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Dystonia and Dystonic Syndromes pp 89–99Cite as

Dystonia in Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration

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Abstract

Dystonia associated with parkinsonism may occur in various clinical settings. Clinicopathological studies have revealed that dystonia is present in all atypical parkinsonian syndromes, including multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. The phenomenology and evolution over time of dystonia may vary. The recognition of specific postural abnormalities in multiple system atrophy (antecollis, Pisa syndrome, limb dystonia, diurnal laryngeal stridor, craniocervical dystonia), progressive supranuclear palsy (limb dystonia, blepharospasm, nuchal dystonia), and corticobasal degeneration (upper limb dystonia, cervical dystonia, cranial dystonia) may be of diagnostic value in patients presenting with parkinsonism. In addition to the deformity it causes, dystonia may reduce dexterity and interfere with activities of daily living and gait, increase the likelihood of falling, and produce discomfort and pain. Consequently, the presence of dystonia may exacerbate overall functional disability, which is already high in patients affected by these conditions. Dystonic features associated with atypical parkinsonism may be treated with pharmacological or non-pharmacological approaches as necessary. In this regard, a reduction in or withdrawal of levodopa therapy should be considered if a close relationship between the onset of involuntary movements/contractions and dopaminergic therapy is documented. Botulinum toxin therapy may be recommended, in selected cases, as replacement therapy for both motor and nonmotor symptoms.

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Authors and Affiliations

  1. Department of Neurology and Psychiatry, Sapienza University of Rome, Viale dell’Università, 30, 00185, Rome, Italy

    Luca Marsili & Carlo Colosimo

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  1. Luca Marsili
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  2. Carlo Colosimo
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Correspondence to Carlo Colosimo .

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Editors and Affiliations

  1. Department of Neurology, Palacky University Medical School University Hospital Olomouc, Olomouc, Czech Republic

    Petr Kanovsky

  2. Sobell Department of Motor Neuroscience and Movement Disorders, University College London Institute of Neurology, London, United Kingdom

    Kailash P. Bhatia

  3. Department of Neurology and Psychiatry, The Royal and Pontifical University of Santo Tomas, Manila, Philippines

    Raymond L. Rosales

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Marsili, L., Colosimo, C. (2015). Dystonia in Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration. In: Kanovsky, P., Bhatia, K., Rosales, R. (eds) Dystonia and Dystonic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1516-9_7

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  • DOI: https://doi.org/10.1007/978-3-7091-1516-9_7

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