Telehealth Helpful in Monitoring Spinal and Bulbar Muscular Atrophy

Scales of Kennedy's disease progression, captured online, show 'expected' changes

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

Share this article:

Share article via email
spinal and bulbar muscular atrophy | SMA News Today | telehealth study | illustration of patient and doctor using telehealth

Currently used clinical scales for men with spinal and bulbar muscular atrophy (SBMA) can be easily and reliably used remotely to monitor disease progression when patients are unable to visit a doctor’s office, according to a study in Italy.

The study, “Reliable virtual clinical assessment in spino-bulbar muscular atrophy (SBMA),” was published as a short report in the Journal of Neurology, Neurosurgery and Psychiatry.

SBMA, also called Kennedy’s disease after the physician who identified it, is a rare adult-onset form of spinal muscular atrophy that almost exclusively affects males.

Recommended Reading
SMA exoskeleton | SMA News Today | boy with SMA type 2 report | news announcement illustration

Boy With SMA Type 2 Able to Walk, Play Wearing Exoskeleton at Home

Symptoms often begin between the ages of 30 and 50, and include cramps and muscle spasms in the arms and legs that make walking more difficult and a risk of falls more likely. Over time, bulbar muscles, those of the face and throat, also weaken and make it harder to speak and swallow.

Spinal and bulbar muscular atrophy changes captured with AMAT, SBMA-FRS

Because symptoms can worsen with time, it is important that doctors keep track of the disease’s course. Two scales are typically used: the Adult Myopathy Assessment Tool (AMAT) and the SBMA-Functional Rating Scale (SBMA-FRS).

AMAT is a standardized physical performance test comprising 13 items to assess functional limitations and muscle endurance in adults. It takes about 25–35 minutes to complete and requires only minimal equipment (for example, a stopwatch and a step or steps). Its total score can range from zero (worst) to 45 (normal).

The SBMA-FRS is a validated, SBMA-specific scale that consists of 14 items exploring five functional domains: bulbar, upper limb, trunk, lower limb, and breathing. Its total score can range from zero (worst) to 56 (normal).

During the COVID-19 pandemic, in-office visits were rare at best. To preserve patients’ safety and limit the risk of contagion, many hospitals and clinics launched online telehealth or telemedicine programs to enable remote visits with a doctor.

In Italy, a team of researchers tested the possibility of applying the AMAT and SBMA-FRS scales remotely to monitor SBMA patients.

The study included 12 men with SBMA who were participating in the Italian SBMA Registry, which includes annual clinical assessments such as AMAT and SBMA-FRS to study the disease’s natural progression. All these men were monitored remotely from April to November 2020.

Patients’ mean age at the time of the remote visit was 61 (range, 32–78 years), and their first symptoms began at a mean age of 37.5 (range, 20–68 years).

On telehealth exams, the group had a mean of 25.3 points (range, 8 to 43 points) on the AMAT scale and a mean of 43.2 points (range, 31 to 56 points) on the SBMA-FRS, the team reported.

Researchers then compared these telehealth scores with those of previous and subsequent in-person visits to assess changes over seven years and evaluate whether the telehealth scores were in line with the disease’s expected, or natural, progression.

All patients had scores for both scales at one and two years before the telehealth visit, some had records stretching back five years, and five patients were also evaluated in person one year after the telehealth visit.

Results showed that patients experienced a steady annual decline over seven years on both scales: of one-to-two points in AMAT and of one point on the SBMA-FRS. A similar decline was also observed with scores taken remotely, meaning they “were within the expected values according to the disease progression rate,” the researchers wrote.

“Although there was some variability in individual cases, with the greatest changes occurring usually for the most severely affected patients, tele-AMAT and tele-SBMA-FRS scores followed the trend of in-person visits for almost all patients,” they added.

Telehealth seen as ‘valid tool’ for Kennedy’s disease monitoring

The scales could be “easily administered remotely,” the team wrote, adding that they “did not encounter any particular problem in administering the scales.”

Particular care, however, was taken “in avoiding risk of falls by asking the presence of a caregiver to assist the patient in the most demanding tasks,” they added.

Patients were asked to rate their telehealth visit, and the men’s degree of satisfaction was a mean 6.6 points (range, 3–8) on a 10-point scale.

“When outpatient visits are impossible, telemedicine is a valid tool to monitor SBMA, also using specific clinical disease scales,” the researchers wrote.

“We believe this approach is valuable also for other neuromuscular disorders, as demonstrated by others, and it is important in clinical practice, beyond the pandemic, as an integration to in-person visits, particularly for patients living at long-distant sites from specialized centers, and for conducting clinical trials, too,” the team concluded.