Dystonia: The Syndrome, Its Term, Concept and Their Evolution

At the end of the 1970s, dystonia was seen as something peculiar, balancing on the edge between organic disorder and psychiatric condition. The everlasting enigma of dystonia has been breeched for the first time by David Marsden, who attributed the clinic

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Dystonia: The Syndrome, Its Term, Concept and Their Evolution Petr Kanovsky, Raymond L. Rosales, and Kailash P. Bhatia

The book fully dedicated to dystonia should start with this word. Nevertheless, going into history, this term has been invented relatively late. What we should mention are the paths of the syndrome in the medical and literary history. Notorically known are the artistic descriptions of torticollis by Dante (La Divina Commedia) and Rabelais (Gargantua and Pantagruel). The first medical depiction of dystonia was probably that of Gowers’ from 1888, where the movement disorder was named “tetanoid chorea” (Fig. 1.1) [1]. Destarac used the term “torticolis spasmodique” to describe the cervical dystonia [2]. Schwalbe used in his doctoral thesis the term “tonic cramps” (Fig. 1.2) [3]. The word “dystonia” has been used for the first time by Oppenheim in 1911 in the form “dystonia musculorum deformans” (Fig. 1.3) [4]. Flatau and Sterling in the same year tried to name the disorder in a more innovative manner and used the term “progressive torsion spasms” (Fig. 1.4) [5]. The term “dystonia” prevailed in the neurological literature and has been used in the form “torsion dystonia” after Meige [6]. Its nature remained practically unknown for more than 50 years. At the end of the 1970s, dystonia was seen as something peculiar, balancing on the edge between organic disorder and psychiatric condition. The everlasting enigma of dystonia has been breached for the first time by David Marsden, who attributed the clinical syndrome to a disorder of basal ganglia

P. Kanovsky (*) Department of Neurology, Palacky University Medical School, Olomouc, Czech Republic e-mail: [email protected] R.L. Rosales, MD, PhD Department of Neurology and Psychiatry, The Royal and Pontifical University of Santo Tomas, Manila, Philippines e-mail: [email protected] K.P. Bhatia Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College London, Queen Square, London, UK 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_1

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4 Fig. 1.1 William Richard Gowers (1845–1915)

Fig. 1.2 Gustav Schwalbe (1844–1916)

Fig. 1.3 Hermann Oppenheim (1858–1919)

P. Kanovsky et al.

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Dystonia: The Syndrome, Its Term, Concept and Their Evolution

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Fig. 1.4 Edward Flatau (1868–1932)

Fig. 1.5 C. David Marsden (1938–1998)

functioning. As he postulated in the seminal work regarding the natural history and clinical presentation of dystonia, dystonia is a syndrome of sustained involuntary muscle contraction, frequently causing twisting or repetitive movements or abnormal postures (Fig. 1.5) [7, 8]. Dystonia has been described as an involuntary movement, which was (in principle) independent of the patient’s aim to prime the movement (or rest) of a certain body part. Although a new definition of dystonia has been recently adopted [9], Marsden’s concept is still a cornerstone of it. One can undoubtedly chara