Corticosteroids in the Treatment of Sydenham's Chorea Leonard N.
Green,
MD
is a retrospective study of the treatment of eight female patients with Sydenham's chorea by corticosteroid administration. The rationale for use of this medication is based on the concept that Sydenham's chorea is caused by an indolent inflammation of the small vessels in the caudate-putamen complex. All patients in this series responded to corticosteroid treatment relatively rapidly, with considerable diminution in abnormal movements. It is concluded that corticosteriod treatment of this condition is effective and deserves wider use. This report should serve as an impetus for more controlled studies. (Arch Neurol 35:53-54, 1978) \s=b\ This
has been 26 years since Aronson and coauthors1 wrote of the use of cortisone in two patients with Syden¬ ham's chorea. In 1955, Lorin Ainger et al2 reported on the so called "dramat¬ ic" response to corticotropin treat¬ ment he achieved in 13 attacks of Sydenham's chorea in 11 patients. Since then, there has been a relative^ silence in the English language litera¬ ture regarding the efficacy of corti¬ costeroid therapy in this condition. Review of more recent textbooks regarding the treatment of Syden¬ ham's chorea shows either omission of reference to corticosteroids or only passing reference, without clear-cut recommendation for its use.36 There is some suggestion in the literature that the pathogenetic mechanism in Sydenham's chorea involves a mild inflammatory reaction of small ves¬ sels78 most likely involving the caudate-putamen complex. Because of the author's favorable experience with treatment of an initial patient with corticosteroids, seven additional con¬ secutive patients with Sydenham's chorea were treated with corticoste-
It
for publication June 30, 1977. From the section of Neurology, the Department of Medicine, Youngstown Hospital Association, Youngstown, Ohio. Reprint requests to 510 Gypsy Lane, Youngstown, OH 44504 (Dr Green).
Accepted
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/15/2015
roids and the uniformly favorable results are herewith reported. MATERIALS AND METHODS The records of eight consecutive patients with Sydenham's chorea who were admit¬ ted to a general hospital under the care of the author, between 1969 and 1976 were reviewed and analysed (Table). All eight were young female patients whose ages ranged from 9 to 21 years. Five of the eight were initially evaluated within two weeks of the onset of abnormal movements. One patient, the only adult in the series, had abnormal movements for seven months before the diagnosis was established. This patient had a normal pneumoencephalogram as well as other negative test results
(viz, lupus preparations, heterophile titer)
to exclude other causes of chorea in a young adult. She did not have Keyser-
Fleischer rings. Motion pictures were obtained from three patients before and after treatment to document improve¬ ment.
eight patients had a clear-cut history of prior pharyngitis, and
Only
two of the
in one, it was nine months before the onset of chorea. Four of the eight patients had some cardiac abnormality. All but one had an elevation of the antistreptolysin O titer, but that one patient (patient 6) had an elevated sedimentation rate. The elevation of the antistreptolysin O titer in patient 1 was minimal. All patients were treated with either corticotropin, prednisone, or a combination of both, as outlined in the table. All but one
Findings Patient/Age, yr 1/21
2/10 3/11 4/9 5/9 6/17 7/14
8/10
Duration of Chorea 7 mo 1 mo 8 days 7 days 14 days 5 days 7
ESR, mm/hr 14 12 21 17 17 30
Antistreptolysin Titer 166
333 333 600
Prednisone
Therapy
625
333
weeks from
patient received an antibiotic, either peni¬ cillin, erythromycin, or ampicillin. Patient 1 and 6 were treated with haloperidol prior
within
to treatment with corticosteroids and had
because of the chorea was able to regain her typing job. Patient 7, who had a somewhat slower response expe¬ rienced a recurrence of chorea after cessation of prednisone treatment so that a second course of alternate day prednisone therapy was reinstituted for a total of three months. She responded as favorably to this second course of treatment, although with lower doses, as she had to the f first course. All patients were observed for at least six months after cessation of therapy and had no recurrences.
shown
improvement. The duration of treatment, except for patients 7 and 8, was not more than three weeks. no
RESULTS All of the patients showed a marked reduction in the frequency and severi¬ ty of their choreic movements within a few days. This improvement was definite and noticeable by patient, family, and physician, taking no longer than three days from onset of corticosteroid therapy in all patients except patient 7, in whom improve¬ ment occurred in three to five days (Table). At the time of discharge from the hospital, chorea was either absent or barely noticeable. All the children were able to resume their school work
40 30 40 40
mg/day for 7 days mg/day for 5 days 30 mg/day for 7 days 60 mg/day for 5 days and 25 mg/day for 14 days 75 mg/day for 8 days, 50 mg/day for 30 days, and 50 mg/day every other day for 90 days 60 mg/day for 10 days, 40 mg/day for 14 days, and 30 mg/day for 30 days
50
16
Sydenham's Chorea
45 40
1,250
days
1 mo
in Patients With
hospital discharge. The young adult, patient 1, who had lost her job as a clerk-typist two
40 40
40
a
rela-
2-3 2-3 2-3 1-2 2-3
units/day
for 6
2-3
days
in their movement disorder. In the one case in which the improvement was not sustained on cessation of medication, a second course of treatment rapidly produced a remission. It is difficult to escape the conclusion that corticoste¬ roid treatment has a direct beneficial effect on this movement disorder and in some way, alters the pathological
tively rapid improvement
process,
presumably
an
inflammatory
one.
Although this report is not a controlled study, the favorable and rapid response of all eight patients to corticosteroid treatment should as an impetus for such a study.
serve
Name and Trademarks of Drug
Nonproprietary
In this
with corticosteroids and made
Response Time, Days
3-5
CONCLUSION
retrospective study, eight consecutively encountered patients with Sydenham's chorea were treated
Corticotropin Therapy units/day for 9 days units/day for 7 days units/day for 7 days units/day for 3 days units/day for 6 days un its/day for 6 days
Corticotropin— Acortan, Acthar, phin, Depo-ACTH, Duración.
Cortro-
References 1. Aronson N, Douglas HS, Lewis JM: Cortisone in Sydenham's chorea: Report of two cases. JAMA 145:230-233, 1951. 2. Ainger LE, Ely RS, Done AK: Sydenham's chorea: Effects of hormone therapy. Am J Dis Child 89:580-590, 1955. 3. Lamm SS; Pediatric Neurology. New York, Appleton-Century-Crofts, 1959, chap 8, p 233.
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/15/2015
4. Farmer TW (ed): Pediatric Neurology. New York, Harper and Row, Publishers, 1964, chap 11, p 519. 5. Calne DB: Therapeutics in Neurology.
London, Blackwell Scientific Publications, 1975, chap 14, p 213. 6. Ford FR: Diseases of the Nervous System in Infancy, Childhood, and Adolescence. Springfield,
Ill, Charles C Thomas, Publisher, 1966, chap 4,
p 588.
JC, Wolfsohn JM: The pathology of Sydenham's chorea. Lancet 2:603-606, 1922. 8. Winkelman NW, Eckel JL: The brain in 7. Greenfield
acute rheumatic fever. Arch Neurol
28:844-870, 1932.
Psychiatry
Green,
MD
is a retrospective study of the treatment of eight female patients with Sydenham's chorea by corticosteroid administration. The rationale for use of this medication is based on the concept that Sydenham's chorea is caused by an indolent inflammation of the small vessels in the caudate-putamen complex. All patients in this series responded to corticosteroid treatment relatively rapidly, with considerable diminution in abnormal movements. It is concluded that corticosteriod treatment of this condition is effective and deserves wider use. This report should serve as an impetus for more controlled studies. (Arch Neurol 35:53-54, 1978) \s=b\ This
has been 26 years since Aronson and coauthors1 wrote of the use of cortisone in two patients with Syden¬ ham's chorea. In 1955, Lorin Ainger et al2 reported on the so called "dramat¬ ic" response to corticotropin treat¬ ment he achieved in 13 attacks of Sydenham's chorea in 11 patients. Since then, there has been a relative^ silence in the English language litera¬ ture regarding the efficacy of corti¬ costeroid therapy in this condition. Review of more recent textbooks regarding the treatment of Syden¬ ham's chorea shows either omission of reference to corticosteroids or only passing reference, without clear-cut recommendation for its use.36 There is some suggestion in the literature that the pathogenetic mechanism in Sydenham's chorea involves a mild inflammatory reaction of small ves¬ sels78 most likely involving the caudate-putamen complex. Because of the author's favorable experience with treatment of an initial patient with corticosteroids, seven additional con¬ secutive patients with Sydenham's chorea were treated with corticoste-
It
for publication June 30, 1977. From the section of Neurology, the Department of Medicine, Youngstown Hospital Association, Youngstown, Ohio. Reprint requests to 510 Gypsy Lane, Youngstown, OH 44504 (Dr Green).
Accepted
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/15/2015
roids and the uniformly favorable results are herewith reported. MATERIALS AND METHODS The records of eight consecutive patients with Sydenham's chorea who were admit¬ ted to a general hospital under the care of the author, between 1969 and 1976 were reviewed and analysed (Table). All eight were young female patients whose ages ranged from 9 to 21 years. Five of the eight were initially evaluated within two weeks of the onset of abnormal movements. One patient, the only adult in the series, had abnormal movements for seven months before the diagnosis was established. This patient had a normal pneumoencephalogram as well as other negative test results
(viz, lupus preparations, heterophile titer)
to exclude other causes of chorea in a young adult. She did not have Keyser-
Fleischer rings. Motion pictures were obtained from three patients before and after treatment to document improve¬ ment.
eight patients had a clear-cut history of prior pharyngitis, and
Only
two of the
in one, it was nine months before the onset of chorea. Four of the eight patients had some cardiac abnormality. All but one had an elevation of the antistreptolysin O titer, but that one patient (patient 6) had an elevated sedimentation rate. The elevation of the antistreptolysin O titer in patient 1 was minimal. All patients were treated with either corticotropin, prednisone, or a combination of both, as outlined in the table. All but one
Findings Patient/Age, yr 1/21
2/10 3/11 4/9 5/9 6/17 7/14
8/10
Duration of Chorea 7 mo 1 mo 8 days 7 days 14 days 5 days 7
ESR, mm/hr 14 12 21 17 17 30
Antistreptolysin Titer 166
333 333 600
Prednisone
Therapy
625
333
weeks from
patient received an antibiotic, either peni¬ cillin, erythromycin, or ampicillin. Patient 1 and 6 were treated with haloperidol prior
within
to treatment with corticosteroids and had
because of the chorea was able to regain her typing job. Patient 7, who had a somewhat slower response expe¬ rienced a recurrence of chorea after cessation of prednisone treatment so that a second course of alternate day prednisone therapy was reinstituted for a total of three months. She responded as favorably to this second course of treatment, although with lower doses, as she had to the f first course. All patients were observed for at least six months after cessation of therapy and had no recurrences.
shown
improvement. The duration of treatment, except for patients 7 and 8, was not more than three weeks. no
RESULTS All of the patients showed a marked reduction in the frequency and severi¬ ty of their choreic movements within a few days. This improvement was definite and noticeable by patient, family, and physician, taking no longer than three days from onset of corticosteroid therapy in all patients except patient 7, in whom improve¬ ment occurred in three to five days (Table). At the time of discharge from the hospital, chorea was either absent or barely noticeable. All the children were able to resume their school work
40 30 40 40
mg/day for 7 days mg/day for 5 days 30 mg/day for 7 days 60 mg/day for 5 days and 25 mg/day for 14 days 75 mg/day for 8 days, 50 mg/day for 30 days, and 50 mg/day every other day for 90 days 60 mg/day for 10 days, 40 mg/day for 14 days, and 30 mg/day for 30 days
50
16
Sydenham's Chorea
45 40
1,250
days
1 mo
in Patients With
hospital discharge. The young adult, patient 1, who had lost her job as a clerk-typist two
40 40
40
a
rela-
2-3 2-3 2-3 1-2 2-3
units/day
for 6
2-3
days
in their movement disorder. In the one case in which the improvement was not sustained on cessation of medication, a second course of treatment rapidly produced a remission. It is difficult to escape the conclusion that corticoste¬ roid treatment has a direct beneficial effect on this movement disorder and in some way, alters the pathological
tively rapid improvement
process,
presumably
an
inflammatory
one.
Although this report is not a controlled study, the favorable and rapid response of all eight patients to corticosteroid treatment should as an impetus for such a study.
serve
Name and Trademarks of Drug
Nonproprietary
In this
with corticosteroids and made
Response Time, Days
3-5
CONCLUSION
retrospective study, eight consecutively encountered patients with Sydenham's chorea were treated
Corticotropin Therapy units/day for 9 days units/day for 7 days units/day for 7 days units/day for 3 days units/day for 6 days un its/day for 6 days
Corticotropin— Acortan, Acthar, phin, Depo-ACTH, Duración.
Cortro-
References 1. Aronson N, Douglas HS, Lewis JM: Cortisone in Sydenham's chorea: Report of two cases. JAMA 145:230-233, 1951. 2. Ainger LE, Ely RS, Done AK: Sydenham's chorea: Effects of hormone therapy. Am J Dis Child 89:580-590, 1955. 3. Lamm SS; Pediatric Neurology. New York, Appleton-Century-Crofts, 1959, chap 8, p 233.
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/15/2015
4. Farmer TW (ed): Pediatric Neurology. New York, Harper and Row, Publishers, 1964, chap 11, p 519. 5. Calne DB: Therapeutics in Neurology.
London, Blackwell Scientific Publications, 1975, chap 14, p 213. 6. Ford FR: Diseases of the Nervous System in Infancy, Childhood, and Adolescence. Springfield,
Ill, Charles C Thomas, Publisher, 1966, chap 4,
p 588.
JC, Wolfsohn JM: The pathology of Sydenham's chorea. Lancet 2:603-606, 1922. 8. Winkelman NW, Eckel JL: The brain in 7. Greenfield
acute rheumatic fever. Arch Neurol
28:844-870, 1932.
Psychiatry
