Publications

Stiff-person syndrome is rare and disabling autoimmune condition that most frequently affects women, with no real predisposition by race. Diagnosis is often arduous, which is why patients concomitantly suffer from anxiety and depression. To date, drug therapy is based on the use of benzodiazepines, barbiturates, and baclofen. Refractory cases are treated with intravenous immunoglobulin, plasmapheresis, B lymphocyte depletion with rituximab, and even the implantation of intrathecal baclofen devices. Botulinum toxin injection is frequently used, even if it still has an unclear role in the literature.

Our case report aims to demonstrate the efficacy of a combined treatment of botulinum toxin and therapeutic exercise in a 65-year-old patient with biceps brachii muscle hypertonia and diffuse spasms of the axial musculature, using rating scales such as the Numeric Rating Scale (NRS) and Modified Ashworth Scale (MAS), joint range of motion (ROM) measurement, and muscle dynamic stiffness mensuration, which is performed by using the MyotonPRO®. All the assessments were conducted at the first evaluation (T0), soon after the combined treatment with botulin toxin and therapeutic exercise (T1), three months (T2), six months (T3), and eight months after the botulinum toxin injection (T4).

The patient demonstrated benefits for more than 6 months with no side effects. The combined therapy of botulinum toxin and therapeutic exercise had an excellent result in our patient.

Keywords: stiff-man syndrome, stiff-person syndrome, botulinum toxin type A, therapeutic exercise

SPS is a disabling condition that severely affects the suffering patients’ lives, forcing them to also abstain from normal activities of daily living. Although for some of them, per os therapy with benzodiazepines, barbiturates, and baclofen can be curative, there is a large portion of affected patients who do not respond and others who cannot intake these drugs at all, such as those with major respiratory problems. For our specific case, the combined treatment with botulinum toxin type A (Dysport®) supplemented with a targeted therapeutic exercise tailored to the patient, proposed by a qualified physiotherapist, resulted in a significant improvement in terms of both the subjective perception of pain (assessed using the NRS scale) and the functional limitation of the compromised segments (assessed using the measurement of joint ROMs, the MAS scale, and the BB dynamic stiffness by using the MyotonPRO®), which remained constant up to six months after the botulinum toxin type A (Dysport®) injection. A future goal will be to monitor the patient over time and consider the disabling outcomes related to this rare syndrome. In addition, it would be desirable to propose the same type of treatment in a larger number of patients so that its effects can be better observed.