Poster 12: Lance-Adams Syndrome: Effective Treatment and Functional Improvement with Phenytoin. A Case Report

Article Outline

• Disclosures
• Patients or Programs
• Program Description
• Setting
• Results
• Discussion
• Conclusions
• Copyright

Keywords: Rehabilitation, Myoclonus, Anoxia

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Disclosures 

D. S. Rosenblum, None.

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Patients or Programs 

One patient.

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Program Description 

Rehabilitation and pharmacological options reviewed and described.

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Setting 

Outpatient and inpatient.

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Results 

Post-hypoxic myoclonus (PHM) was significantly improved, and functional gains were realized.

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Discussion 

PHM, or Lance-Adams syndrome, is a rare and difficult to treat condition that is characterized by involuntary muscular jerking movements in people who have sustained a cerebral anoxic event. Both the incidence and pathogenesis have not clearly been established, and little is known about effective treatments. This report is unusual in that it describes the use of phenytoin as a successful treatment for PHM. A 56-year-old man sustained a cardiac arrest and anoxic brain injury. He developed PHM, which was severe, and rendered him dependent for feeding, washing, bathing and mobility. EEG was negative for seizure, and further work up with MRI was unrevealing. A trial of valproic acid was ineffective and was stopped secondary to resultant leukopenia and thrombocytopenia. Clonazepam was ineffective. A trial of levetiracetam was also unsuccessful in improving function and decreasing the myoclonus. His management continued at home where he was totally dependent for self care and feeding due to the PHM. He was able to ambulate up to 10 feet with a rolling walker with moderate assistance of 2, and he required moderate assist of 2 to transfer. As an outpatient, phenytoin was started and, when it became therapeutic, the PHM started to improve remarkably. He was then admitted for intensive rehabilitation. Upon discharge, his PHM was significantly attenuated, and he was ambulating 18 feet a time with supervision and a rolling walker, he was supervision for his transfers, and he was feeding himself independently- all without significant PHM.

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Conclusions 

Action myoclonus will be reviewed- its differentiating characteristics, reported pathological changes, proposed mechanisms of action, natural history and treatment options. Medications and intensive inpatient rehabilitation can lead to improved function and quality of life for those struggling with PHM.