Læknablaðið : fylgirit - 01.05.1978, Page 138

Gunnar Husby, J. B. Zabriskie, Z. H. Abdin,
R. C. Williams, Jr.
From Department of Medicine, University of New
Mexico, School of Medicine, Albuquerque, New
Mexico and Department of Pediatrics Free
Rheumatic Heart Center, Cairo, Egypt.
Supported in part by Grants AMAI 13824-05 and
AM 13690-05 from the U.S. Public Health
Service and in part by a grant from the American
Heart Association.
Introduction:
Considerable evidence has accumulated concerning
the relationship between streptococcai antigens
crossreactive with various human tissues and the
genesis of rheumatic fever. Cardiac antigens
have particularly been demonstrated to show
antigenic relationship with group A streptococcal
components (2,5,6).
One of the puzzling clinical features associated
with recurrent rheumatic activity is the occur-
rence of Sydenham's chorea (3,4). We have
studied patients seen at the Free Rheumatic and
Heart Center in Giza, Egypt: A considerable
proportion of these children present with recur-
rent attacks of chorea without concurrent signs
of active carditis. The remainder of the children
with rheumatic fever seen at Giza present with
bouts of transient migratory polyarthritis often
followed by carditis. It seemed that either
recurrent chorea or polyarthritis and carditis
constituted two clinically distinct forms of acute
rheumatic activity in this population, and once
the pattern was established in a given - either
chorea or arthritis and carditis, it did not vary
or change from one to the other.
The present study examines the possibility that
chorea might be related to antibodies to strepto-
coccal antigens somehow cross-reactive with
brain structures involved in the genesis of the
chorea syndroma.
Materials and methods:
Eighty children with acute rheumatic fever were
studied at Giza, Egypt during September, 1975,
at the beginning of the rheumatic fever season,
and all children were examined and tested during
the first four weeks of acute rheumatic activity
(Table I). Thirty children, ages 6-14 had rheuma-
tic chorea, and in many of these several attacks
of chorea had occurred ranging from a few weeks
to 6 months. Fifty children, ages 5-13, with
acute rheumatic fever, active carditis and transi-
ent arthritis were also included in the study
(Table I).
ANTIBODIES REACTING WITH
CYTOPLASM OF SUBTHALAMIC
AND CAUDATE NUCLEI NEURONS
IN CHOREA AND ACUTE
RHEUMATIC FEVER
A direct approach was made to search for
circulating antibodies reactive with components
of the central nervous system known to be
involved in Sydenham's chorea, which include
the subthalamic and caudate nuclei. Accordingly,
fresh human brain was obtained from a 45 year
old previously healthy male within 4 hours of
death in a traffic accident. Specimens from
subthalamic and caudate nuclei, cerebral cortex
and medullary nuclei were dissected from the
brain. Unfixed frozen sections from the speci-
mens were used as substrates for antigens in an
indirect immunofluorescence assay (1). Briefly,
the sections were incubated with undiluted heat
inactivated test and control sera, washed, and
thereafter overlaid with fluoresceinated antibodies
to human immunoglóbulins and finally examined
by immunofluorescence microscopy. Various
titration and absorption experiments were also
carried out. For control human and mouse
liver served as antigens for antinuclear anti-
bodies.
Results and discussion:
The indirect immunofluorescence staining
reactions using the tissue antigens described are
summarized in Table I. Fourteen of the 30 sera
(47%) from children with chorea showed concordant
staining of neurons in both caudate and subtahlamic
nuclei. By contrast, only 7 out of 50 sera (145
from the children with carditis showed the same
staining. Both the strength of fluorescence and
the titers of positive sera from the chorea
patients were also generally higher than those of
the carditis patients. None out of 10 otherwise
healthy Egyptian children hospitalized for ortho-
pedic surgery or 10 normal adult Egyptian indi-
viduals showed staining. The incidence of similar
staining in a total of 55 normal controls was 2%,
and it was 3% in 148 additional miscellaneous
hospitalized controls including patients with SLE,
multiple scierosis, rheumatoid arthritis, post-
streptococcal glomerulonephritis, and bacterial
infections (Table I). The use of fluoresceinated
antisera specific for the heavy chain of the various
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