CAG repeat number correlates with the rate of brainstem and cerebellar atrophy in Machado-Joseph disease

1 September 1998

journal article
Published by Wolters Kluwer Health in Neurology

Vol. 51 (3) , 882-884
https://doi.org/10.1212/wnl.51.3.882

Abstract

We compared the CAG repeat length and the severity of the brainstem and cerebellar atrophy visualized by MRI in 30 patients with Machado-Joseph disease. We found a strong correlation between the CAG repeat number and the quotient of the degree of atrophy divided by age at examination. These results suggest that the rate of disease progression is dependent on the CAG repeat size and disease progression may commence at birth.

Keywords

REPEAT NUMBER
BRAINSTEM
CEREBELLAR
ATROPHY
CAG REPEAT
BIRTH
MRI
COMMENCE

This publication has 3 references indexed in Scilit:

The age of women
The Lancet, 1996
Message from the editor
Annals of Neurology, 1996
A salute to the departing aaem executive director and a longtime supporter of muscle & nerve
Muscle & Nerve, 1995