Lance-Adams Syndrome: Difference between revisions
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The acute post-hypoxic myoclonus presents within 24 hours of the event is often called myoclonic status epilepticus (MSE), which is one of the predictors for poor prognosis according to the American Academy of Neurology<ref name=":0">Yadavmali T, Lane A. The Lance-Adams Syndrome: Helpful or Just Hopeful, after Cardiopulmonary Arrest. J Intensive Care Soc. 2011 Oct 1;12:324–8 https://www.researchgate.net/publication/285909789_The_Lance-Adams_Syndrome_Helpful_or_Just_Hopeful_after_Cardiopulmonary_Arrest</ref> | The acute post-hypoxic myoclonus presents within 24 hours of the event is often called myoclonic status epilepticus (MSE), which is one of the predictors for poor prognosis according to the American Academy of Neurology<ref name=":0">Yadavmali T, Lane A. The Lance-Adams Syndrome: Helpful or Just Hopeful, after Cardiopulmonary Arrest. J Intensive Care Soc. 2011 Oct 1;12:324–8 https://www.researchgate.net/publication/285909789_The_Lance-Adams_Syndrome_Helpful_or_Just_Hopeful_after_Cardiopulmonary_Arrest</ref> | ||
The chronic post-hypoxic myoclonus which starts after a few days or weeks in patients who regained consciousness is often stated as LAS, where intentional myoclonus is accompanied when attempting voluntary movements or during a voluntary movement. The myoclonus usually subsides with rest or with relaxation. The severity of the myoclonus is very much related to the precision of the movement<ref name=":0" /> The myoclonus gets exaggerated with startle or emotional stress.<ref>The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy - PubMed [Internet]. [cited 2023 Jun 4]. Available from: https://pubmed.ncbi.nlm.nih.gov/13928398/</ref> | The chronic post-hypoxic myoclonus which starts after a few days or weeks in patients who regained consciousness is often stated as LAS, where intentional myoclonus is accompanied when attempting voluntary movements or during a voluntary movement. There have been few cases reported with acute onset LAS. The myoclonus usually subsides with rest or with relaxation. The severity of the myoclonus is very much related to the precision of the movement<ref name=":0" /> The myoclonus gets exaggerated with startle or emotional stress.<ref>The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy - PubMed [Internet]. [cited 2023 Jun 4]. Available from: https://pubmed.ncbi.nlm.nih.gov/13928398/</ref> | ||
=== Clinical Features === | |||
* Generalized, multifocal or focal myoclonus | |||
* Intentional myoclonus sometimes presents with spontaneous myoclonus | |||
* Limb involvement depends on the area of insult whether Cortical or subcortical | |||
* Coma in the acute phase due to sedation, otherwise awake, alert and cognition relatively preserved<br /> | |||
=== Neurophysiological Findings === | |||
'''EEG Findings'''<ref name=":1">Freund B, Kaplan PW. Post-hypoxic myoclonus: Differentiating benign and malignant etiologies in diagnosis and prognosis. Clin Neurophysiol Pract. 2017 Jan 1;2:98–102.https://www.sciencedirect.com/science/article/pii/S2467981X17300100#b0055</ref> | |||
* One of the three case of LAS presents with epileptiform activity | |||
* Spike or poly spike-wave discharges maximally or primarily at the vertex | |||
* EEG was found to be normal in between the myoclonic jerks in some cases | |||
'''SSEPs'''<ref name=":1" /> | |||
* Giant and normal-sized somatosensory evoked potentials (Freund et al., 2016) | |||
'''EEG-EMG Polygraphy'''<ref name=":1" /> | |||
* Demonstrated Jerk-locking noted in 60% of the cases (Freund et al., 2016) | |||
=== Medical Management === | |||
=== Physiotherapy Management === | |||
=== References === | === References === | ||
<references /> | <references /> | ||
Revision as of 20:10, 4 June 2023
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Introduction[edit | edit source]
James W Adams and Raymond D Adams described this as a syndrome characterized by intentional myoclonus as a sequel to hypoxic encephalopathy. Hypoxic encephalopathy can occur due to a cardiopulmonary arrest. Despite advanced practices in cardiopulmonary resuscitations, the outcomes after cardiopulmonary arrest seem to be poor and People after successful cardiopulmonary resuscitation often remain with some neurological deficits. LAS is reported very rarely less than 0.5 % in people who survives cardiac arrest. It is commonly confused with myoclonic status epilepticus (MSE) even though examination, imaging, level of disability and time of onset are very different [1]
The acute post-hypoxic myoclonus presents within 24 hours of the event is often called myoclonic status epilepticus (MSE), which is one of the predictors for poor prognosis according to the American Academy of Neurology[2]
The chronic post-hypoxic myoclonus which starts after a few days or weeks in patients who regained consciousness is often stated as LAS, where intentional myoclonus is accompanied when attempting voluntary movements or during a voluntary movement. There have been few cases reported with acute onset LAS. The myoclonus usually subsides with rest or with relaxation. The severity of the myoclonus is very much related to the precision of the movement[2] The myoclonus gets exaggerated with startle or emotional stress.[3]
Clinical Features[edit | edit source]
- Generalized, multifocal or focal myoclonus
- Intentional myoclonus sometimes presents with spontaneous myoclonus
- Limb involvement depends on the area of insult whether Cortical or subcortical
- Coma in the acute phase due to sedation, otherwise awake, alert and cognition relatively preserved
Neurophysiological Findings[edit | edit source]
EEG Findings[4]
- One of the three case of LAS presents with epileptiform activity
- Spike or poly spike-wave discharges maximally or primarily at the vertex
- EEG was found to be normal in between the myoclonic jerks in some cases
SSEPs[4]
- Giant and normal-sized somatosensory evoked potentials (Freund et al., 2016)
EEG-EMG Polygraphy[4]
- Demonstrated Jerk-locking noted in 60% of the cases (Freund et al., 2016)
Medical Management[edit | edit source]
Physiotherapy Management[edit | edit source]
References[edit | edit source]
- ↑ Wijdicks EFM, Hijdra A, Young GB, Bassetti CL, Wiebe S, Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter: prediction of outcome in comatose survivors after cardiopulmonary resuscitation (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2006 Jul 25;67(2):203–10 https://pubmed.ncbi.nlm.nih.gov/16864809/
- ↑ 2.0 2.1 Yadavmali T, Lane A. The Lance-Adams Syndrome: Helpful or Just Hopeful, after Cardiopulmonary Arrest. J Intensive Care Soc. 2011 Oct 1;12:324–8 https://www.researchgate.net/publication/285909789_The_Lance-Adams_Syndrome_Helpful_or_Just_Hopeful_after_Cardiopulmonary_Arrest
- ↑ The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy - PubMed [Internet]. [cited 2023 Jun 4]. Available from: https://pubmed.ncbi.nlm.nih.gov/13928398/
- ↑ 4.0 4.1 4.2 Freund B, Kaplan PW. Post-hypoxic myoclonus: Differentiating benign and malignant etiologies in diagnosis and prognosis. Clin Neurophysiol Pract. 2017 Jan 1;2:98–102.https://www.sciencedirect.com/science/article/pii/S2467981X17300100#b0055
