Lance Adams Syndrome in the Setting of COVID-19 Pneumonia.

Lance-Adams syndrome (LAS) is a rare clinical presentation of hypoxic-ischemic brain injury typically occurring in the setting of cardiac arrest. It is rare for it to be associated with respiratory failure. The advent of the COVID-19 pandemic heralded a new cause of respiratory failure, and not much is known about the occurrence of Lance-Adams syndrome in the context of COVID-19 pneumonia. A 23-year-old male was brought to the emergency department (ED) after being found unconscious at home. He had prominent generalized myoclonus in the context of COVID-19 pneumonia and a possible clonazepam overdose. Magnetic resonance imaging (MRI) of the brain with and without contrast revealed findings suggestive of hypoxic-ischemic brain injury. A diagnosis of LAS was made based on electroencephalography (EEG). As LAS typically carries a relatively favorable prognosis, aggressive treatment was pursued. This resulted in a fairly good outcome, although he had to be maintained on several antiseizure medications. Our case is a rare occurrence of Lance-Adams syndrome in the setting of respiratory failure and COVID-19 pneumonia in the absence of cardiac arrest. It is critical to distinguish myoclonic status epilepticus (MSE) from Lance-Adams syndrome due to the difference in prognosis. Our case can provide future direction for studies in a larger cohort of patients to see if LAS is frequently associated with respiratory failure secondary to COVID-19 pneumonia in the absence of cardiac arrest. It is important to consider Lance-Adams syndrome as one of the emerging neurological complications of COVID-19 pneumonia.

Lance-Adams Syndrome in the Intensive Care Unit: A Case Report.

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. The presented case involves a 77-year-old male diagnosed with LAS post-CPR. Despite the presence of early myoclonic jerks EEG, laboratory testing, and neuroimaging showed no definitive proof of irreversible neurological damage. Once diagnosed, treatment involved sequential antiseizure medications and physical therapy when the patient achieved full consciousness. However, the patient ultimately faced severe disabilities and was unable to recover. This case report emphasizes the importance of limiting sedation, comprehensive clinical examination, and the use of complementary tests when no definitive proof of irreversible neurological damage is present after acute cerebral hypoxia. While LAS has a better vital prognosis than acute PHM, it is associated with poor neurofunctional recovery and chronic disability in most cases. Further research is essential for evidence-based management.

Lance-Adams Syndrome: Case series and literature review.

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. Electroencephalogram (EEG) pattern of midline spike-wave discharge associated with favorable prognosis. There is no consensus in treatment of LAS but use of various anti-epileptic medications has been documented in literature. Case Presentation: In this case series, all of the patients presented after achieving return of spontaneous circulation (ROSC) and subsequently developed myoclonus. EEG findings beyond the initial hospitalization continued to show later showed changes consistent with LAS in three of the four patients. Different combinations of AEDs were used in the management of LAS with variable success and adverse effects. Conclusion: Our cases highlight that the characteristic EEG pattern can be useful in the diagnosis of LAS and allow for better prognostication and management in patients with posthypoxic myoclonus. We reviewed the available literature to better understand the prevalence, mechanism, clinical presentation, diagnosis, and management of LAS.

Post hypoxic myoclonus: A tale of two minds.

Post hypoxic myoclonus (PHM) is considered a poor prognostic sign and may influence decisions regarding withdrawal of treatment. PHM is generally categorized in literature as either acute or chronic (also commonly referred to as Lance-Adams Syndrome) based on the onset of myoclonus. However, it may be more accurate to differentiate between the various presentations of PHM based on the clinical characteristics and electroencephalogram (EEG) findings for prognostication. Here, we describe a case of a 33-year-old female who presented after a cardiopulmonary arrest. MRI of the brain and cervical spine on admission were unremarkable. Twelve hours later, she developed generalized, stimulus-sensitive myoclonus suggestive of acute PHM. Various medications were trialed, and her symptoms eventually improved on clonazepam. On day 14, she started having resting and intention myoclonus, and dysarthria, consistent with LAS. Several adjustments were again made to her regimen, and she was eventually switched from clonazepam to baclofen which improved her resting myoclonus. This case highlights that PHM can present differently and have a markedly different outcome. It is important to develop a better understanding of the various types of PHM so as to avoid premature withdrawal of care.

A Case of Lance Adams Syndrome in a Patient with Attempted Hanging.

Lance Adams syndrome is the term used to describe late post-hypoxic myoclonus. Here we describe a patient who developed action and intention myoclonus after 7 days of attempted partial hanging. The similarity of Lance Adams syndrome, which is a treatable condition to a cerebellar syndrome, and the diagnostic difficulties have been highlighted. How to cite this article: Subramanian M, Velayudham S, Jeyaraj M, Arunan S, Perumal S, Mohan K. A Case of Lance Adams Syndrome in a Patient with Attempted Hanging. Indian J Crit Care Med 2022;26(9):1052-1053.

A Case of Lance-Adams Syndrome with Mixed Cortical and Reticular Reflex Myoclonus.

Background: Lance Adams syndrome is a chronic post-hypoxic myoclonus. Phenomenology Shown: This video abstract illustrates Lance Adams Syndrome with mixed cortical and reticular reflex myoclonus in a 32-year-old woman following respiratory arrest in the setting of an asthma attack, as well as improvement in her exam following pharmacologic management. Educational Value: Lance Adams syndrome can include both cortical and reticular reflex myoclonus features while interdisciplinary intervention and pharmacological treatment can improve symptomatology.

Lance Adams syndrome: two cases report and literature review.

Hypoxic myoclonus, also known as Lance Adams syndrome, is a rare syndrome that results from the serious brain damage caused by cerebral hypoxia that often follows cardiopulmonary resuscitation. This current case report describes two patients with post-hypoxic myoclonus, both of whom received cardiopulmonary resuscitation. The neurological symptoms of these two patients were significantly improved by the administration of clonazepam and sodium valproate sustained-release tablets. The report presents a literature review detailing the pathogenesis, diagnosis and treatment of Lance Adams syndrome. The timely diagnosis and treatment of Lance Adams syndrome can significantly improve the quality of life of patients. Valproic acid, clonazepam and other antiepileptic drugs can be used. Whether levetiracetam is effective for cortical myoclonus requires further clinical study.

Lance-Adams syndrome: An unusual complication of snakebite envenomation.

Neuroparalytic snakebite envenomation is common in tropics and sub-tropics. The clinical history is sometimes unclear and misleading, leading to delay in diagnosis and initiation of life-saving treatments. It often gets so delayed that the patient may end up in neuromuscular respiratory failure. If sustained hypoxia occurs, a permanent neurological squeal may be the endpoint. We report a novel case of a 21-year-old previously healthy young Indian man who had a rapidly progressive neuroparalytic respiratory failure following a cobra bite for which he required mechanical ventilation for four days. After weaning successfully from the ventilator, he developed Lance-Adams syndrome, which persisted for over a month and eventually responded well to medical treatment.

A Rare Case of Lance-Adams Syndrome: Status Post-Successful Cardiopulmonary Resuscitation.

Lance-Adams syndrome (LAS), also known as chronic post-hypoxic myoclonus (PHM), is a rare condition that may present with intention myoclonus in a patient who has regained consciousness after cardiorespiratory arrest. This case report describes a patient who received successful cardiopulmonary resuscitation (CPR) after going into cardiac arrest. And regaining consciousness, the patient developed myoclonic jerks diagnosed as LAS. The patient responded well to treatment with clonazepam and physical rehabilitation.

Lance Adams Syndrome: A Rare Case Presentation of Myoclonus From Chronic Hypoxia Secondary to COVID-19 Infection.

Coronavirus disease 2019 (COVID-19) pandemic is caused by the severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) and since the outbreak, many neurological features and syndromes are reported with this multi-organ viral infection. Lance-Adams syndrome (LAS) also referred to as chronic post hypoxic myoclonus is defined as action myoclonus which can occur as generalized, focal, or multifocal repeated myoclonic motor movements which involve the face, trunk, or extremities and it is one of the neurological complications that are related to COVID-19 infection. LAS is reported as a delayed complication of cardiac arrest, which causes cerebral hypoxia leading to myoclonus. We report a case of a 58-year-old male patient diagnosed as a case of LAS secondary to hypoxia occurring because of COVID-19 without cardiac arrest and to the best of our knowledge it is the second case reported with this similar mechanism. Moreover, we discuss the possible pathophysiological relationship between LAS and COVID-19 and various treatment strategies. Eventually, we review the related articles in the literature regarding the LAS and various types of myoclonus associated with COVID-19 infection.

A Case Study of Lance-Adams Syndrome.

Lance-Adams syndrome (LAS) was first described in 1963 by Lance and Adams, who cited four cases of posthypoxic action myoclonus. Since then, less than 150 cases have been reported world-wide. LAS differs from acute posthypoxic myoclonus, which usually occurs within twelve hours of the anoxic event, with the patient remaining in a deep coma with an extremely poor prognosis. Patients with LAS retain neurological function and develop myoclonus days or weeks after their hypoxic event. This report describes the case of a 64-year-old male who developed LAS following cardiac arrest. His progression from ICU to outpatient was followed through multiple electroencephalograms.

Respuesta a perampanel en un paciente con mioclono posthipóxico crónico / Response to perampanel in a patient with chronic post-hypoxic myoclonus

Introducción: El mioclono posthipóxico crónico es un cuadro cuya clínica predominante son las mioclonías que acontecen tras un daño cerebral hipóxico, generalmente por parada cardiorrespiratoria. Es una entidad que se trata generalmente con fármacos antiepilépticos, con una modesta respuesta clínica en la mayoría de los casos. Caso clínico: Paciente que comienza con movimientos de sacudidas, compatibles con mioclonías de las cuatro extremidades y faciales al día siguiente de una parada cardiorrespiratoria recuperada. Se realizó un electroencefalograma durante el cual se registraron las mioclonías sin presentar correlato eléctrico. Durante el ingreso, y en sucesivas visitas tras el alta, se probaron diferentes tratamientos antiepilépticos para las mioclonías, que fueron refractarias y comportaron una afectación de la calidad de vida del paciente. Tras dos años de evolución, se inició tratamiento con perampanel hasta una dosis de 4 mg y el paciente refirió una mejoría clínica importante, evidenciada en consultas. Conclusiones: El perampanel puede suponer una alternativa eficaz para el tratamiento de las mioclonías en pacientes con mioclono posthipóxico crónico.(AU)

Introduction: Chronic post-hypoxic myoclonus is a condition in which the predominant clinical picture is myoclonus following hypoxic brain damage, usually due to cardiorespiratory arrest. It is a condition that is usually treated with antiepileptic drugs, in most cases with a modest clinical response. Case report: We report the case of a patient who started with jerking movements, compatible with myoclonus in the four limbs and the face the day after recovering from a cardiorespiratory arrest. An electroencephalogram was performed during which the myoclonias were recorded with no electrical correlates. During admission, and in successive visits after discharge, different antiepileptic treatments were tried for the myoclonias, which were refractory and affected the patient’s quality of life. Two years after onset, treatment with perampanel up to a dose of 4 mg was initiated and the patient reported a significant clinical improvement, as evidenced in the visits. Conclusions: Perampanel may be an effective alternative for the treatment of myoclonias in patients with chronic post-hypoxic myoclonus.(AU)

Lance-Adams Syndrome Treated by Perampanel in the Acute Term.

Lance-Adams syndrome (LAS) is chronic post-hypoxic myoclonus after a hypoxic encephalopathy. Recently, the report on LAS in the chronic term treated by perampanel (PER) is increasing. However, PER's efficacy in the "acute term" has not been reported. Here, we report an LAS patient who markedly improved when PER was added to his existing treatment regime in the acute term. The 65-year-old patient presented with a return of spontaneous circulation after cardiopulmonary arrest. He developed myoclonus on the admission day, and it led to tonic-clonic convulsion. We started levetiracetam 3000 mg/day, lacosamide 400 mg/day, general anesthesia using midazolam 180 mg/day, dexmedetomidine 1000 µg/day, and fentanyl 1.2 mg/day. We could stop the convulsions after 18 h from the onset. We tried to reduce sedatives, but his convulsion recurred. We added PER 2 mg/day for three days, PER 4 mg/day for next four days, then used PER 8 mg/day and we could gradually reduce the sedatives. Single-photon emission computed tomography on day 40 showed cerebral blood flow (CBF) increase at the bilateral anterior lobes of the cerebellum, medial temporal lobes, and supplementary motor and premotor areas, while CBF decrease at the brain surface of the frontal, parietal, and temporal lobes. The myoclonus disappeared since day 12, and he was transferred to another rehabilitation hospital on day 56. The optimal treatment strategy has not been established for LAS, but our case suggested that PER could be one of the choices to treat LAS in the acute term.

[A case of the successful treatment of severe myoclonus with Lance-Adams syndrome by add-on perampanel showing long term effects].

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.

Early risk stratification after resuscitation from cardiac arrest.

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. While current guidelines recommend against neurological prognostication in the first hours or days after return of spontaneous circulation, there are early findings suggestive of irrecoverable brain injury in which the patient would receive no benefit from transfer. In this Concepts article, we describe a simplified approach to quickly evaluate neurological status in cardiac arrest patients and identify findings concerning for irrecoverable brain injury. Characteristics of the arrest and resuscitation, initial neurological assessment, and brain computed tomography together can identify patients with high likelihood of irrecoverable anoxic injury. Patients who may benefit from centers with access to continuous electroencephalography are discussed. This approach can be used to identify patients who may benefit from rapid transfer to cardiac arrest centers versus those who may benefit from care close to home. Risk stratification also can provide realistic expectations for recovery to families.

Functional Neural Changes after Low-Frequency Bilateral Globus Pallidus Internus Deep Brain Stimulation for Post-Hypoxic Cortical Myoclonus: Voxel-Based Subtraction Analysis of Serial Positron Emission.

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. The patient required low-frequency stimulation to show myoclonus improvement. Using voxel-based statistical parametric mapping, we identified a decrease in glucose metabolism in the prefrontal lobe including the dorsolateral, orbito-, and inferior prefrontal cortex, which was suspected to be the origin of the myoclonus from postoperative PET/magnetic resonance imaging (MRI) after DBS. Based on the present study results, voxel-based subtraction of PET appears to be a useful approach for monitoring patients with PHM treated with DBS. Further investigation and continuous follow-up on the use of PET analysis and DBS treatment for patients with PHM are necessary to help understanding the pathophysiology of PHM, or LAS.

Treatment of Post-Hypoxic Myoclonus using Pallidal Deep Brain Stimulation Placed Using Interventional MRI Methods.

Background: Post-hypoxic myoclonus (PHM) is characterized by generalized myoclonus after hypoxic brain injury. Myoclonus is often functionally impairing and refractory to medical therapies. Deep brain stimulation (DBS) has been used to treat myoclonus-dystonia, but few cases of PHM have been described. Case report: A 33-year-old woman developed severe, refractory generalized myoclonus after cardiopulmonary arrest from drowning. We performed MRI-guided asleep bilateral pallidal DBS placement, resulting in improvement in action myoclonus at one year. Discussion: Our case contributes to growing evidence for DBS for PHM. Interventional MRI guided DBS technique can be used for safe and accurate lead placement. Highlights: We report a case of a patient who developed post-hypoxic myoclonus after cardiopulmonary arrest from drowning, who later underwent deep brain stimulation to treat refractory myoclonus. This is the first case to describe asleep, interventional MRI-guided technique for implanting DBS leads in post-hypoxic myoclonus.

Clinical Outcome and Intraoperative Neurophysiology of the Lance-Adams Syndrome Treated with Bilateral Deep Brain Stimulation of the Globus Pallidus Internus: A Case Report and Review of the Literature.

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. However, within 1 month, the patient developed sensory stimulation-induced myoclonus in her face and extremities. Because her myoclonic symptoms were refractory to pharmacotherapy, deep brain stimulation of the GPi was performed 1 year after the hypoxic attack. RESULTS: Continuous bilateral GPi stimulation with optimal parameter settings remarkably improved the patient's myoclonic symptoms. At the 2-year follow-up, her Unified Myoclonus Rating Scale score decreased from 90 to 24. In addition, we observed burst firing and interburst pause patterns on intraoperative microelectrode recordings of the bilateral GPi and stimulated this area as the therapeutic target. CONCLUSION: Our results show that impairment in the basal ganglion circuitry might be involved in the pathogenesis of myoclonus in patients with LAS.

Improvement of "Bouncy Gait" in Lance-Adams Syndrome with Perampanel.

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.