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The case of Cassian H in 1893 and his importance to the history of the extrapyramidal movement disorders
  1. E Kumbier,
  2. K Haack
  1. Department of Psychiatry and Psychotherapy, Rostock University, Germany
  1. Correspondence to:
 Ekkehardt Kumbier
 Department of Psychiatry and Psychotherapy, Rostock University, Gehlsheimer Straße 20, D-18147 Rostock, Germany; ekkehardt.kumbiermedizin.uni-rostock.de

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In the years 1893 and 1896 the neuropsychiatrist Gabriel Anton (1858–1933) reported on the role of the basal ganglia in choreic movements.1,2 He described the case of a boy with choreoathetosis and suspected isolated changes in the corpus striatum to be the cause. Until then, theories of normal brain function were not prominent and clinical disorders were poorly described. The explanations of Otto Kahler (1849–1893), Arnold Pick (1851–1924) (“Pyramidenreiztheorie”), and Jean-Martin Charcot (1825–1893) (the existence of choreabundles) were not accepted generally.

Today, Anton’s name is known by Anton’s syndrome and the Anton-von Bramannsche Balkenstich method.* Although Anton’s contribution to research on basal ganglia disorders is less well known, undoubtedly it represents an important advance in the systematic research of movement disorders initiated by Oskar Vogt (1870–1959) and especially by Cécile Vogt (1875–1962) at the beginning of the 20th century. Anton’s attempts were developed as a pathophysiological explanation concerning the causal relationship between changes in the striatum and the development of choreoathetosis.

The case of Cassian H

In 1891, Anton became an associate professor of psychiatry and neuropathology in Innsbruck. From there he reported the case of the 9 year old Cassian Huber, son of a farmer, whom he had examined in the department of psychiatry. At the age of 9 months the patient was taken ill with scarlet fever and suddenly, one week later, general hyperkinesis was evident. This early physical and mental development had not been disturbed. Anton described involuntary movements in the extremities on both sides. First, he believed it was a generalised chorea but had difficulty in distinguishing the choreiform and the typical athetotic movements. Muscle tone was normal and paraesthesiae lacking. The pathological-anatomic examination after death from scarlatina only showed bilateral lesions of the striatum. In the lenticular nuclei of the putamina, the grey matter had dissolved and, in between, white matter was visible. The pyramidal tract appeared intact. He suspected the isolated lesions in the putamina to be the cause of the choreoathetosis, resulting from vascular underperfusion after an infectious disorder, perhaps with a hereditary disposition.

The contribution to research on the functions of the basal ganglia

Taking into consideration the clinical symptoms and the neuropathological findings in the case of Cassian H, Anton suspected the complex interaction of the basal ganglia had been disturbed. He concluded that, through the absence of inhibition of movement, the necessary precondition for normal movements disappeared. He assumed the involuntary movements were caused by the isolated changes in the corpus striatum. Furthermore he concluded that, because of the existence of an intact pyramidal tract, an extrapyramidal tract must also exist.

Anton’s first description of the changes in the striatum was later named status marmoratus (état marbré) by Cécile Vogt. She discovered the relationship between this and the extrapyramidal movement disorders. Cécile Vogt reported several case studies portraying similarities and from this is derived the term “syndrôme du corps strié”. The status marmoratus in the striatum (Vogt’s syndrome) was considered the cause of double athetosis (athétose double) in childhood. Cécile Vogt, with her husband Oskar Vogt, subdivided the basal ganglial disorders on the basis of the characteristic neuropathological lesions.4

The neurologist Alfons Jakob (1884–1931) referred to the differences between the case of Cassian H and other patients with status marmoratus.5 In contrast to Vogt’s description, Cassian H showed normal development after birth. Later, after scarlatina in the ninth month of life, the involuntary movements were observed for the first time.

The characteristic history in the case of Cassian H supports the possibility of a post-infectious, autoimmune mechanism. On views current today, it is possible Cassian H suffered from the condition described in 1686 by Thomas Sydenham (1624–1689) and now named Sydenham’s chorea. Another early medical description of chorea was given by the Dutch physician Steven Blankaart (Stephanus Blancardus, 1650–1704) in his Lexicon Medicum Greco-Latino-Germanicum in 1696.6

With his work on the functions of the basal ganglia, Anton made an important contribution to the research of movement disorders.

References

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Footnotes

  • * One of Anton’s most renowned scientific achievements was the Anton-von Bramannsche Balkenstich method. In collaboration with the surgeon Gustav von Bramann (1854–1913), he proposed a new clinical procedure for the treatment of hydrocephalus: the Balkenstich method.3 This operational procedure was recommended to release pressure in hydrocephalus.

  • Competing interests: Although Professor Didier Leys is an associate editor of the journal the manuscript was handled by Professor Martin Rossor, editor in chief. Didier Leys did not participate in any stage of the editorial process for this manuscript.

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