Vibration Device Fails to Halt Motor Deterioration in Children with Friedreich’s Ataxia, Small Study Shows

Muscle stimulation through focal vibrations produced by a specialized device may prevent further deterioration of motor function in children with cerebellar ataxia, but not in Friedreich’s ataxia patients, according to a small study.

The study, “Non-invasive Focal Mechanical Vibrations Delivered by Wearable Devices: An Open-Label Pilot Study in Childhood Ataxia,” was published in the journal Frontiers in Neurology.

Friedreich’s ataxia belongs to a group of disorders known as childhood ataxia, characterized by the development of movement dyscoordination (ataxia) during childhood or early adolescence. According to the study, it is estimated that 26 in 100,000 children have some form of childhood ataxia.

Patients with Friedreich’s ataxia have progressive nerve and muscle damage, causing movement dyscoordination and muscle weakness, which affects not only the limbs, but also the eyes, hearing, and speech.

There is increasing evidence that muscle stimulation with vibrations induces changes in motor nerve circuits, which can lead to improved motor control and function if administrated repeatedly.

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Non-invasive focal mechanical vibrations (NIFMV), usually delivered through electromechanical-based devices, are designed to improve motor control and balance in several neurological diseases.

Recent advances in nanotechnology have led to the development of a device that transforms minimal temperature variations into mechanical energy (vibrations).

Equistasi is a registered medical nanodevice for NIFMV, consisting of a transparent patch with a rectangular plate composed of nanotechnology fibers in the center.

Previous studies have shown that the use of this device improved the walking ability of patients with Parkinson’s disease and of adults with hereditary cerebellar ataxias, another form of ataxia. However, its therapeutic benefits in people with childhood ataxia are still unknown.

Researchers in Italy evaluated the feasibility, safety and effectiveness of NIFMV given through this wearable patch in six Friedreich’s ataxia patients and four patients with slow or non-progressive cerebellar ataxias.

Participants were recruited at the Bambino Gesù Children’s Hospital, in Rome in 2017 and 2018. The Friedreich’s ataxia patients’ mean age was 15.66 years; the cerebellar ataxia patients, 10 years. None of the participants had severe motor or intellectual disability.

Patients were prescribed three Equistasi patches — one to wear over the seventh cervical vertebra and one on each calf muscle — for one hour a day for five days in the first week, then for two hours a day (one in the morning, one in the afternoon), five days a week for the following three weeks.

All participants followed the same protocol of physiotherapy (50-minute individual session for walking and balance training three times a week) and continued their medical therapy.

Researchers assessed patients’ muscle coordination through the Scale for the Assessment and Rating of Ataxia (SARA), Nine-Hole-Peg Test (9-HPT), 6 Minute Walk Test (6MWT), and the analysis of walking parameters through a system with eight cameras. Assessments were made at the beginning of the study, after four weeks of treatment, and four weeks after the end of treatment.

Six patients (60%) did not complete the study, dropping out after the four weeks of treatment due to the absence of subjective improvements and discomfort associated with the device (difficulties in being autonomous and the presence of pain and itchiness while using it). However, no adverse events were reported during the study.

Since clinical data of four weeks after the end of treatment were only available for four patients, the researchers decided to proceed to the statistical analysis using only the data collected before treatment and after four weeks of treatment.

The team found no significant changes in motor coordination after treatment, except for the Friedreich’s ataxia patients’ walking parameters (stance time and stride time), which got worse.

These preliminary findings suggest that NIFMV through wearable nanodevices may prevent further deterioration of motor coordination in people with cerebellar ataxia, but not in those with Friedreich’s ataxia.

The researchers hypothesized that these results may be explained by the impaired sensory system of Friedreich’s ataxia patients (essential for NIFMV to work) and to the faster progression of the disease.

“Larger studies are necessary to confirm these preliminary observations, to define standardized schemes of treatment and the correct criteria of eligibility, especially in complex conditions such as [childhood ataxia],” the researchers said.