Evening star., 1940-04-06

Disease That Gives
Girls WrinkIed
Faces Described
6. W. Doctor Tells
Pediutriciuns OF Rare
Broin MoIody
Bq THOMAS r. HENRY.

Bq THOMAS r. HENRY.
A weird brain disease which gives
girls "the features of one of the
witches In Macbeth and the hips of
a madonna by Reubens" was de-
scribed before the American Acad-
emy of Pediatrics meeting here
today.
It is very rare. Less than 100
cases have been reported in the
world in more than half a century
since the first case was described
in medical literature.
Starting with the face, fat COM
.pletely disappears from the upper
half Of the body. It is likely to
appear ebnormally on the lower
half. The malady may begin any
time from babyhood to middle age,
but is most likely to come in ado
lescense. 1t is twice as frequent in
girls as in boys.
The fat even disappears, DR.. p.
A. McLendon of George Washing
ton University told the child spe-
cialistS, in the so-called "buccal
pads" on the cheeks where it other
wise persists even in cases of ex-
treme starvation. The muscles re-
main just as strong and the skin as
clear as ever, except that 1t may
become hideously wrinkled.
Disturbance Traced to Brain.
Hitherto this malady, known as
lypodystrophy, has supposedly been
due to some deficiency in glandular
secretions, but no specific lack has
been found Various physicians
have blamed all the glands in turn,
on slender evidence.
It now is becoming clear, DR.. Nc
Lendon said, that it is due to dis
turbance of some fat-controlling
center in the brain, located approxi-
mately in the region of the third
ventricle. Irregularities in glandu-
lar secretions may be caused by this
and in turn give rise to the peculiar
overt symptoms.
The disease, DR.. McLendon said,
is peculiarly apt to follow diseases
like measles and influenza. In this
way it is like the dreaded enceph-
alitis. or sleeping sickness, where
disturbances also are shown in the
third ventricle. Here. Psychologists
now believe, is located the human
sleep center" In some way infec-
tious diseases appear to leave this
region of the brain peculiarly sus-
ceptible to infections. Apparently
the sleep center is less protected
than the hypothetical fat center
which has not been localized and
probably constitutes a very small
portion of the brain tissue. The
pituitary gland in the base of the
brain, which is responsible for the
abnormal fatness of circus freaks,
does not appear to be affected.
Patients Eventually Recover.
At present, it was stressed, there
is little to De done for the condition
and patients eventually may recover
through the natural recuperative
powers OF the body. Except for the
freakish appearance, they may be
none the worse of. In one case re-
ported by DR.. McLendon the little
girl is now doing well in school.
Some OF the reports, he said, are
that victims have acted queer, sug-
costing the possible involvement OF
higher brain centers. No evidence
Ct this has been found, he said, and
Bychiatric symptoms might be ex-
pected from person distinguished
from her fellows by such a striking
physical appearance.
The progress of the strange NAL
ady is SO insidious, DR.. McLendon
said, that usually couple of months
elapse before the child's parents be-
come suspicious that something is
wrong. BY that time the condition
is well established and is difficult
to trace back to its beginnings. The
hypothetical brain center apparent
ly controls the disposition, rather
than the formation of fat. In the
few cases that have died abnormal
fat deposition has been shown at
the base of the skull and autopsies
have shown an abnormal appear
ance of the third ventricle.
Defective Oxidation Shown.
Recent studies at Duke University
he said, have shown a normal alb
sorption of fats from the food, but
a defective oxidation, or burning
of this fat which is the fuel OF
the body furnace. There is also an
increase of fats in the blood serum
after meals which contain much
fatty material. On the other hand
he pointed out, there seems to be
no disturbance of the nervous sys-
tenn, as is the case with the after
math of encephalitis. All the deep
nerve ref1exes remain normal and
the patients are perfectly COM
Portable. They remain as intelli-
gent as ever, if they are left alone
SO tina. they do not develop mental
complexes.
Patients differ, DR.. McLendon
said, in the extent of the fat
stripping progress. In some cases it
does not 60 below the neck. In
others 1t extends to the chest, and
in extreme cases goes down to the
hips. The extent may depend on
the degree of involvement of the
fat distribution brain center. In a
few cases isolated patches of fat
on the upper half of the body
have been reported. The blood cir
culation does not seem disturbed
! in the least.