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Life expectancy and mortality in chorea-acanthocytosis and McLeod syndrome

Ruth H Walker1, Marcelo Miranda2, Hans H Jung3

  • 1Department of Neurology, James J. Peters Veterans Affairs Medical Center, Bronx, NY, USA; Department of Neurology, Mount Sinai School of Medicine, New York, NY, USA.

Parkinsonism & Related Disorders
|September 25, 2018
PubMed

Insights

Life expectancy and causes of death in chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS) were documented. Pneumonia, cardiac disease, and seizures are leading causes of mortality in these rare neurodegenerative disorders.

Area of Science:

  • Neuroscience
  • Genetics
  • Clinical Medicine

Background:

  • Chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS) are rare, inherited neurodegenerative disorders.
  • These conditions share phenotypic similarities with Huntington's disease, necessitating a clear understanding of their specific mortality profiles.

Purpose of the Study:

  • To document life expectancy and causes of death in patients with ChAc and MLS.
  • To inform disease management strategies and future clinical trial design.

Main Methods:

  • A comprehensive review of personal databases and published literature was conducted.
  • Cases with adequate information on disease onset, death, and clinical details were identified for ChAc and MLS.

Main Results:

  • Data were obtained for 52 ChAc patients and 34 MLS patients.
  • Common causes of death included pneumonia, cardiac disease, seizures, suicide, and sepsis.
  • Mean disease duration was 11 years for ChAc and 21 years for MLS.

Conclusions:

  • Causes of mortality in ChAc and MLS are comparable to Huntington's disease, with added risks from seizures and cardiac issues.
  • Suicidality was observed in 10% of ChAc patients.
  • Focusing on symptom management is crucial for improving morbidity in the absence of disease-modifying treatments.
Abstract

Keywords:
Chorea-acanthocytosisMcLeod syndromeMorbidity

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