Eur J Paediatr Neurol. Sep;16(5) doi: / Epub Jan Beyond the Burke-Fahn-Marsden Dystonia Rating Scale. Burke RE, Fahn S, Marsden CD, Bressman SB, Moskowitz C, Friedman J. Validity and reliability of a rating scale for the primary torsion dystonias. Download Table | Burke-Fahn-Marsden Dystonia Rating Scale from publication: Early Globus Pallidus Internus Stimulation in Pediatric Patients With.
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Many rating scales have been applied to the evaluation of dystonia, but only few have been assessed for clinimetric properties. The Movement Disorders Society commissioned this task force to critique existing dystonia rating scales and place them in the clinical and clinimetric context.
A systematic literature review was conducted to identify rating scales that have either been validated or used in dystonia. Thirty six potential scales were identified.
Eight were excluded because they did not meet review criteria, leaving twenty-eight scales that were critiqued and rated by the task force.
All the scales are individually reviewed in the online appendix. The task force recommends five specific dystonia scales and suggests to further validate in dystonia two recommended generic voice-disorder scales. Existing scales for oromandibular, arm and task-specific dystonia should be refined and fully assessed. Scales should be developed for body regions where no scales are available, such as lower limbs and trunk. Dystonia is one of the most common movement disorders, with an overall prevalence of This met analysis prevalence figures is likely to be an underestimate, as it is based on studies with recruitment of diagnosed cases only and it is clear that under-ascertainment and under-diagnosis is a significant problem 2.
The broad spectrum of clinical features that encompass dystonia syndromes ranges from severe generalised childhood dystonia, to adult-onset focal dystonias, to secondary dystonias and dystonias as a feature of complex neurological disorders. Dystonia can be localized to a single body region focal or has spread to contiguous segmental or to non-contiguous multifocal regions.
In generalised dystonia the trunk and at least two other sites are involved 3whereas hemidystonia affects the body and limbs on one side. Dystonia is typically considered a movement disorder characterized by motor manifestations, primarily sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both 3.
FMDRS – Fahn-Marsden Dystonia Rating Scale
However, growing evidence indicates the importance of non-motor component to dystonia, including abnormalities in sensory and perceptual functions, as well as neuropsychiatric, cognitive and sleep domains 4. Treatment possibilities have greatly expanded in recent years after discovering the efficacy of botulinum neurotoxins and functional surgery 56.
Pre-treatment evaluation aims at characterizing the severity and topography of motor symptoms and their impact on activities of daily living and provides a baseline reference for post-treatment evaluations. The quality and accuracy of the pre-treatment assessment and the choice of assessment tools are crucial as they will affect all subsequent post-treatment comparisons.
To facilitate research and clinical practices aimed at improving the dystonis and treatment of dystonia syndromes, the Movement Disorders Society convened a task force to evaluate the dystonia rating instruments that have been used in published studies.
This review is part of a process to assess scales currently in use for evaluating clinical aspects of movement disorders. This task force consisted of eight members from Europe and North America with diverse background and expertise, including neurologists, a neuropediatrician, and a clinical dsytonia, who had worked extensively in the area of dystonia.
This group followed the same procedure as the task forces that appraised other rating scales in movement disorders. Initial discussions among these task force members were on the construct to be reviewed, in the case its concept was not universally accepted. Then the task force members selected the scales to be included in the review see criteria below and identified unresolved issues and limitations of the critiqued scales. A standardized form was drawn up to allow structured assessment of the scales with regard to their descriptive properties, availability, content, use, acceptability, clinimetric properties, and overall impression inpatients with dystonia see online supporting material.
Each scale was reviewed by one task force member. The completed reviews were then assessed by two members and modified according to their suggestions. This terminology was also hurke in recent reviews on rating scales of other MDS task forces 8. The final assessment was based on consensus among the task force members and the Steering Committee of the Task Force on Rating Scales for Movement Disorders. The following criteria were specifically distilled from the available evidence.
The scale has been applied to dystonia patients; criterion 2: The official definitions for task force critiques are: We considered all scales and questionnaires that have either been designed or used to rate dystonia, and in addition scales and questionnaires that, based on literature review and expert evaluation, have potential utility in dystonia based on their content, their widespread use, and clinimetric evidence from studies in patients without dystonia.
We did not consider scales and questionnaires specifically designed for secondary dystonias or requiring measurement devices. All scales are listed in the online supporting material. Medline on PubMed was systematically searched for relevant papers published up to June using the following query: In addition, published articles known to the Task Force members were included in this review.
Only published or in press peer reviewed papers or published abstracts were evaluated. Thirty six scales and questionnaires were identified. Among the generic scales, one was developed to measure coping in chronically ill populations and six were originally developed to quantify the degree of dysphonia and to objectively determine the efficacy of voice therapy in voice disorders.
Eight scales were excluded, because evaluating secondary dystonia, requiring measurement devices, or having no potential use in dystonia. The scales are grouped based on the affected body region they intend to explore. It is a disease-specific patient-rated disability scale that measures impairment of specific activities of daily living caused by blepharospasm. The range of scores is zero to 24, with higher scores indicating a greater disability. It is available only in English, although the scale has been used extensively in Europe and Israel.
The BSDI was designed specifically for patients with blepharospasm. The BSDI showed high internal consistency and the retest reliability of the single items was adequate 9. Data from two large randomized trials designed to evaluate the effects of BoNT type A products for blepharospasm showed that the BSDI was sensitive to change but did not detect differences between two BoNT products 10 The BSDI has been specifically designed to measure disability in blepharospasm due to dystonic movements that affect vision.
The scale focuses on daily activities and is ease of use; the scoring system is also rather simple. The BSDI focuses on disability related to sight and does not specifically measure dystonic motor abnormalities; it should be combined with a more specific scale that rates the movement disorder.
Concern has been raised regarding poor sensitivity of the scale to mild disability or small changes The CDIP is a disease-specific patient-rated questionnaire that measures quality of life in patients with cervical dystonia It was developed for use in clinical research, audit, and treatment trials. It is composed of fifty-eight 5-point items grouped into eight subscales that measure symptoms head and neck movements, pain and discomfort in neck and shoulders, sleep disturbance as a result of torticollisactivity limitations in upper limb activities and walking, and psychosocial features annoyance, mood, psychosocial functioning.
Eight summary scale scores are generated by summing items and then transformed to a 0— score. This scale is available only in English. The CDIP has been specifically developed for patients with cervical dystonia.
Fahn-Marsden Dystonia Rating Scale (FMDRS)
The CDIP has been used to measure the health impact of cervical dystonia and the impact of treatment in cervical dystonia 15 — New psychometric techniques Rasch analyses revealed that the CDIP performs well and, in addition, traditional psychometric properties such as reliability internal consistency, item-total correlation, test-retest and validity within-scale analyses and comparisons with external measures have been supported 1415 The CDIP afhn a disease-specific validated questionnaire.
It is more sensitive in detecting statistical and clinical changes than other subscales, although it has not been widely used as an outcome measure. The authors themselves 15 dysyonia further studies examining the responsiveness of the CDIP as well as refinement of the walking subscale.
It is composed of 3 subscales that measure symptom severity, disability, and pain. The severity scale, clinician-rated, is composed of 11 items that assess head movements, duration of symptoms, effects of sensory tricks, shoulder elevation and anterior displacement, range of motion, and time in neutral position; the maximal score is The disability scale, patient-rated, comprises 6 items, including daily activities, work, reading, and driving; the maximal score is The pain scale, patient-rated, comprises 3 items including severity, duration and disability due to pain; the maximal score is A training tape for clinicians is available for the severity scale The only available version is in English.
Dystonia rating scales: critique and recommendations
Individual subscales and the total TWSTRS score have been used as outcome measures in many treatment trials, evaluating BoNT therapy, pharmacotherapy and surgery 22 — Evidence for validity is shown by moderate within-scale correlations Responsiveness to change has been demonstrated 25 Weaknesses consist in an unclear definition of midline for assessing range of motion, lack of a separate scoring category assessing dystonic tremor, and the specification of duration for the effect of sensory tricks The CDQ is a patient-rated health-related quality of life HRQoL measure for craniocervical dystonia, featuring cervical dystonia and blepharospasm.
It was developed for use in clinical research It is composed of 24 items, forming five subscales: Items are rated on a 5-point scale. Although only the original German version was validated, an exact translation into English, including back-translation, was performed The CDQ has also been translated and validated in Serbian The CDQ has been specifically developed for patients with craniocervical dystonia, who had both cervical dystonia and blepharospasm.
The CDQ has been used by multiple groups to measure the impact on quality of life of focal, segmental and even generalized dystonia 16 and also as a HRQoL measure to assess responsiveness to treatment-induced changes 60 The CDQ also showed good reliability properties, internal consistency and test-retest reliability.
Validity was assessed by checking convergent and discriminant validity as well as the dimensional structure of CDQ; sensitivity to change was confirmed after BoNT treatment The CDQ is a brief and easy instrument. It can be used to evaluate the impact of the disease on areas not covered by generic measures, such as SF, which are of considerable concern to patients with craniocervical dystonia. The CDQ evaluates also pain, sleep and depression due to dystonia.
The VHI is a patient-rated scale addressing disability related to verbal communication. It was developed to determine the level of disability experienced by patients with different voice disorders The complete VHI has 30 items organized in 3 domains: The rating is on a 5-point scale and the total score ranges from 0 to The validity of the French translation has been confirmed, although the quality of translation needs further improvements VHI has been used to measure outcomes after interventions for a broad range of laryngeal disorders, including cancer and mass lesions, vocal fold polyps and cysts, and laryngeal dystonia 65 — In the development and validation study performed on a heterogeneous set of disorders 63the VHI proved to have good internal consistency and good test-retest reliability for subscales and total scores.
Construct validity was not fully evaluated. The VHI has been used in several studies to assess efficacy of treatments for laryngeal dystonia. However, considering that the VHI was validated on few patients with laryngeal dystonia compared to the total number of patients assessed, it still needs further validation for dystonia. The VHI is a simple and efficient scale, but as a disability scale, it has no discriminant value and is not specific for dystonia.
Therefore, the scale should be further validated specifically in spasmodic dysphonia.