Lance-Adams Syndrome: Respiratory Acidosis Precedes Anoxia in All Cases
Kevin Duque1, Kelsey McDonald2, Abhimanyu Mahajan1, Alberto Espay1
1James J. and Joan A. Gardner Family Center for Parkinson’s Disease and Movement Disorders, Department of Neurology, College of Medicine, 2College of Medicine, University of Cincinnati
Objective:

To determine whether additional respiratory acidosis or hypercapnia is necessary prior to hypoxia to induce Lance-Adams syndrome (LAS).

Background:
Chronic post-hypoxic myoclonus or LAS is a disabling complication of patients surviving a cardiac arrest. However, most cardiac arrests are not followed by LAS.
Design/Methods:

We used Epic’s SlicerDicer tool and searched the 19,372 video records of the Movement Disorders Center at the University of Cincinnati to capture all LAS patients evaluated between 01/01/2000 and 08/01/2024. SlicerDicer identified 4,009 patients with a diagnosis or history of myoclonusof whom 770 were examined by a movement disorders neurologist and were manually reviewed. We collected demographic and clinical data, laboratory results and electroencephalography and brain MRI reports.

Results:
After reviewing the patients’ charts and video records, 17 patients with LAS were identified (10 females [6 African American and 4 White] and 7 males [1 African American and 6 White]). The mean age at cardiorespiratory arrest (n=13) or acute severe respiratory failure (n=4) was 47.8±17.4 years (range, 20–78). The average duration of the arrest was 14.0±10.8 minutes, and of the hospitalization among all patients was 23±10.7 days. Among the 12 patients with blood gases measured within 1.5 hours after the arrest, all showed primary respiratory acidosis and acidemia (pH<7.35; 7.06±0.16; range, 6.75–7.29), and 7 showed hypercapnia (pCO2>45 mmHg; 70.5±18.5; 55–92). Five patients showed normocapnia (pCO2, 35–45) and primary metabolic acidosis (HCO3-, 14.5±2.7; 10.5–16.4 mmol/L) in addition to respiratory acidosis. The remaining 5 patients without blood gas reports had respiratory arrest preceding cardiac arrest due to choking on food (n=2), septic shock, bacterial pneumonia, and pneumonia with recurrent pleural effusion. No other consistent abnormalities were found in serology, electroencephalograms or brain MRI. 
Conclusions:
Isolated anoxia is insufficient for LAS. A pre-cardiac arrest period of respiratory insufficiency may be required before onset of anoxia in all patients with LAS. 
10.1212/WNL.0000000000212464
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.