The aim of this research project is to study a large a group of patients with tonic dystonia longitudinally, to accurately describe the phenotype, to investigate the potential underlying mechanisms and to identify possible causes and risk factors.
ID
Source
Brief title
Condition
- Movement disorders (incl parkinsonism)
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Medical history is obtained following a semi-structured interview. The primary
patient group will be evaluated with the CRPS diagnose form. 2 EDTA tubes of
blood (20 ml) will be collected for genetic testing only during the first
visit.
Several self-administered questionnaires will be completed: 1). Hospital
anxiety and depression scale (HADS); 2). Tampa scale for Kinesiophobia; 3).
Pain coping inventory (PCI); 4). Numeric rating Scale Pain (NRS-Pain); 5).
McGill Pain Questionnaire (MPQ); 6). Dissociative experience scale (DES); 7);
Somatoform dissociation questionnaire (SDQ20); 8). SCOPA AUT; 9). Radboud
Skills Questionnaire; 10). Questionnaire on walking & rising; 11). Questions
about profession and education (first visit)
The severity and progression of the dystonia, together with the impairments on
daily activities, will be measured with the *DYstonia Assessment Scale (DYAS)*
rating scale and the *Burke Fahn Marsden* (BFM) scale. A rapid finger movement
task is performed to test
the velocity and fluency of movements (bradykinesia).
Quantative sensory testing is used to assess wind-up, pain thresholds and
perception to touch, temperature and vibration. Diffuse Noxious Inhibitory
Control (DNIC), a test that interrogates the function of endogenous pain
regulation, will also be determined. Surface temperature of hands and feet are
determined with an infrared skin temperature device. The ability to accurately
recognise the laterality of pictures of left and right extremities is performed
with the Recognise© lateralization computer program.
Secondary outcome
not applicable
Background summary
Dystonia is the most common type of movement disorder that may develop after
peripheral trauma, but can also arise in combination with Complex Regional Pain
Syndrome (CRPS) or chronic pain. The phenotype of this dystonia differs from
the primary *mobile* form and is usually *tonic*, referring to the presence of
continuous muscle contractions leading to abnormal postures, from which return
to the neutral position is not possible or only with great difficulty. The
pathophysiology of tonic dystonia is unknown and there is still controversy
about the contribution of psychological factors in the development of the
disorder. Evidence from primary dystonia suggests that the condition may arise
in response to trauma or pain in subjects with an increased susceptibility,
whereby a (pre-existent) maladaptive plasticity and disturbances in sensimotor
integration may lead to dystonia.
Study objective
The aim of this research project is to study a large a group of patients with
tonic dystonia longitudinally, to accurately describe the phenotype, to
investigate the potential underlying mechanisms and to identify possible causes
and risk factors.
Study design
The proposed study is a combination of a prospective cohort study and a case
control comparison. The primary patient group will be examined once a year at
the LUMC, for a period of 4 years, while the healthy control group will only be
measured once and the patient control group at year 1 and 3.
Study burden and risks
The tests are non-invasive. Although the chances are small, it can not be
excluded that patients with tonic dystonia develop more severe symptoms,
patients with CRPS an exacerbation of the complaints and more pain.it can not
be excluded In these situations the study will be adapted to the persons
wishes, or will be ended. If necessary, the neurologist on duty will be
consulted.
Tonic dystonia is an invalidating disorder with an unknown cause, however
little research has been performed to learn more about the disoder. Tonic
dystonia is not always recognized by doctors, and is sometimes referred as a
psychogenic disorder. This study asks some effort from the patients and is not
directly helping the individual, but will provide more insight in the disorder
and could be beneficial for treatment and understanding patients in the future.
Albinusdreef 2
2333 ZA Leiden
NL
Albinusdreef 2
2333 ZA Leiden
NL
Listed location countries
Age
Inclusion criteria
patients with tonic dystonia
Exclusion criteria
- mobile dystonia
- patients with a known genetic form of dystonia, e.g. DYT1-DYT17, Wilson*s disease
- lesions or diseases of the central nervous system (e.g. as a result of head trauma)
- implantation of drug-delivery pump
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL21732.058.09 |