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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.
UNASSIGNED: 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.

Lance-Adams syndrome (LAS) is a rare complication of successful cardiopulmonary resuscitation, often accompanied by action myoclonus. Myoclonus may occur as generalized, focal, or multifocal movements and can include the face, trunk, and/or extremities. Only 100 cases of LAS have been reported worldwide. Here, we present the case of a 53-year-old female who had a cardiac arrest event after being admitted for posterior cervical wound dehiscence management following a posterior cervical fusion from C3-T1. The patient was successfully resuscitated but developed action myoclonus in all extremities shortly after. Anoxic brain injury and myoclonus led to debilitation and prolonged hospital stay. During her inpatient stay, she was treated with clonazepam, levetiracetam, and sodium valproate with mild improvement.

Speech-induced action myoclonus may occur as a component of a generalized myoclonus syndrome. However, it may also present in isolation, or with a paucity of other findings, and be diagnostically challenging.
To report a retrospective case series of restricted speech-induced action myoclonus.
We reviewed cases of speech-induced action myoclonus evaluated at Mayo Clinic Rochester from 1989 to 2020. We eliminated cases where a more generalized myoclonic disorder was also present. Clinical, imaging, and electrophysiologic data were extracted.
Four cases were identified in which speech-induced action myoclonus of craniofacial muscles was the predominant clinical presentation. All described cranial muscle twitching induced by speaking, and two cases also reported speech interruptions. Diagnosis was confirmed by expert speech pathologists in all cases. Diagnostic aids included modulation with different speech tasks and speaking rates, and surface electrophysiology which confirmed craniofacial myoclonus induced by speaking tasks (three cases). Previous misdiagnosis included functional, dystonic, neuromuscular junction pathology, or hemifacial spasm. Two cases had isolated speech-induced myoclonus, and the other two had coexistent upper limb tremor. Potential etiologic factors were identified in three cases - medication (2), epilepsy (1) - while in one patient no cause was identified. One patient partially improved with anti-myoclonic medication and speech therapy.
Speech-induced action myoclonus may occur in isolation and is frequently misdiagnosed. Diagnostic aids include modulation with different speech tasks and speaking rates, and surface electrophysiology.

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.

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. Conclusions: Over long-term observation the results suggested permanent synaptic rearrangements of the neuronal networks due to brain plasticity in the patient after the brain hypoxia.

UNASSIGNED: Kufor-Rakeb syndrome (KRS) is a rare autosomal recessive neurologic disease with diverse phenotypic features. Herein we report an Iranian KRS family with seizure and action myoclonus in addition to other typical manifestations of this syndrome.
UNASSIGNED: All family members underwent careful neurologic examination. Exome sequencing was performed and ATP13A2 variation genotyped in all family members.
UNASSIGNED: Cognitive deficits, hypokinesia, rigidity, spasticity, brisk deep tendon reflexes, upward gaze palsy, tremor, and facial-faucial-finger mini-myoclonus were the common manifestations of all affected siblings. Two cases had seizure and the most severely affected sibling demonstrated severe action myoclonus. Exome sequencing identified a homozygous nonsense mutation c.2455C>T;p.Arg819* in ATP13A2 gene.
UNASSIGNED: We reported five KRS affected siblings who manifested myoclonus and seizure. The most severely affected one demonstrated action myoclonus, which has not been reported so far.

Chronic posthypoxic action myoclonus is usually recognized as multifocal or generalized and, in most cases, causes severe disability. It is also commonly associated with other neurological symptoms, such as ataxia. We report two cases of focal predominant postanoxic action myoclonus and review the limited relevant literature. The first case presented with action and stimulus sensitive myoclonus in the lower limbs that caused him limited disability. He had not received any diagnosis in almost 2 years. The second patient exhibited predominantly focal facial myoclonus, although she was more limited by dysphasia and hemiplegia as a consequence of an ischemic stroke. Both patients responded to antiepileptic drugs. We found 9 of 159 previously reported cases with this focal distribution. Physicians should be aware of this limited phenotype given that the unusual focal nature of the movements may result in incorrect diagnosis and treatment.

BACKGROUND: To explore the cortical network sustaining action myoclonus and to found markers of the resulting functional impairment, we evaluated the distribution of the cortico-muscular coherence (CMC) and the frequency of coherent cortical oscillations with magnetoencephalography (MEG). All patients had EPM1 (Unverricht-Lundborg) disease known to present with prominent and disabling movement-activated myoclonus.
METHODS: Using autoregressive models, we evaluated CMC on MEG sensors grouped in regions of interests (ROIs) above the main cortical areas. The movement was a repeated sustained isometric extension of the right hand and right foot. We compared the data obtained in 10 EPM1 patients with those obtained in 10 age-matched controls.
RESULTS: As expected, CMC in beta band was significantly higher in EPM1 patients compared to controls in the ROIs exploring the sensorimotor cortex, but, it was also significantly higher in adjacent ROIs ipsilateral and contralateral to the activated limb. Moreover, the beta-CMC peak occurred at frequencies significantly slower and more stable frequencies in EPM1 patients with respect to controls. The frequency of the beta-CMC peak inversely correlated with the severity of myoclonus.
CONCLUSIONS: the high and spatially extended beta-CMC peaking in a restricted range of low-beta frequencies in EPM1 patients, suggest that action myoclonus may result not only from an enhanced local synchronization but also from a specific oscillatory activity involving an expanded neuronal pool. The significant relationship between beta-CMC peak frequency and the severity of the motor impairment can represent a useful neurophysiological marker for the patients\' evaluation and follow-up.

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. However, our patient was successfully managed and showed clinical features of LAS. Thus, making an early diagnosis and proper management of hypoxic brain insults is positively related to improving the patient\'s functional outcome.