Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Feb 25:11:139.
doi: 10.3389/fneur.2020.00139. eCollection 2020.

The Maximum Bite Force for Treatment Evaluation in Severely Affected Adult SMA Patients-Protocol for a Longitudinal Study

Teresa Kruse  1   2 , Helmar C Lehmann  3 , Bert Braumann  1   2 , Gereon R Fink  3 , Gilbert Wunderlich  2   3
Affiliations

The Maximum Bite Force for Treatment Evaluation in Severely Affected Adult SMA Patients-Protocol for a Longitudinal Study

Teresa Kruse et al. Front Neurol. .

Abstract

Spinal muscular atrophy (SMA) is a severe neuromuscular disorder characterized by the degeneration of motor neurons in the spinal cord, and comprises a broad clinical spectrum. With the advent of new therapies (e.g., Nusinersen) for patients of all ages and disease stages, sensitive clinical measures are needed to detect slight changes in muscle force even in immobilized, severely affected patients often unable to move limbs. As for these patients, well-established outcome scales set out to evaluate motor function do not work properly, we propose measurement of maximum bite force which is able to detect subtle changes of bulbar function. Requirements for this approach are mentioned, challenges are discussed, and first insights from a pilot study are presented. Finally, a study design is proposed to evaluate the measurement of maximum bite force during the follow up of SMA patients with and without a disease modifying therapy.

Keywords: antisense oligonucleotides; bulbar neuromuscular function; masticatory force; motor neurons; piezoelectric transducer; spinal muscular atrophy.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Customized device for bite force measurement in SMA patients. (A) Use of a thin piezoelectric force sensor on a severely affected adult patient with SMA type 2. (B) Sensor placed unilaterally in a region of good intercuspidation. Teeth are partially covered by occlusal bite blocks protecting the sensor from damage, assuring good contact, and precise repositioning. (C) The thin Flexiforce transducer A201-100 (Tekscan Inc., USA) guarantees minimal jaw separation. Written informed consent was obtained from the individual for publication of this image.
Figure 2
Figure 2
Individually adjusted piezoelectric T-Scan sensor foil (Tekscan Inc., USA) with individually adjusted surface (dental silicone, Honigum, DMG, Hamburg) to compensate for dental or skelettal anomalies and to ensure a reproducible position in each measurement.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Mercuri E, Bertini E, Iannaccone ST. Childhood spinal muscular atrophy: controversies and challenges. Lancet Neurol. (2012) 11:443–52. 10.1016/S1474-4422(12)70061-3 - DOI - PubMed
    1. Finkel RS, Mercuri E, Darras BT, Connolly AM, Kuntz NL, Kirschner J, et al. . Nusinersen versus sham control in infantile-onset spinal muscular atrophy. N Engl J Med. (2017) 377:1723–32. 10.1056/NEJMoa1702752 - DOI - PubMed
    1. Mercuri E, Darras BT, Chiriboga CA, Day JW, Campbell C, Connolly AM, et al. . Nusinersen versus sham control in later-onset spinal muscular atrophy. N Engl J Med. (2018) 378:625–35. 10.1056/NEJMoa1710504 - DOI - PubMed
    1. Day J. Nusinersen experience in teenagers and young adults with spinal muscular atrophy. In: Muscular Dystrophy Association Clinical Conference. Arlington, VA: (2018).
    1. DMD- und SMA-Patientenregister ,. (2019). Available online at: https://www.treat-nmd.de/register/ (accessed June 06, 2019).