Charcot-Marie-Tooth 1A Patients Have Significant Clinical Decline After Age 50, Study Shows

Researchers have found that disease progression in Charcot-Marie-Tooth type 1A (CMT1A) patients reaches a critical point at age 50, after which clinical decline occurs faster. In response, patients should be encouraged to engage in a healthy and active lifestyle in order to preserve their function, a new study recommends.
The article “Motor performance deterioration accelerates after 50 years of age in Charcot-Marie-Tooth type 1a patients” was published in the European Journal of Neurology.
The aim of the study was to describe the clinical decline in CMT1A patients, by comparing their clinical impairment and age. CMT1A is the most common CMT subtype.
Researchers at the University of Naples “Federico II” in Naples, Italy, recruited and evaluated 70 CMT1A patients (26 men and 44 women from 20 to 81 years of age) and 70 sex- and age-matched healthy participants as controls.
All healthy participants had no neurological symptoms or signs or other disabilities. In the CMT1A patient group, 38 patients were from 14 different families.
Clinical impairment was measured through motor performance using three different tests: the 10-Meter Walk Test, the 6-Minute Walk Test, and the 9-Hole Peg Test of dominant and non-dominant side. All three tests determine the ability and capacity of the participant to achieve specific physical tasks.
In addition, muscle strength, disability and quality of life were evaluated in CMT1A patients.
The researchers analyzed the relationships between age and all clinical measures. In general, motor performance deteriorated with age in both CMT1A patients and healthy participants. However, motor performance deterioration was more accentuated in CMT1A patients.
The authors found that CMT1A patients and healthy participants had similar clinical measures until 50 years of age. However, from age 50 onwards, CMT1A patients had significantly greater clinical impairment when compared with healthy participants.
“Our results support this assumption demonstrating that after the 50th year of age the extent of deterioration of clinical impairment accelerates in CMT1A patients,” the authors wrote. “This is relevant for clinicians in the current management of CMT1A patients. A healthy lifestyle and physical activity should be encouraged in order to preserve the functional reserve.”