Lance Adams Syndrome

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Classification according to ICD-10
G40.5 Special epileptic syndromes
ICD-10 online (WHO version 2019)

The Lance-Adams syndrome ( LAS ) is a neurological disorder image by the appearance of action myoclonus few days to weeks after a lack of oxygen had taken place of the brain is characterized. The lack of oxygen is usually the result of cardiovascular arrest with subsequent resuscitation and coma . Lance-Adams syndrome is also known as chronic posthypoxic myoclonus syndrome to differentiate it from acute posthypoxic myoclonus syndrome .

The syndrome is named after the first person to describe a total of 4 cases in 1963: the neurologist James Waldo Lance from Australia and Raymond Delacy Adams from the USA.

frequency

Overall, the disease is relatively rare. The exact frequency is not known. Just over 120 cases have been described in the medical literature to date.

Symptoms

Lance-Adams syndrome is characterized by action myoclonia that occurs a few days to weeks after an oxygen deficiency in the brain ( cerebral hypoxia ), i.e. posthypoxic. Action myoclonia are rapid, involuntary muscle contractions that are triggered by voluntary movements.

root cause

Lance Adams syndrome is a result of cerebral hypoxia . Accordingly, it often occurs after complications of anesthesia , asthma attacks , drug intoxication or heart attacks with cardiovascular arrest and resuscitation .

Posthypoxic myoclonia can generally be seen in patients who are or have been in a coma. The chronic posthypoxic myoclonus occurring in the context of Lance-Adams syndrome occur a few days to weeks after a coma. In contrast to this, acute posthypoxic myoclonus occurs immediately or a few days after hypoxia and has a poor prognosis , especially when the status is myoclonicus , i.e. when myoclonus is continuous or persistent .

therapy

The following drugs have been shown to be effective in about 50 percent of patients with Lance-Adams syndrome: clonazepam , valproic acid , piracetam, and levetiracetam .

Individual evidence

  1. ↑ a b c d e Y. X. Zhang, JR Liu et al: Lance-Adams syndrome: a report of two cases. In: Journal of Zhejiang University. Science. B. Volume 8, Number 10, October 2007, pp. 715-720, ISSN  1673-1581 . doi: 10.1631 / jzus.2007.B0715 . PMID 17910113 . PMC 1997224 (free full text). (Review).
  2. ↑ JW Lance, RD Adams: The syndrome of intention or action myoclonus as a sequel to hypoxic encephalopathy. In: Brain: a journal of neurology. Volume 86, March 1963, pp. 111-136, ISSN  0006-8950 . PMID 13928398 .
  3. ^ KJ Werhahn: Myoclonus. In: B. Schmitz, B. Tettenborn: Paroxysmal disorders in neurology. Springer Verlag , 2004, ISBN 3-540-40789-8 .
  4. ↑ S. Frucht, S. Fahn: The clinical spectrum of posthypoxic myoclonus. In: Movement disorders: official journal of the Movement Disorder Society. Volume 15, Suppl 1, 2000, pp. 2-7, ISSN  0885-3185 . PMID 10755265 . (Review).
  5. ↑ P. Genton, P. Gélisse: Antimyoclonic effect of levetiracetam. In: Epileptic Disord . tape 2 , 2000, pp. 209-212 .
  6. ↑ GL Krauss, A. Bergin, RE Kramer et al.: Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam. In: Neurology . tape 56 , 2001, p. 411-412 .