclonus (PHM) can be a challenge in patients not responsive to first-line medications. PMH is a rare condition that has a dramatic impact on patients' quality of life. Refractory cases are not uncommon. Case report: We report a patient with PHM non-responsive to conventional treatments who showed a dramatic improvement with sodium oxybate (SBX). Cases of PHM treated with SBX reported in the literature were reviewed. Discussion: Resting and stimulus-induced myoclonus respond robustly to SBX, with significant improvement in patients' quality of life...

Objectives: Chronic post-hypoxic myoclonus, known as Lance-Adams syndrome (LAS), is a rare complication of successful cardiopulmonary resuscitation. It is characterized by intention myoclonus, cerebellar ataxia, and preserved intellect. The basis of the disease and its long-term prognosis remain unclear. Case report: The authors present a 53-year-old woman with a history of asthma bronchiale who suffered from myoclonus after hypoxic brain damage due to cardiac arrest. Advanced electrophysiological (quantitative EEG) and MR (MR spectroscopy) techniques were employed...

Lance-Adams syndrome (LAS) is an uncommon neurological disorder characterized by the development of chronic post-hypoxic myoclonus. There are relatively few cases described following successful cardiopulmonary resuscitation. We report a patient who developed LAS 3 months after successful resuscitation. Cerebral imaging studies indicate that brain pathology in LAS patients is not uniform, suggesting that the pathophysiology of myoclonus may vary from patient to patient. Our patient adds to this etiological heterogeneity by demonstrating the unusual feature of cerebellar pathology by both cerebral magnetic resonance imaging and single-photon emission computed tomography scans...

BACKGROUND: The prevalence of metabolic syndrome (MetS) generally rises with increasing adiposity, but tends to plateau at the highest levels of body mass index (BMI) with some individuals, even with severe obesity, expressing few or no components of MetS. We examined factors associated with the expression of MetS in severely obese women participating in a large observational study. METHODS: Anthropometrics, including Heath equation-adjusted bioimpedance-determined fat-free mass (FFM) and fat mass (FM), lipids and related laboratory measurements, resting energy expenditure (REE), and respiratory quotient (RQ), were studied in 949 women with severe obesity...

INTRODUCTION: Early myoclonus after cardiac arrest (CA) is traditionally viewed as a poor prognostic sign (status myoclonus). However, some patients may present early Lance-Adams syndrome (LAS): under appropriate treatment, they can reach a satisfactory functional outcome. Our aim was to describe their profile, focusing on pharmacologic management in the ICU, time to return of awareness, and long-term prognosis. METHODS: Adults with early LAS (defined as generalized myoclonus within 96h, with epileptiform EEG within 48h after CA) were retrospectively identified in our CA registry between 2006 and 2016...

The nematode Caenorhabditis elegans is well established as a system for characterization and discovery of molecular mechanisms mediating microbe-specific inducible innate immune responses to human pathogens. Coxiella burnetii is an obligate intracellular bacterium that causes a flu-like syndrome in humans (Q fever), as well as abortions in domesticated livestock, worldwide. Initially, when wild type C. elegans (N2 strain) was exposed to mCherry-expressing C. burnetii (CCB) a number of overt pathological manifestations resulted, including intestinal distension, deformed anal region and a decreased lifespan...

BACKGROUND: Myoclonus may occur after hypoxia. In 1963, Lance and Adams described persistent myoclonus with other features after hypoxia. However, myoclonus occurring immediately after hypoxia may demonstrate different syndromic features from classic Lance-Adams syndrome (LAS). The aim of this review is to provide up-to-date information about the spectrum of myoclonus occurring after hypoxia with emphasis on neurophysiological features. METHODS: A literature search was performed on PubMed database from 1960 to 2015...

Predicting the neurological outcome in survivors of cardiorespiratory arrest is difficult. A distinction has been made between acute and chronic posthypoxic myoclonus, called myoclonic status epilepticus and Lance-Adams syndrome (LAS), respectively, with the acute condition carrying a bad prognosis. Here, we report a case of a 37-year-old female who developed seizures after a successful cardiopulmonary resuscitation. The available literature on such cases is very rare and has generally mentioned a poor outcome...

Posthypoxic myoclonus (Lance-Adams syndrome) is characterized by myoclonus involving multiple muscle groups which is resistant to most conventional antiepileptic drugs. We present a case of hypoxic brain injury-induced myoclonic status epilepticus successfully controlled with isoflurane. The antimyoclonic effects of isoflurane are likely due to potentiation of inhibitory postsynaptic GABAA receptor-mediated currents and its effects on thalamocortical pathways. It is effective even when intravenous agents fail to control myoclonus...

BACKGROUND: Marfan syndrome (MFS) is a multisystem connective tissue disorder that can lead to aortic dilation requiring aortic root replacement. Neonatal MFS (nMFS) is a rare and severe form of MFS compared to classic MFS (cMFS). Aortic root histology in MFS is thought to demonstrate predominantly medial degeneration (MD) of a translamellar mucoid extracellular matrix accumulation (MEMA-T) vs. the intralamellar mucoid extracellular matrix accumulation (MEMA-I) seen in other aortopathies...

Perampanel (PER) is the first-in-class selective, noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist that has been licensed and marketed as antiepileptic drug (AED) indicated for patients with partial-onset and primary generalized tonic-clonic seizures. A positive effect was reported in some patients with epileptic myoclonic jerks in idiopathic generalized epilepsy and in progressive myoclonic epilepsy. We treated a male patient with posthypoxic nonepileptic myoclonus (Lance-Adams syndrome) with add-on PER and achieved an almost complete cessation of jerks...

Acute posthypoxic myoclonus portends a poor prognosis. Another form of posthypoxic myoclonus, Lance-Adams syndrome, is associated with a better outcome. Differentiating these two entities is important in prognostication and guiding further medical intervention. This can be difficult in the acute setting after hypoxic brain injury but the use of neurophysiologic studies may be helpful. In this article, we present a case of a patient who presented after pulseless electrical activity arrest, underwent targeted temperature management and subsequently developed Lance-Adams syndrome...