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Life expectancy and mortality in chorea-acanthocytosis and McLeod syndrome


Walker, Ruth H; Miranda, Marcelo; Jung, Hans H; Danek, Adrian (2019). Life expectancy and mortality in chorea-acanthocytosis and McLeod syndrome. Parkinsonism & Related Disorders, 60:158-161.

Abstract

OBJECTIVE To document life expectancy and causes of death in chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS). METHODS We reviewed our personal databases and the published literature to identify cases of ChAc and MLS for whom adequate information was available regarding ages of disease onset and death, cause of death, and other clinical information. RESULTS Adequate information was obtained on 52 patients with ChAc and 34 with MLS. Causes of death included pneumonia, cardiac disease, seizure, suicide, and sepsis. Mean disease duration from diagnosis was 11 years for ChAc, while for MLS it was 21 years. CONCLUSIONS Given the current data, causes of death in ChAc and MLS are similar to those for the phenotypically similar Huntington's disease, with additional risks due to the presence of seizures and cardiac disease. Suicidality was seen in 10% of patients with ChAc. Identifying causes of mortality is valuable for disease management and ultimately for clinical trials. In the absence of disease-modifying agents, disease management should focus upon treating symptoms which may contribute to morbidity.

Abstract

OBJECTIVE To document life expectancy and causes of death in chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS). METHODS We reviewed our personal databases and the published literature to identify cases of ChAc and MLS for whom adequate information was available regarding ages of disease onset and death, cause of death, and other clinical information. RESULTS Adequate information was obtained on 52 patients with ChAc and 34 with MLS. Causes of death included pneumonia, cardiac disease, seizure, suicide, and sepsis. Mean disease duration from diagnosis was 11 years for ChAc, while for MLS it was 21 years. CONCLUSIONS Given the current data, causes of death in ChAc and MLS are similar to those for the phenotypically similar Huntington's disease, with additional risks due to the presence of seizures and cardiac disease. Suicidality was seen in 10% of patients with ChAc. Identifying causes of mortality is valuable for disease management and ultimately for clinical trials. In the absence of disease-modifying agents, disease management should focus upon treating symptoms which may contribute to morbidity.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Neurology
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Life Sciences > Neurology
Health Sciences > Geriatrics and Gerontology
Health Sciences > Neurology (clinical)
Language:English
Date:1 March 2019
Deposited On:23 Nov 2018 06:55
Last Modified:29 Jul 2020 08:03
Publisher:Elsevier
ISSN:1353-8020
OA Status:Closed
Free access at:PubMed ID. An embargo period may apply.
Publisher DOI:https://doi.org/10.1016/j.parkreldis.2018.09.003
PubMed ID:30245172

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