Objective: To describe the clinical presentation and response to treatment of cases of Lance-Adams Syndrome in a Movement Disorders Clinic in El Salvador.

Background: Lance-Adams Syndrome is a chronic post-hypoxic myoclonus, it is a rare entity and a disabling complication with onset from days to weeks after a hypoxic brain injury. Spontaneous, action and reflex myoclonus are typically multifocal and may be accompanied by “bouncy gait”.

Method: We analyzed consecutive adult subjects referred to a movement disorder clinic from 1991 to 2020, all of them assessed by a movement disorder specialist, with video recording, Barthel Index scale and response to treatment.

Results: Six women had clinical presentation consistent with Lance-Adams Syndrome, with an average follow-up of 15 years (5-29 years). After starting monotherapy treatment and then polytherapy, all the patients presented progressive improvement of their symptoms, although in the following years, a reduction in the dose and number of medications was made. One patient presented complete remission of symptoms even after stopping medications. (Table 1 and Table 2).

Conclusion: Although Lance-Adams Syndrome is a non-epileptic condition, some antiepileptic medications are a relatively effective treatment. Early recognition and accurate diagnosis are important factors to decide the most effective intervention and to regain independence in activities of daily living.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/lance-adams-syndrome-a-case-series-in-el-salvador/