Botulinum toxin type A treatment of four cases of Wilson disease with lower limb dystonia: A prospective study

Cao, Zeyu; Rao, Rao; Wu, Tong Tong; Chen, Shangzhi; Xing, Saiwei; Han, Yantao

doi:10.1016/j.toxicon.2022.106959

Cited by 3 publications

(3 citation statements)

References 20 publications

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“…A case series of five patients with jaw-opening OMD in WD from Brazil showed mild improvements in Burke-Fahn-Marsden dystonia scores following treatment with botulinum toxin type A [29]. An improvement in lower extremity secondary dystonia symptoms was also reported in a small series of four WD patients [30]. Our data further support the benefits of botulinum toxin treatment of WD patients with blepharospasm, OMD, cervical dystonia, and limb dystonia in all our treated WD patients who had relatively severe dystonia associated with paradoxical worsening after chelation therapy [31].…”

Section: Discussionmentioning

confidence: 81%

Role of Botulinum Toxin in Treatment of Secondary Dystonia: A Case Series and Overview of Literature

Mohanty,

Riordan,

Hedera

2024

Toxins

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Introduction: Dystonia can present in primary and secondary forms, depending on co-occurring symptoms and syndromic associations. In contrast to primary dystonia, secondary forms of dystonia are often associated with lesions in the putamen or globus pallidus. Such disorders are commonly neurodegenerative or neurometabolic conditions which produce varied neurologic as well as systemic manifestations other than dystonia. Chemo-denervation with botulinum toxin has been successfully used for focal or segmental dystonia. However, studies evaluating the effect of BoNT therapy on patients with secondary dystonia are sparse, given the heterogeneity in etiology and presentation. Methods: We present a series of patients with secondary dystonia who were managed with botulinum toxin therapy. Patients included in this series had a confirmed neurometabolic cause of dystonia. Results: A total of 14 patients, with ages ranging from 17 to 36 years, with disorders including Wilson’s disease, pantothenate kinase-associated neurodegeneration (PKAN), Niemann–Pick disease type C (NPC), glutaric aciduria type 1, Sanfilippo syndrome (Mucopolysaccharidosis Type IIIb), and GM2 gangliosidosis (Sandhoff disease) are presented. Most patients experienced a mild to moderate improvement in treated dystonia with benefits ranging from 6 to 12 weeks, with the median length of the benefits lasting approximately eight weeks, without any significant adverse effects. Conclusion: Although the secondary causes of dystonia are complex and diverse, our presented data and the available reports of the use of botulinum toxin support the conclusion that chemo-denervation plays an important role in symptom alleviation.

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Section: Discussionmentioning

confidence: 81%

Role of Botulinum Toxin in Treatment of Secondary Dystonia: A Case Series and Overview of Literature

Mohanty,

Riordan,

Hedera

2024

Toxins

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“…It can be focal (involves one body part, for example, one hand), segmental (involves one body segment, for example, upper extremity), multisegmental (involves multiple segments, for example, face and leg) or may even be generalized [35]. Multisegmental dystonia, manifested as limb dystonia, is also common in clinical practice and may affect other extrapyramidal symptoms [15,20,21]. In previous studies, extensive brain functional and structural impairments, particularly in basal ganglia nucleus, were found in other dystonia-related diseases [36,37].…”

Section: Discussionmentioning

confidence: 99%

“…As a special neurogenetic disease, dystonia of the extremities is also common in WD patients and mainly caused by muscle tension impairment, as reported by previous studies, and its impact on the quality of life and prognosis of patients was particularly prominent [20,21]. So, it is very meaningful to study WD with extremities dystonia.…”

Section: Clinical and Muscle Biomechanical Assessmentmentioning

confidence: 91%

Dysfunction of the Lenticular Nucleus Is Associated with Dystonia in Wilson’s Disease

et al. 2022

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Dysfunction of the lenticular nucleus is thought to contribute to neurological symptoms in Wilson’s disease (WD). However, very little is known about whether and how the lenticular nucleus influences dystonia by interacting with the cerebral cortex and cerebellum. To solve this problem, we recruited 37 WD patients (20 men; age, 23.95 ± 6.95 years; age range, 12–37 years) and 37 age- and sex-matched healthy controls (HCs) (25 men; age, 25.19 ± 1.88 years; age range, 20–30 years), and each subject underwent resting-state functional magnetic resonance imaging (RS-fMRI) scans. The muscle biomechanical parameters and Unified Wilson Disease Rating Scale (UWDRS) were used to evaluate the level of dystonia and clinical representations, respectively. The lenticular nucleus, including the putamen and globus pallidus, was divided into 12 subregions according to dorsal, ventral, anterior and posterior localization and seed-based functional connectivity (FC) was calculated for each subregion. The relationships between FC changes in the lenticular nucleus with muscle tension levels and clinical representations were further investigated by correlation analysis. Dystonia was diagnosed by comparing all WD muscle biomechanical parameters with healthy controls (HCs). Compared with HCs, FC decreased from all subregions in the putamen except the right ventral posterior part to the middle cingulate cortex (MCC) and decreased FC of all subregions in the putamen except the left ventral anterior part to the cerebellum was observed in patients with WD. Patients with WD also showed decreased FC of the left globus pallidus primarily distributed in the MCC and cerebellum and illustrated decreased FC from the right globus pallidus to the cerebellum. FC from the putamen to the MCC was significantly correlated with psychiatric symptoms. FC from the putamen to the cerebellum was significantly correlated with muscle tension and neurological symptoms. Additionally, the FC from the globus pallidus to the cerebellum was also associated with muscle tension. Together, these findings highlight that lenticular nucleus–cerebellum circuits may serve as neural biomarkers of dystonia and provide implications for the neural mechanisms underlying dystonia in WD.

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Tackling the neurological manifestations in Wilson’s disease – currently available treatment options

Litwin,

Dusek,

Antos

et al. 2023

Expert Review of Neurotherapeutics

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Botulinum toxin type A treatment of four cases of Wilson disease with lower limb dystonia: A prospective study

Cited by 3 publications

References 20 publications

Role of Botulinum Toxin in Treatment of Secondary Dystonia: A Case Series and Overview of Literature

Role of Botulinum Toxin in Treatment of Secondary Dystonia: A Case Series and Overview of Literature

Dysfunction of the Lenticular Nucleus Is Associated with Dystonia in Wilson’s Disease

Tackling the neurological manifestations in Wilson’s disease – currently available treatment options

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