Lance-Adams syndrome

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.[4][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]

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, ISSN 1673-1581. doi:10.1631/jzus.2007.B0715. PMID 17910113. PMC 1997224. (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, doi:10.11251/ojjscn.41.357
  5. Lance JW, Adams RD (1963). "The Syndrome of Intention or Action Myoclonus as a Sequel to Hypoxic Encephalopathy". Brain. 86: 111–136. doi:10.1093/brain/86.1.111. PMID 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 (Oct 1984). "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. 54 (10): 1112–1117. hdl:10470/5430. ISSN 0040-9022. NAID 120002357826.
  7. S. Frucht; S. Fahn (2000). "The clinical spectrum of posthypoxic myoclonus". Movement Disorders. 15 (Suppl 1): 2–7. doi:10.1002/mds.870150702. ISSN 0885-3185. PMID 10755265. S2CID 9811874. (Review).
  8. Krauss GL, Bergin A, Kramer RE, Cho YW, Reichi SG (2001). "Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam". Neurology. 56 (6): 411–412. doi:10.1212/WNL.57.6.1144-b. PMID 11571362. S2CID 5258397.
  9. P. Genton; P. Gélisse (2000). "Antimyoclonic effect of levetiracetam". Epileptic Disord (in German). 2 (4): 209–212. PMID 11174151.
  10. G. L. Krauss; A. Bergin; R. E. Kramer; et al. (2001). "Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam". Neurology (in German). 56 (3): 411–412. doi:10.1212/WNL.56.3.411. PMID 11171914. S2CID 219206319.
  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. 東京都文京区: 日本救急医学会. 28 (9): 623.
  12. Goldsmith D, Minassian BA (2016). "Efficacy and tolerability of perampanel in ten patients with Lafora disease". Epilepsy Behav. 62: 132–135. doi:10.1016/j.yebeh.2016.06.041. PMC 5691360. PMID 27459034.
  13. Crespel A, Gelisse P, Tang NP, et al. (2017). "Perampanel in 12 patient swith Unverricht-Lundborg disease". Epilepsia. 58: 543–547. doi:10.1016/j.yebeh.2016.06.041. PMC 5691360. PMID 27459034.
  14. Steinhoff BJ, Bacher M, Kurth C, et al. (2016). "Add-on perampanel in Lance-Adams syndrome". Epilepsy Behav Case Rep. 6: 28–29. doi:10.1016/j.ebcr.2016.05.001. PMC 4939387. PMID 27437182.
  15. Shiraishi H, Egawa K, Ito T, et al. (2017). "Efficacy of perampanel for controlling seizures and improving neurological dysfunction in a patient with dentatorubral-pallidoluysian atrophy (DRPLA)". Epilepsy Behav Case Rep. 8: 44–46. doi:10.1016/j.ebcr.2017.05.004. PMC 5565628. PMID 28856097.
  16. Daiji Saita; Satoru Oishi; Masanori Saito (2022). "Administration of a small dose of perampanel improves walking ability in a case of Lance-Adams Syndrome". Psychiatry and Clinical Neurosciences. 76 (3): 89–89. doi:10.1111/pcn.13320. PMID 34878204. S2CID 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" (PDF). 臨床神経学. 東京都文京区: 日本神経学会. 61: 18–23. ISSN 0009-918X. Retrieved 2021-07-04.
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