Dystonia

Berman, Brian D.; Jinnah, Hyder A.

doi:10.1212/cpj.0000000000000128

Cited by 15 publications

(6 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[7][8][9] Throughout childhood (until about 17 years of age), these networks mature by physiologic neurodevelopmental processes, such as selective elimination of neuronal connections and myelination. 13,[37][38][39][40] As implicated by the interconnecting brain networks between the basal ganglia and cerebellum, [10][11][12] we also recognized a striking similarity between the presently reported BFMMS age-relatedness and the previously reported age-relatedness of the Scale for the Assessment and Rating of Ataxia (SARA). 21 Comparing age-relatedness between BFMDRS and SARA scales showed that the BFMMS agerelatedness lingered for a longer time course than that of BFMDS and SARA (≥16 years versus 12 and 10 years 21 of age, respectively).…”

Section: Discussionsupporting

confidence: 83%

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

Kuiper

Vrijenhoek

Brandsma

et al. 2016

Movement Disord Clin Pract

View full text Add to dashboard Cite

View Supplementary Video 1 Background The Burke‐Fahn‐Marsden Dystonia Rating Scale is a universally applied instrument for the quantitative assessment of dystonia in both children and adults. However, immature movements by healthy young children may also show “dystonic characteristics” as a consequence of physiologically incomplete brain maturation. This could implicate that Burke‐Fahn‐Marsden scale scores are confounded by pediatric age. Objective In healthy young children, we aimed to determine whether physiologically immature movements and postures can induce an age‐related effect on Burke‐Fahn‐Marsden movement and disability scale scores. Methods Nine assessors specializied in movement disorders (3 adult neurologists, 3 pediatric neurologists, and 3 MD/PhD students) independently scored the Burke‐Fahn‐Marsden movement scale in 52 healthy children (4–16 years of age; 2 boys and 2 girls per year of age). Independent of that, parents scored their children's functional motor development according to the Burke‐Fahn‐Marsden disability scale in another 52 healthy children (4–16 years of age; 2 boys and 2 girls per year of age). By regression analysis, we determined the association between Burke‐Fahn‐Marsden movement and disability scales outcomes and pediatric age. Results In healthy children, assessment of physiologically immature motor performances by the Burke‐Fahn‐Marsden movement and disability scales showed an association between the outcomes of both scales and age (until 16 years and 12 years of age, β = −0.72 and β = −0.60, for Burke‐Fahn‐Marsden movement and disability scale, respectively [both P < 0.001]). Conclusions The Burke‐Fahn‐Marsden movement and disability scales are influenced by the age of the child. For accurate interpretation of longitudinal Burke‐Fahn‐Marsden Dystonia Rating Scale scores in young dystonic children, consideration of pediatric age‐relatedness appears advisory.

show abstract

Section: Discussionsupporting

confidence: 83%

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

Kuiper

Vrijenhoek

Brandsma

et al. 2016

Movement Disord Clin Pract

View full text Add to dashboard Cite

show abstract

“…Our results are supports the idea of two separate pathophysiology contributing to head oscillations in CD, one originating from the cerebellum and basal ganglia and the other from proprioception. This concept is consistent with the notion that dystonia is a network disorder [28][29][30]. The results also support the idea that although dystonia and tremor often coexist, they may still represent distinct entities with different underlying pathophysiological mechanisms [31].…”

Section: Dystoniasupporting

confidence: 91%

“…According to the network model in CD, the impairment anywhere in the network, even outside of the integrator, may lead to deficits in the feedback dependent neural integration. There is increasing evidence for the involvement of cerebellum, proprioception, and basal ganglia, as three separate sources of the feedback in the network model for CD [28][29][30][31][32][37][38][39]. The cerebellar role in dystonia is supported when the oscillations coexisting with dystonia have sinusoidal features as seen in tremor that is also thought to be related to cerebellar deficits.…”

Section: Dystoniamentioning

confidence: 99%

Effects of botulinum neurotoxin on regularity of head oscillations in cervical dystonia

Agharazi,

Jinnah,

Zee

et al. 2024

Dystonia

View full text Add to dashboard Cite

Introduction: This study explores the effects of botulinum neurotoxin (BoNT) on the relationship between dystonia and tremor, specifically focusing on cervical dystonia (CD) and its connection to head tremor.Methods: Fourteen CD patients were recruited; eight (57%) with clinically observable head oscillations were included in further analysis. A high-resolution magnetic search coil system precisely measured head movements, addressing two questions: 1) BoNT’s effects on head movement amplitude, frequency, and regularity, and 2) BoNT’s influence on the relationship between head position and head oscillations. For the first question, temporal head position measurements of three patients were analyzed before and after BoNT injection. The second question examined the effects of BoNT injections on the dependence of the oscillations on the position of the head.Results: Three distinct trends were observed: shifts from regular to irregular oscillations, transitions from irregular to regular oscillations, and an absence of change. Poincaré analysis revealed that BoNT induced changes in regularity, aligning oscillations closer to a consistent “set point” of regularity. BoNT injections reduced head oscillation amplitude, particularly in head orientations linked to high-intensity pre-injection oscillations. Oscillation frequency decreased in most cases, and overall variance in the amplitude of head position decreased post-injection.Discussion: These findings illuminate the complexity of CD but also suggest therapeutic potential for BoNT. They show that co-existing mechanisms contribute to regular and irregular head oscillations in CD, which involve proprioception and central structures like the cerebellum and basal ganglia. These insights advocate for personalized treatment to optimize outcomes that is based on individual head oscillation characteristics.

show abstract

“…условлен появлением публикаций [4] о наличии высокого уровня тревожно-депрессивных расстройств при данной патологии. G. Heiman и R. Ottman [5] опубликовали данные, доказывающие, что депрессия чаще встречается у носителей мутации дистонии DYT1 при отсутствии клинических проявлений гиперкинеза.…”

unclassified

Emotional and personality characteristics of patients with dystonia

Чернуха

Tarasevich

2017

Z. nevrol. psikhiatr. im. S.S. Korsakova

View full text Add to dashboard Cite

Higher levels of anxiety and depression in patients with dystonia compared to the control groups were identified. The level of anxiety and depression was not correlated with disease severity. Personality profile study confirmed the high level of anxiety in patients with dystonia and additionally showed increased emotional instability and poor emotional control. Intellectual personality characteristics of patients with dystonia had no differences compared to the control groups. An analysis of socio-psychological characteristics of personality in patients with dystonia and GFS showed restricted interpersonal and social communications since the motor defect noticeable to others leads to severe social exclusion and the desire for self-isolation.

show abstract

Dystonia

Cited by 15 publications

References 22 publications

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

Effects of botulinum neurotoxin on regularity of head oscillations in cervical dystonia

Emotional and personality characteristics of patients with dystonia

Contact Info

Product

Resources

About