Disclosures: David G Olsen: Nothing to disclose

Case Description: The patient was found in cardiac arrest triggered by status asthmaticus in the setting of polysubstance abuse. She was resuscitated in the field and later intubated for respiratory failure and concurrent myoclonic status epilepticus. Once stabilized she continued to experience myoclonic seizure episodes, which were ultimately controlled with a regimen of divalproex sodium, levetiracetam, and clonazepam. She continued to demonstrate severe intention myoclonus in all extremities and vocal musculature and was diagnosed with chronic post-hypoxic myoclonus (Lance-Adams syndrome/LAS). Deficits were noted in cognition, dysphagia requiring PEG placement, dysarthria, coordination and muscular weakness.

Setting: Academic inpatient rehabilitation facility

Patient: 22-year-old female with severe chronic post-hypoxic myoclonus.

Assessment/Results: On admission to inpatient rehabilitation medications were uptitrated to 1.5 g divalproex sodium BID, 2 g levetiracetam BID, and clonazepam dosed TID for a total dose of 10 mg/day. Given concerns for mood aggravation levetiracetam was tapered, however worsening myoclonus was observed with functional decline. Levetiracetam was resumed at maximum dose and 50 mg pyridoxine QD was added with some mitigation of mood symptoms. Continued severe intention myoclonus limited progress with therapies. The patient did make modest improvements with dysarthria and swallow function, was weaned from tube feeds and PEG was removed. Despite aggressive pharmacologic treatment mobility remains limited and the patient is dependent for self-care and transfers with overhead lift.

Discussion: Diffuse anoxic injury to cortical, subcortical and spinal tract regions is known to produce severe chronic post-hypoxic myoclonus that is often resistant to pharmacotherapy. Due to the rare prevalence of this condition best practices and appropriate functional goals in the rehabilitation setting remain unknown.

Conclusion: While some existing case reports identify significant functional improvements in LAS with monotherapy, our current case illustrates a case of severely refractory post-hypoxic myoclonus despite high dose triple pharmacotherapy and intensive rehabilitation.

Level of Evidence: Level V

« Back to AAPM&R Annual Assembly 2019

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/triple-pharmacotherapy-in-the-treatment-of-refractory-chronic-post-hypoxic-myoclonus-lance-adams-syndrome-a-case-report/