Megalo-Cephalie, or Leontiasis Ossea. 
BY 
M. ALLEN STARR, M.D., Ph.D., 
PROFESSOR OF NERVOUS DISEASES IN THE COLLEGE OF PHYSICIANS AND SURGEONS 
NEW YORK; CONSULTING PHYSICIAN TO THE PRESBYTERIAN AND ORTHOPEDIC 
HOSPITALS, AND TO THE NEW YORK EYE AND EAR INFIRMARY. 
FROM 
THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES, 
December, 1894. Extracted from The American Journal of the Medical Sciences, December, 1894. 
MEGALO-CEPHALIE, OR LEONTIAS1S OSSEA. 
By M. Allen Starr, M.D., Ph.D., 
PROFESSOR OF NERVOUS DISEASES IN THE COLLEGE OF PHYSICIANS AND SURGEONS, NEW YORK ; 
CONSULTING PHYSICIAN TO THE PRESBYTERIAN AND ORTHOPEDIC HOSPITALS, 
AND TO THE NEW YORK EYE AND EAR INFIRMARY. 
Within the past few years attention has been drawn to various disturb- 
ances of nutrition occurring in the human body, either limited to certain 
parts or affecting the entire organism. We are at present familiar with 
changes in the growth and size of single parts or segments of the limbs, 
such as are seen in the various forms of atrophy and hypertrophy of 
muscles or of the skin, and also with changes in the appearance and size of 
the hands and feet, such as appear in acromegaly. Disturbances of nutri- 
tion of a more diffuse kind, attended by changes in the size and appear- 
ance of many tissues, are known to occur in myxoedema; and in fact, it 
is the study of this latter disease that has thrown much light upon the 
nature and origin of all disturbances of growth and nutrition. The fact 
that the secretion of the thyroid gland, when absorbed into blood, has a 
direct influence upon the nutrition of the hair, the skin, the subcutaneous 
tissues, the muscles, and many of the internal organs, cannot be denied. 
And there are many facts which show that an excess of thyroid activity 
is just as detrimental to the human organism as an arrest of its func- 
tions, for it seems quite apparent that in certain of the cases of Graves’ 
disease some of the symptoms of malnutrition are to be ascribed to an 
increase in thyroid activity. The facts ascertained regarding myxoedema 
and its dependence upon the thyroid gland lend a certain amount of 
probability to the statement that acromegaly is dependent upon disease 
of the pituitary body, although this is not fully proven. We do know 
that the progressive growth in size in the hands, feet, and bones of the 
face, is often found associated with a hypertrophy of the pituitary body. 
Again, it is probable that all the symptoms of Addison’s disease are 
definitely to be assigned to disease of the adrenal bodies. We are war- 
ranted in the surmise, in view of these facts, that there are certain dis- 
eases of nutrition which are dependent upon lesions in various glandular 
organs, whose function has been hitherto obscure, which deserve further 
study. 
There has lately come under my observation, in association with Dr. 
G. K. Dickinson, of Jersey City, a case of a peculiar character, which, so 
far as I know, has not as yet been described, of a trophic disease quite 
analogous to acromegaly, the chief symptom of which is a gradual or 2 
STARR: MEGALO-CEPHALIE. 
progressive enlargement of the head and neck, and which I venture to 
name megalo-cephalie. 
Fig l. 
condition in 1889, before enlargement of head and neck began. 
The patient is a woman, aged fifty-two years, American by birth, and 
of healthy parentage, her mother being still alive at the age of seventy- 
Fig. 2. 
Present condition, 1894, showing uniform enlargement of the head. 
eight. She has one brother and two sisters living and healthy; the 
family history is free from all evidence of cachexia or neurotic habit; STARR: MEG ALO-CEPH AL1E. 
3 
she has been married since the age of twenty-one, but has had no children; 
she has been in excellent health until the beginning of this disease—six 
years ago. The first symptoms noticed were formication felt in the tips 
of the fingers, particularly of the right hand, which gradually extended 
upward to the shoulder; this numbness was associated with a slight 
clumsiness of movement and uncertainty in tactile perception, although 
there has never been and is not now any actual anaesthesia, either of 
touch, temperature, or of pain, and the muscular sense is normal. She 
has never felt any of this numbness in the legs or body, but she has 
noticed for the past five years a disturbance in the gait, consisting of an 
uncertainty in movement and a subjective feeling of weakness. During 
all this time there has been a slowly progressive enlargement of the head 
and neck, and at the same time a gradual diminution in stature. Five 
years ago she was five feet seven inches in height, now she is five feet 
Fig. 3. 
Present condition, 1894, showing enlargement of the neck and head. 
two; this shortening is partly due to a stooping posture resembling that 
assumed in paralysis agitans, partly due to an apparent sinking of the 
head and neck between the shoulders, which causes a very peculiar 
appearance. 
The striking feature of this disease and its chief characteristic, as 
shown in our patient, for whose history I am indebted to Dr. Dickinson, 
is a slowly progressing increase in the size of the head, face, and neck, 
both the hard and soft tissues being apparently affected. The skin is 
diffusely thickened to a slight extent, is thrown into folds and not merely 
stretched over the enlarged bones, as it would be were it not involved 
in the process; and while it is not rough or scaly, is slightly pigmented 
and has something the appearance of the skin in myxoedema. The sub- 4 
STARR: M E G A Ij O —C EPHALIE. 
cutaneous tissue is also diffusely thickened, so that it throws the skin out 
and forms a loose body beneath it. The bone appears to be the portion 
primarily and chiefly affected, just as in acromegaly. There is appar- 
ently a general hypertrophy of the bone over the entire calvarium, 
more marked just behind the temples. This is not limited to definite 
small areas, but is quite uniform everywhere, so that the skull is enlarged 
uniformly. The head measures twenty-four inches in circumference 
about the forehead, fifteen inches from ear to ear over the vertex, and 
sixteen inches from the root of the nose to the occipital protuberance. 
Even the vertebrae of the cervical region appear to take part in the 
enlargement, and as a result the head is less movable upon the spine 
than it should be, and the spines of the vertebrae from the fourth to the 
seventh are decidedly prominent in the neck. A feeling of weight in 
the head i3 quite intense and had led to the hair being cut short, and it 
was noticed that the hair had become quite thin, especially at the sides, 
when formerly it had been thick and heavy. This diffuse growth of the 
head and neck is quite well shown in the photographs, which are all the 
more instructive, as the contrast between the woman in her ordinary 
state of previous health five years ago and in her present condition is 
shown. The thickening of the facial tissues tends to decrease the natural 
mobility of the face, so that the facial expression is more fixed than in 
a state of health. The thickening seems to have encroached upon the 
cavities of the nose and mouth, there being some obstruction to breathing 
and swallowing without thickening of the tongue. There is a hard res- 
piratory sound in the trachea constantly in breathing, and this has been 
present for a year past. 
The general symptoms which this patient suffers from are gradually 
increasing mental irritability and general nervousness, without any 
mental or emotional defect; an increasing disability to walk and to use 
her hands, attended by much numbness, especially in the hands, an 
occasional pain in the neck, arms, and hands; some awkwardness of 
movement in the fingers, but no marked ataxia, for she can knit, can 
thread a needle and do fancy work. There is no anmsthesia present, 
and there is no loss of pain or thermal sense. The act of walking is 
performed with difficulty and is now only possible with the aid of hold- 
ing a chair, though there is no ataxia of movement and no spastic 
rigidity of the legs; the knee-jerks are slightly increased, but there is no 
clonus; the control of the sphincters is perfect. In walking she has a 
general sense of insecurity and feebleness, but there is no evidence of 
paralysis. She stoops forward much like a patient suffering from paral- 
ysis agitans, but has no tendency to fall or to festination. She is able 
to walk up and down stairs with help. Her neck is sixteen inches in 
circumference—at least four inches larger than formerly. There are 
two soft non-fluctuating swellings below the ears having the feel of dif- STARR: MEGALO —CEPHALIE. 
5 
fuse, fatty infiltrations, and the neck is uniformly increased in size. 
There is no apparent swelling of the thyroid gland. The cervical ver- 
tebrae feel thick and are unduly prominent, but are not tender. She has 
had considerable pain along the course of the occipital nerves; in other 
respects her general health is very good, the digestive and urinary func- 
tions being perfect. The heart is regular, is not enlarged, and the pulse 
tension is normal. There are no cardiac murmurs, but a blowing mur- 
mur is heard over the arch of the aorta synchronous with the first 
sound. There is no area of dulness, and there are no other symp- 
toms to suggest aneurism. No cause is to be found for this condition, 
which has advanced so gradually that its actual date of onset is diffi- 
cult to determine, though she is certain that it began more than five 
years ago. 
The very extraordinary appearance of the head in this patient sug- 
gested to me that she might be suffering from a condition known as 
leontiasis; but a careful study of the literature regarding leontiasis 
convinced me that under this name a number of entirely different 
conditions have been grouped. 
The term leontiasis has been applied to a form of leprosy affecting 
the face, and very deary described by Leloir in his monograph upon 
lepra, where several plates illustrating the appearance of the face in 
this disease are to be found. In this condition there occurs a deposit 
of new material here and there upon the face beneath the skin, and 
these deposits gradually enlarge and coalesce until the entire face is 
changed into a hard mass with deep seams and roughened surface, the 
features being almost obliterated and the eyes sometimes closed. The 
bones of the face are not usually involved, and the disease does not 
extend upward beyond the line of the hair, and does not produce any 
enlargement of the cranial bones. The term leontiasis has been applied 
to this because the rough, seamed, and thickened appearance about the 
mouth suggests the idea of the jaw of a lion. It is said that the term 
has been in use since the time of Galen. 
There is a second condition to which the term leontiasis has been 
applied—namely, a condition of elephantiasis affecting the tissues of 
the head and neck. This is mentioned by Virchow in his work upon 
tumors. He describes it as a diffuse thickening of the soft parts of the 
head and face, consisting of an increase in fibrous tissue, and considers it 
a form of fibroma molluscum. This form corresponds in its history to 
elephantiasis; it begins quite suddenly with an acute condition, which 
may be either erysipelas or dermatitis or phlebitis; the skin becomes 
swollen and red simultaneously, and there is often an eruption of vesi- 
cles after the acute symptoms subside, but the swelling does not dis- 
appear entirely, and the skin pits upon pressure. Later, there is a 
recurrence of the acute condition and an increased swelling; and so the 6 
STARR: MEGALO-CEPHALIE . 
disease goes on with acute exacerbations and remissions; ulceration of 
the surface and very deep pigmentation are commonly observed in the 
parts affected. After describing this condition and recording a clinical 
case described by Gruber, Virchow says: “ I do not wish to affirm that 
there is not a form of leprosy which calls to mind the appearance of 
leontiasis; on the contrary, I have often seen such a form of leprosy in 
Norway, but the greatest deformity occurring ordinarily without ulcera- 
tion is the peculiar characteristic of fibroma molluscum, or elephantiasis 
molluscum.”1 
I think it is quite evident that the disease from which our patient is 
suffering does not in any way correspond to either of these two diseases 
to which the term leontiasis had been applied. 
There is a third disease to which Virchow gives the name bony 
leontiasis (leontiasis ossea), which, however, resembles megalo cephalie 
more closely. In his description of tumors of a bony nature, after de- 
scribing exostosis and periosteal thickenings, Virchow devotes a section 
to hyperostosis of the face and head. This may take two forms: first, 
a diffuse hyperostosis of the cranium ; secondly, localized hyperostosis 
upon the maxillary bones. This latter condition we may dismiss with- 
out further consideration, inasmuch as it presents no points of a resem- 
blance to our own case. It has been studied carefully by Le Pentu in 
La Revue mensuelle de Mededne et de Chirurgie, 1879. The diffuse 
hyperostosis of the cranium is, according to Virchow, symmetrical and 
uniform, so that it does not present any bony excrescences and suggests 
the idea of tumor. Virchow’s description is based upon the examination 
of certain skulls, which are preserved in various museums, the first 
one having been described by Malpighi, 1697. There are about six 
of these skulls known in Europe, all of them presenting tremendous 
enlargement of the bones of the head of a diffuse character. Virchow 
says: “ This condition is for the skeleton what leontiasis is for the 
soft parts; the appearance of these pathological specimens, seen a 
certain distance, produces the effect of leontiasis modelled in plaster. 
Hence, I have the less hesitation in giving the name of bony leontiasis, 
since, as I believe, the disease corresponds exactly with that which we 
call elephantiasis in the soft parts. Ordinarily, the hyperostosis is 
associated with a fibrous change in the soft parts; in the rare cases 
which we have seen, it is the periosteum which is the principal seat 
of the disease. Unfortunately, we have no records of clinical observa- 
tions, and no knowledge of the exact conditions present by recent 
autopsy.”2 
In a monograph upon leontiasis ossea,3 Baumgarten has made a 
1 Virchow: Pathol, des Tumeurs, vol. i. p. 324. 
2 Virchow : loc. cit., vol. ii. p. 22. 
3 La Leontiasis Ossea, par Dr. F. Baumgarten, Paris. Steinheil, 1892. STARR: MEGALO-CEPHALIE. 
7 
careful study of this condition described by Virchow, and has collected 
the descriptions of all these skulls in the various museums of Europe. 
He has shown that some of these skulls present a uniform thickening 
of all the bones, while others have shown an enlargement limited to the 
bones of the face, the cranium escaping. He points out the fact that 
the latter class of skulls probably belonged to patients suffering from 
the disease acromegalia, in which, as is well known, the bones of the face 
are very markedly enlarged. It is evident from Baumgarten’s study 
that the term leontiasis ossea must be limited to the cases in which the 
hyperostosis of bone has affected the bones of the cranium only, or 
these, together with the bones of the face. He considers that the 
pathological chauges present are either a thickening of the surface of 
the bone alone, or a change both in the surface and the diploe; the 
latter being in some cases transformed into a spongy tissue; in others, 
into a hard tissue like ivory. He admits that the literature does not 
contain any description of this disease observed during life, and he does 
not think that cases have been observed. 
It is my opinion that the case here described is this disease, and that 
we can affirm that in these cases there is not only a hypertrophy of the 
bony tissue, but also of the soft parts about it. It is probable that the 
diffuse thickening of the bones of the head and neck causes some pressure 
upon the contents of the skull and upon the nerves at their exits between 
the vertebrae. This might explain the symptoms which are present in 
our patient—especially the complaints of numbness and the difficulty in 
her gait. 
Various forms of treatment have been pursued in the case under con- 
sideration. A thorough trial of thyroid extract failed to affect the 
condition in any way, and did not cause any variation in temperature 
from normal. The use of arsenic and strychnine, given in moderate 
doses, appears to have had some tonic effect, and these, together with 
the moderate use of corrosive sublimate internally, are the remedies 
relied upon. 
I have ventured to give the condition the name megalo-cephalie, 
because, as already shown, the term leontiasis has been applied to three 
entirely distinct conditions, and therefore leads to confusion. 
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