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Review
. 2017 Feb 3:8:18.
doi: 10.3389/fneur.2017.00018. eCollection 2017.

How Many Dystonias? Clinical Evidence

Alberto Albanese  1
Affiliations
Review

How Many Dystonias? Clinical Evidence

Alberto Albanese. Front Neurol. .

Abstract

Literary reports on dystonia date back to post-Medieval times. Medical reports are instead more recent. We review here the early descriptions and the historical establishment of a consensus on the clinical phenomenology and the diagnostic features of dystonia syndromes. Lumping and splitting exercises have characterized this area of knowledge, and it remains largely unclear how many dystonia types we are to count. This review describes the history leading to recognize that focal dystonia syndromes are a coherent clinical set encompassing cranial dystonia (including blepharospasm), oromandibular dystonia, spasmodic torticollis, truncal dystonia, writer's cramp, and other occupational dystonias. Papers describing features of dystonia and diagnostic criteria are critically analyzed and put into historical perspective. Issues and inconsistencies in this lumping effort are discussed, and the currently unmet needs are critically reviewed.

Keywords: definition and concepts; dystonia; history; movement disorders; phenomenology.

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Figures

Figure 1
Figure 1
“Possible interrelations between blepharospasm, oromandibular dystonia, dystonic writer’s cramp, and torticollis/axial dystonia (focal dystonias), and idiopathic torsion dystonia of segmental or generalized type. Common associations are shown by solid lines, and rare transitions by dashed lines” modified from (47). Marsden’s handwritten schemes can be found also in other publications, such as the Roberg Wartenberg lecture (49) or the Phoenix and Firkin beermats (48).
Figure 2
Figure 2
Synopsis table of expected clinical subtypes and status of proposed diagnostic criteria for different dystonias.
See this image and copyright information in PMC

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