Table Of ContentFederico E. Micheli
Peter A. LeWitt Editors
Chorea
Causes and
Management
Chorea
Federico E. Micheli (cid:129) Peter A. LeWitt
Editors
Chorea
Causes and Management
Editors
Federico E. Micheli, MD, PhD Peter A. LeWitt, MD
Parkinson Disease and Movement Disorders Parkinson’s Disease and Movement
Program and Neurology Department Disorders Program
Hospital de Clínicas, José de San Martín Henry Ford West Bloomfi eld Hospital
Buenos Aires West Bloomfi eld
Argentina Michigan
USA
ISBN 978-1-4471-6454-8 ISBN 978-1-4471-6455-5 (eBook)
DOI 10.1007/978-1-4471-6455-5
Springer London Heidelberg New York Dordrecht
Library of Congress Control Number: 2014943524
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To my family whose emotional and intellectual
support has served as an inspiration to make
this book possible. To my patients, from whom
I learn every single day.
Federico E. Micheli
Dedicated, in appreciation of their love and
support, to Jan, Eli, and Tessa, and to my
parents Bernard and Celeste.
Peter A. LeWitt
Prol ogue
Within the broad categories of neurologic diseases, the abnormal movement c horea
(from Greek, choros, and from the Latin, choreus, both meaning dance) belongs
among the conditions referred to as movement disorders. The fi eld of movement
disorders consists of both hypokinetic and hyperkinetic conditions. The hypokinetic
group comprises those disorders that manifest slowness and decreased amplitude of
movement, of which parkinsonism is by far the major type and the prime example
and has early cardinal signs of bradykinesia, rigidity, and tremor-at-rest. In contrast,
the hyperkinetic group has a much larger range of disorders, represented by differ-
ent motor phenomenologies. The big six in this group – in alphabetical order – are
the ataxias, choreas, dystonias, myoclonias, tics and tremors. The fi rst step in diag-
nosing these hyperkinetic conditions is the precise awareness of the phenomenol-
ogy so that the correct type of hyperkinesia can be identifi ed. Then the clinical
workup searching for a specifi c etiology of the abnormal movements is undertaken;
this can include genetic testing, neuroimaging, investigations for neuroinfl amma-
tion, hepatic and other metabolic disorders, and other possible etiologies. Depending
on the etiology, the appropriate treatment is then applied [5].
Drs. Micheli and LeWitt have embarked on an ambitious project, putting together
in a single monograph the complex fi eld of the choreas. Other than tremor ( mentioned
in the Bible), chorea was the earliest hyperkinesia to be described [1]. Thomas
Sydenham, an English physician known as the English Hippocrates because of his
keen observations and sound clinical judgment, gave the fullest description of the
phenomenology of chorea in children with the condition now referred to as
Sydenham chorea in 1686 [13], more than 100 years earlier than James Parkinson
[12] described the condition now bearing his name. Sydenham did not use the term
chorea , but called the condition he was describing as S t. Vitus ’ D ance . This was a
misapplied term, resulting in confusion, for St. Vitus’ Dance was initially used in
reference to the dancing mania that occurred in waves of epidemics in the middle
ages (fourteenth and fi fteenth centuries). As interpreted by Paracelsus, these danc-
ing manias were likely psychogenic in etiology, and today we would describe them
as a form of mass hysteria. (Note: Paracelsus’ born name was Philippus Aureolus
Theophrastus Bombastus von Hohenheim. The English translation of his writing
vii
viii Prologue
about the dancing mania is given in monographs by Temkin and colleagues [14] and
Ehrenwald [4]).
A ccording to Barbeau [1], Bruyn [3], Hayden [8], and Harper [7], Paracelsus
was the fi rst to use the term chorea (in the sixteenth century) when he discussed the
dancing mania (St. Vitus’ Dance). Paracelsus called this c horea lasciva (chorea
from voluptuous desires). He offered two other types of chorea, namely chorea
imaginativa aestimative (caused by imagination) and c horea naturalis coatta (aris-
ing from an organic cause). But Paracelsus provided no detailed description of the
choreic movements we recognize today; that credit goes to Sydenham. The chorea
described by Sydenham was subsequently referred to as c horea minor, and the
dancing mania as c horea major [8].
F ollowing Sydenham’s publication, it was likely that patients with a variety of
abnormal involuntary movements were subsequently being called chorea, as empha-
sized by William Osler in his treatise, O n Chorea and Choreiform Affections [11].
Attempts to classify all the movement disorders was rather recent in medical his-
tory. Baumann [2] attempted a classifi cation based on neuroanatomical localization.
Lakke et al. [10] advanced the fi eld by classifying the conditions based on phenom-
enology of the movements, posture and muscle tone (see history of classifi cation in
Fahn [5], which also provides a modern classifi cation). An advance in classifying
the choreas was the growing recognition of hereditary forms of chorea. A number
of such reports were published prior to the paper of George Huntington in 1872 [9],
but it was Huntington’s precise and brief description that made a major impact.
What made his description stand out, besides the hereditary aspect and the abnormal
movements, were his inclusion of features of inappropriate behavior that could lead
to insanity and a relentlessly progressive course of the illness.
T he abnormal movements of chorea are involuntary, irregular, purposeless, non-
rhythmic, abrupt, rapid, and unsustained that seem to fl ow from one body part to
another (from Fahn et al. [6]). Choreic movements are unpredictable in timing,
direction, and distribution (i.e., random). Distinguishing chorea from dystonic
movements, tics and myoclonus is not always easy, and reports in the literature of
unusual cases of chorea need to be viewed with caution, for they could have simply
been misdiagnosed. Choreic movements can be of small amplitude involving just
the digits of the fi ngers or toes to fl inging and fl ailing limb movements of the entire
limb; the latter condition is usually called ballism, which is simply chorea of
extremely large amplitude movements. The phenomenology of choreic movements
can differ slightly from one disorder to another. For example, the chorea of
Sydenham disease and of withdrawal emergent syndrome (seen in children who
were suddenly withdrawn from chronic treatment with dopamine receptor blocking
agents) have a restless appearance with fi dgetiness, in contrast to the more brief and
abrupt involuntary movements seen in Huntington disease and the slightly slower
abnormal movements of choreoathetosis seen in hyperosmotic hyperglycemic non-
ketotic syndrome that can occur in patients with diabetes mellitus with extremely
high blood sugar levels. When chorea is somewhat slow, it thereby merges into the
mobile spasms of athetosis and the twisting movements of dystonia; this combina-
tion is usually referred to as choreoathetosis.
Prologue ix
T his monograph edited by Drs. Micheli and LeWitt provides the details and
revealing discussions to explain the various disorders that can produce chorea.
These chapters will provide rewarding reading to both the expert movement disor-
der specialist and to others in the health profession with less subspecialization.
There is much valuable information in this book.
References
1. Barbeau A. The understanding of involuntary movements: an historical approach. J Nerv Ment
Dis. 1958;127(6):469–89.
2. Baumann J. The classifi cation of the diseases of the extrapyramidal system. Acta Neurol Scand
Suppl. 1963;39(S4):102–7.
3. Bruyn GW. Huntington’s chorea: historical, clinical and laboratory synopsis. In: Vinken PJ,
Bruyn GW, editors. Handbook of clinical neurology. Vol. 6, Amsterdam: North-Holland Publ
Co; 1968. p. 300–1.
4. Ehrenwald J. Chapter 14. Paracelsus, the magician who turned scientist. In: Ehrenwald J, edi-
tor. The history of psychotherapy: from healing magic to encounter. Northvale: Jason Aronson
Inc.; 1991. p. 199–200. Available in Google Books.
5. Fahn S. Classifi cation of movement disorders. Mov Disord. 2011;26(6):947–57.
6. Fahn S, Jankovic J. Hallett M. Chapter 1. Principles and practice of movement disorders. 2nd
ed. Edinburgh: Saunders Elsevier; 2011.
7. Harper P, Morris M. Chapter 1. Introduction: a historical background. In: Harper PS, editor.
Huntington’s disease. London: W.B. Saunders Ltd.; 1991. p. 9.
8. Hayden MR. Huntington’s Chorea. Berlin: Springer; 1981.
9. Huntington G. On chorea. Medical and surgical reporter. 1872;26:320–1.
10. Lakke JPWF, editor. Classifi cation of extrapyramidal disorders. J Neurol Sci. 1981;51:
311–27.
11. Osler W. On chorea and choreiform affections. Philadelphia: Blakison; 1894.
12. Parkinson J. An essay on the shaking palsy. London: Sherwood, Neely, and Jones; 1817.
13. Sydenham T. Schedula Monitoria de Novae Febris Ingressu. London: G. Kettilby; 1686.
1 4. Temkin CL, Rosen C, Zilboorg G, Sigerist HE. Theophrastus von Hohenheim called
Paracelsus. Baltimore: Johns Hopkins Press; 1941.
New York , NY , USA Stanley Fahn , MD
