An Early Medical Illustration of Paroxysmal Dystonic Phenomena

Dystonia has a long history in medical literature. Before Hermann Oppenheim (1858–1919) introduced the term dystonia in 1911, ¹ torticollis had been described in medical accounts dating back to the 17th century, ² and cases of hemidystonia had been previously classified within the context of athetosis, first described by William Alexander Hammond (1828–1900) in 1871. ³

Furthermore, dystonia had probably been represented in visual art and literature well before its first medical descriptions. ² Herein, we report what to our knowledge is the earliest illustration of dystonia published in the medical literature, preceding by 10 years the famous drawing of the writer's cramp provided by William Gowers (1845–1915) in the second volume of “A Manual of Diseases of the Nervous System”. ⁴ As Gowers pointed out, writer's cramp had been previously described by Sir Charles Bell (1774–1842) in 1830. ⁴

In the “Iconographie photographique de la Salpêtrière” (1878) Désiré Magloire Bourneville (1840–1909) and Paul Regnard (1850–1927) presented a woman diagnosed with “partial epilepsy with tonic component or contracture” (“épilepsie partielle tonique ou avec contracture”). ⁵ The case was beautifully illustrated by Paul Richer (1849–1933). The paroxysmal episodes of the patients were characterized by a marked torsional and rotatory movements with the forearm drawn across the dorso‐lumbar region, sometimes involving also the inferior extremity (Fig. 1; Appendix S1). This case was interpreted as a paradigmatic example of “partial epilepsy of a tonic character with contracture”; actually, head version with ictal limb dystonia and preserved awareness could be seen in frontal seizures. However, the marked and fixed head position ipsilateral to the extended (not flexed) right arm, followed by a marked rotation of the arm behind the dorsum are not characteristic of focal seizures, ⁶ and could point to a diagnosis of dystonia. However, other features are not typically encountered in dystonia (mydriasis, face becoming pale, eyes spasmodically closed, complaints of “flashes of light shooting before the eyes”; Appendix S1). The lack of further details on clinical course and symptoms prevent us from reaching an established retrospective diagnosis of the paroxysmal dystonic phenomena presented by this patient.

FIG. 1.

Drawings by Paul Richer (1849–1933) showing a female patient with paroxysmal dystonic phenomena. They represent the earliest illustrations of dystonic phenomena known so far in the medical literature.

Before EEG was discovered and introduced in clinical practice, epilepsy was only a clinical diagnosis, and most movement disorders were labeled as chorea or athetosis, or were diagnosed as hysteria. In doing so, 19th century neurologists sometimes carried the risk of classifying symptoms and making diagnoses tending to confirm acquired knowledge, instead of recognizing that they were dealing with phenomena that could not be traced back to known clinical entities. Movement disorders that must have occurred in the past were appreciated and recognized as distinct nosological entities until a framework of knowledge was created around a name, allowing the recognition of certain clinical patterns. ² The recent description of movement disorders and epileptic disorders characterized by paroxysmal dystonic phenomena associate with certain autoantibodies ⁷ have blurred the borders between what were previously considered separate nosographic groups, emphasizing the importance of a multidisciplinary approach to reach a deeper physiopathological understanding of these complex and fascinating clinical manifestations.

Author Roles

(1) Research project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript Preparation: A. Writing of the first draft, B. Review and Critique.

F.B.: 1A, 1B, 1C, 3A

E.T.: 3A, 3B

P.B.: 3B

S.G.: 3B

L.L.: 3B

Disclosures

Ethical Compliance Statement

The authors confirm that the approval of an institutional review board was not required for this work. The authors confirm that patient consent was not required for this work. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines.

Funding Sources and Conflicts of Interest

No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work.

Financial Disclosures for the Previous 12 Months

Francesco Brigo declares that there is no additional disclosures to report. Eugen Trinka has received consultation fees and/or speakers honoraria from Angelini, Argenx, Arvelle, Bial, Biogen‐Idec, Boehringer Ingelheim, Eisai, Epilog, EVER Pharma, Genzyme, GL Pharma, GW Pharmaceuticals, Hikma, LivaNova, Marinus, Medtronic, NewBridge, Novartis, Sanofi, and UCB Pharma.

Supporting information

Appendix S1. English translation of the clinical case reported by Bourneville and Regnard. The text was published in: The American Journal of the Medical Sciences, 1879:173–182.