Lance-Adams Syndrome
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Lance-Adams syndrome (LAS) is a sequela of hypoxic encephalopathy due to respiratory arrest, airway obstruction, cardiac arrest, etc. , several days after the onset of hypoxic encephalopathy. A condition that presents with functional myoclonus associated with increased cortical excitability in a few weeks.Y. X. Zhang, J. R. Liu u. a.: ''Lance-Adams syndrome: a report of two cases.'' In: ''Journal of Zhejiang University. Science. B.'' Band 8, Nummer 10, Oktober 2007, S. 715–720, . doi:10.1631/jzus.2007.B0715. PMID 17910113. . (Review).ベッドサイドの神経の診かた 改訂17版 P179 南山堂 ISBN 978-4525247171神経診察クローズアップ-正しい病巣診断のコツ 改訂2版 P104 メジカルビュー社 ISBN 978-4758303804星野愛, 熊田聡子, 横地房子, 八谷靖夫, 花房由季子, 冨田直, 沖山亮一, 栗原栄二, 「 十分量のpiracetam投与が有効であったLance-Adams症候群の1例 」『脳と発達』 2009年 41巻 5号 pp. 357-360, It was first reported by
James Lance James Frederick Grenville Lance (born 29 September 1974) is an English actor, best known for his appearances in a number of British comedy series and the British-American comedy series ''Ted Lasso'' for which he was nominated for a Primetime E ...
and Raymond Adams in 1963. It is a disease that presents
Myoclonus Myoclonus is a brief, involuntary, irregular (lacking rhythm) twitching of a muscle or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus (myo "muscle", clonic "jerk") describes a medical sign and, generally, is ...
as a sequela of hypoxic disorders in the brain due to asphyxiation and cardiopulmonary arrest. It is exacerbated by mental and physical anxiety such as intention, intentional movement, and tension.


Pathology

It appears due to
Basal ganglia The basal ganglia (BG), or basal nuclei, are a group of subcortical nuclei, of varied origin, in the brains of vertebrates. In humans, and some primates, there are some differences, mainly in the division of the globus pallidus into an extern ...
lesions due to hypoxic encephalopathy .


Treatment

Clonazepam Clonazepam, sold under the brand names Klonopin and Rivotril, is a medication used to prevent and treat seizures, panic disorder, anxiety disorders, and the movement disorder known as akathisia. It is a tranquilizer of the benzodiazepine clas ...
and
Valproate Valproate (VPA) and its valproic acid, sodium valproate, and valproate semisodium forms are medications primarily used to treat epilepsy and bipolar disorder and prevent migraine headaches. They are useful for the prevention of seizures in those ...
, which enhance
serotonin Serotonin () or 5-hydroxytryptamine (5-HT) is a monoamine neurotransmitter. Its biological function is complex and multifaceted, modulating mood, cognition, reward, learning, memory, and numerous physiological processes such as vomiting and vas ...
and
GABAA receptor The GABAA receptor (GABAAR) is an ionotropic receptor and ligand-gated ion channel. Its endogenous ligand is γ-aminobutyric acid (GABA), the major inhibitory neurotransmitter in the central nervous system. Upon opening, the GABAA receptor on ...
, are widely used as therapeutic agents . It has been reported that
Levetiracetam Levetiracetam, sold under the brand name Keppra among others, is a medication used to treat epilepsy. It is used for partial-onset, myoclonic, or tonic–clonic seizures and is taken either by mouth as an immediate or extended release formul ...
was effective in cases in which clonazepam and valproic acid were ineffective. Other reports have shown that
piracetam Piracetam is a drug marketed as a treatment for myoclonus. It is also used as a cognitive enhancer to improve memory, attention, and learning. Evidence to support its use is unclear, with some studies showing modest benefits in specific populat ...
has been shown to be effective.


Perampanel

From around 2017, reports that
Perampanel Perampanel, sold under the brand name Fycompa, is an anti-epileptic medication developed by Eisai Co. that is used in addition to other drugs to treat partial seizures and generalized tonic-clonic seizures for people older than twelve years. I ...
are effective have been gathered, and cases of complete cure have been reported. In April 2021, a group at
Kyoto University , mottoeng = Freedom of academic culture , established = , type = National university, Public (National) , endowment = ¥ 316 billion (2.4 1000000000 (number), billion USD) , faculty = 3,480 (Teaching Staff) , administrative_staff ...
reported a case of Lance Adams syndrome 11 years after onset due to hypoxic encephalopathy caused by a bronchial asthma attack, and in March 2022, a group at
Kitasato University is a private medical university headquartered in Shirokane, Minato, Tokyo, Japan. The head of the university is on the Shirokane campus, neighboring the original Kitasato Institute, the first private medical research facility in Japan which was ...
reported a case of Lance Adams syndrome after a hanging neck injury 1 year and 6 months after onset. There are an increasing number of reports showing improvement even in chronic cases, such as a case report of a patient who was able to hold a standing position after treatment with perampanel and significant improvement in motor myoclonus.


References

{{DEFAULTSORT:lanceadamssyndrome Medical signs Brain disorders Extrapyramidal and movement disorders Neurological disorders Neurology Wikipedia neurology articles ready to translate