Lance–Adams syndrome explained

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.^{[1] [2] [3] [4]} It was first reported by James Lance and Raymond Adams in 1963.^{[5] [6]}

It is a disease that presents Myoclonus as a sequela of hypoxic disorders in the brain due to asphyxiation and cardiopulmonary arrest.^{[2] [3]} It is exacerbated by mental and physical anxiety such as intention, intentional movement, and tension.^{[2] [3]}

Pathology

It appears due to Basal ganglia lesions due to hypoxic encephalopathy.^[2]

Treatment

Clonazepam and Valproate, which enhance serotonin and GABAA receptor, are widely used as therapeutic agents .^{[2] [4] [7]} It has been reported that Levetiracetam was effective in cases in which clonazepam and valproic acid were ineffective.^{[8] [9] [10]} Other reports have shown that piracetam has been shown to be effective.^[11]

Perampanel

From around 2017, reports that Perampanel are effective have been gathered, and cases of complete cure have been reported.^{[12] [13] [14] [15] [16]}

In April 2021, a group at Kyoto University 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 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.^{[16] [17]}

Notes and References

1. 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, . . PMID 17910113. . (Review).
2. ベッドサイドの神経の診かた 改訂17版 P179 南山堂 ISBN 978-4525247171
3. 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
4. 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,
5. Lance JW, Adams RD . The Syndrome of Intention or Action Myoclonus as a Sequel to Hypoxic Encephalopathy . Brain . 86 . 111–136 . 1963 . 10.1093/brain/86.1.111. 13928398 .
6. 長山隆 . 山根清美 . 阿部裕光 . 島國義 . 高橋正宏 . 太田舜二 . 小林逸郎 . 竹宮敏子 . 丸山勝一 . Postanoxic Encephalopathy の1症例 ： 特に Lance-Adams症候群について . 東京女子医科大学雑誌 . 東京女子医科大学学会 . Oct 1984. 54 . 10 . 1112–1117 . 10470/5430 . . 0040-9022.
7. S. Frucht . S. Fahn . The clinical spectrum of posthypoxic myoclonus . Movement Disorders . 15 . Suppl 1 . 2000 . 2–7 . 10.1002/mds.870150702 . 0885-3185 . 10755265. 9811874 . (Review).
8. Krauss GL, Bergin A, Kramer RE, Cho YW, Reichi SG . Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam . Neurology . 2001 . 56 . 6 . 411–412 . 10.1212/WNL.57.6.1144-b. 11571362 . 5258397 .
9. P. Genton . P. Gélisse . Epileptic Disord . Antimyoclonic effect of levetiracetam. 2. 209–212. 2000. 4 . 10.1684/j.1950-6945.2000.tb00445.x . 11174151 . de.
10. G. L. Krauss . A. Bergin . R. E. Kramer . et al. Neurology. Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam . 56. 411–412. 2001. 3 . 10.1212/WNL.56.3.411 . 11171914 . 219206319 . de.
11. 林元久，木下喬弘，山川一馬，松田宏樹，藤見聡 . Sep 2017. ルプロ酸，クロナゼパムに抵抗性の Lance‒Adams 症候群におけ る薬物治療の検討 . 日本救急医学会雑誌 . 28 . 9. 623. . .
12. Goldsmith D, Minassian BA . 10.1016/j.yebeh.2016.06.041 . Efficacy and tolerability of perampanel in ten patients with Lafora disease . Epilepsy Behav . 2016 . 62 . 132–135. 27459034 . 5691360 .
13. Crespel A, Gelisse P, Tang NP, et al . Perampanel in 12 patient swith Unverricht-Lundborg disease . Epilepsia . 2017 . 58 . 543–547 . 10.1016/j.yebeh.2016.06.041. 27459034 . 5691360 .
14. Steinhoff BJ, Bacher M, Kurth C, et al . Add-on perampanel in Lance–Adams syndrome . Epilepsy Behav Case Rep . 2016 . 6 . 28–29 . 10.1016/j.ebcr.2016.05.001. 27437182 . 4939387 .
15. Shiraishi H, Egawa K, Ito T, et al . Efficacy of perampanel for controlling seizures and improving neurological dysfunction in a patient with dentatorubral-pallidoluysian atrophy (DRPLA) . Epilepsy Behav Case Rep . 2017 . 8 . 44–46 . 10.1016/j.ebcr.2017.05.004. 28856097 . 5565628 .
16. Daiji Saita . Satoru Oishi . Masanori Saito . Administration of a small dose of perampanel improves walking ability in a case of Lance–Adams Syndrome . Psychiatry and Clinical Neurosciences . 2022 . 76 . 3 . 89–89 . 10.1111/pcn.13320 . 34878204. 244954036 .
17. 齋藤和幸、大井和起、稲葉彰、小林正樹、池田昭夫、 和田義明 . Apr 2021. 長期経過で持続した Lance-Adams 症候群の重症ミオクローヌスにペランパネルが奏効した 1 例 . 臨床神経学 . 61 . 18–23 . . . 0009-918X . 2021-07-04.