Dystonia in Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration
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
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Dystonia in Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration Luca Marsili and Carlo Colosimo
Dystonia associated with parkinsonism may occur in various clinical settings. Rare hereditary conditions involving the basal ganglia, such as Wilson’s disease, Huntington’s disease, neurodegeneration with brain iron accumulation type 1 disease (NBIA1), and familial basal ganglia calcifications, are frequently characterized by both these symptoms [1–3]. Generalized dystonia with parkinsonian features is also a classical presentation of dopa-responsive dystonia [4]. As regards more common sporadic neurodegenerative disorders, dystonia is frequently observed in Parkinson’s disease (PD) patients experiencing motor complications, though it may also be seen, usually in the form of foot or hand posturing, in early untreated patients. Clinicopathological studies have also revealed that dystonia is frequently present in all atypical parkinsonian syndromes (APS), such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The phenomenology and evolution over time of dystonia in APS varies [2, 5–7]. The recognition of specific postural abnormalities in APS might be of diagnostic value as such abnormalities are often mistakenly attributed to rheumatic or orthopedic problems when present at disease onset, particularly if the typical parkinsonian signs are not evident. In addition to the deformity it causes, dystonia may reduce dexterity and interfere with activities of daily living and gait, increase the likelihood of falls, and induce discomfort and pain. Consequently, the presence of dystonia may exacerbate overall functional disability, which is already high in patients affected by APS [8, 9] (Table 7.1).
L. Marsili • C. Colosimo (*) Department of Neurology and Psychiatry, Sapienza University of Rome, Viale dell’Università, 30, 00185 Rome, Italy e-mail: [email protected] © Springer-Verlag Wien 2015 P. Kanovsky et al. (eds.), Dystonia and Dystonic Syndromes, DOI 10.1007/978-3-7091-1516-9_7
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Table 7.1 Main types of dystonia and relative frequencies in atypical parkinsonism Type of dystonia Blepharospasm Laryngeal stridor Craniocervical Antecollis Retrocollis Limb dystonia
MSA
PSP
− ++ ++a ++ − ±
++ − ± ± + ++
CBD ± − ± − ± ++
(++) very common, (+) common, (±) uncommon, (−) occasional This is frequently drug induced
a
7.1
Multiple System Atrophy
The lack of detailed studies on dystonia in pathologically proven MSA means it is difficult to define its prevalence, nature, and extent in such patients: while the first review of the literature on this topic, conducted by Rivest and coworkers, concluded that dystonia is relatively rare in pathologically confirmed MSA [2], a subsequent prospective clinical study suggested that dystonia is common in untreated MSA-P patients, it being observed in 42 % of patients [6]. According to Boesch and coworkers (24 cases) and Kollensperger and coworkers (100
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