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Final Program

1348 - Severe Intention Myoclonus in a Young Patient Found Down: A case of Lance Adams Syndrome

Thursday, February 27, 2025
5:00 PM - 6:30 PM MST

    Presenting Author(s)

  • LL

    Lilly Lerer, MD

    Resident
    Northwestern University
    Chciago, Illinois, United States

    • Co-Author(s)

  • CR

    Christopher D. Reger, MD

    Attending Physician
    Shirley Ryan Ability Lab
    Chicago, Illinois, United States

Case Diagnosis: 26 year old female with a past medical history of relapsing remitting MS on periodic infusions, past psychiatric history of polysubstance use and depression with previous psychiatric hospitalizations, developed generalized myoclonic jerking after being found down for an unknown period of time in an Emergency Department waiting room. Patient was intubated and sedated with resolution of jerking. Urine toxicity positive for alcohol and cocaine. CT head was unremarkable. MRI brain showed stable MS lesions. EEG showed generalized periodic discharges, and 3-4 hz spikes when stimulated that resolved at rest. Patient regained consciousness and was extubated. Patient was treated with clonazepam 1.5 mg every 8 hours with improvement in symptoms, however significant intention myoclonus persisted. Myoclonic jerks were absent at rest, present with intention, and exacerbated by anxiety and distress. Patient was diagnosed with Lance Adams Syndrome.

Case Description:

On admission to an acute inpatient rehabilitation hospital, the patient required maximum assistance with transfers and ambulation. Jerks in facial and oral distribution caused dysarthria and dysphagia, in legs caused weakness and impaired motor planning, in arms and hands caused impaired coordination and inability to perform self-care tasks. She made functional improvements and required supervision and minimal assistance for mobility and self-care at discharge. She started abilify for depressed mood. Mindfulness and psychological treatment were important aspects of her care. 

Discussions:

Lance Adams Syndrome occurs after hypoxic brain injury and presents with intention myoclonus. Facial involvement appears to be common. EEG can help distinguish the syndrome from focal seizure, and consciousness is preserved. Interdisciplinary care with psychology is recommended as symptoms appear to worsen with subjective distress. 

Conclusions:

Patients with Lance Adams Syndrome can significantly improve function in the acute inpatient rehabilitation setting. Treatment emphasizing psychosocial support may be particularly useful.