Background and aims Chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS), the two core neuroacanthocytosis syndromes, are progressive neurodegenerative conditions which underlie a wide spectrum of Huntington Disease-like neurological presentations. To date there are no data regarding life expectancy and causes of death in these rare disorders. Identifying causes of mortality is valuable for disease management and ultimately for clinical trials.

Methods We reviewed our personal databases and the published literature to identify cases of ChAc and MLS for whom adequate information was available regarding age of disease onset, age at death, cause of death, and clinical information such as presence of seizures or cardiac disease.

Results Adequate information was obtained on 55 patients with ChAc and 30 with McLeod syndrome. Causes of death included pneumonia, cardiac disease, seizure, suicide, and sepsis. Mean disease duration for ChAc was 11 years, while for McLeod syndrome it was 20 years.

Conclusions Causes of death in ChAc and McLeod syndrome are similar to those in Huntington’s disease, with additional risks due to the presence of seizures and cardiac disease. Suicidality was seen in 10% of patients with ChAc. Sudden unexplained deaths were frequently seeen. In the absence of disease-modifying agents, disease management should focus upon treating symptoms which may contribute to morbidity and mortality.