Society Transactions

PHILADELPHIA NEUROLOGICAL SOCIETY

Regular Stated Meeting, March 25, 1924

CHARLES W. BURR, M.D., President, in the Chair

AN UNUSUAL CASE OF AMYOTROPHIC LATERAL SCLEROSIS. DR. M. A. BURNS. The case is presented because of early development of symptoms with gradual return of function, although symptoms of anterior horn and lateral tract lesions are still present.

A youth, aged 19, a messenger, whose father and mother had died from tuberculosis, one sister having died in infancy, and who had one brother living and well, was a healthy boy until the age of 6, when, he says, he was struck by a heavy plank on the right shoulder. When between 6 and 8 years old, he was placed in a home for orphans and was told that his right arm and leg were not normal, and that he was unable to use his right hand to carry food to his mouth. After he had been in the home a short time a brace was applied to his right leg, which he wore for two years. He left the home when he was 13, and was able to walk fairly well without a brace, although he had little use of the right arm and hand.

Two years later, it was thought that the boy had tuberculosis, and he was sent to Mount Alto, remaining there for six months. There he received massage, but since that time he has received no treatment.

His chief complaint was partial paralysis of the right upper extremity and, to a less degree, of the right lower extremity, with marked muscular atrophy. He believed that in the last four years the arm and leg had improved.

Examination.-Station was good and gait was spastic on the right side. There was marked atrophy in the upper limbs, chiefly in the shoulder girdles, more marked on the right side. He had a tendency to a wrist drop on the right side and some vasomotor disturbance. No fibrillation could be demonstrated. Biceps and triceps reflexes were exaggerated on the right and slightly increased on the left. The grip on the right side was greatly diminished. Resistance to passive motion on the right side was poor, although it was fairly well preserved on the left.

There was marked atrophy of thigh and leg muscles on the right, especially in the gluteal region. The patellar reflex on the right was markedly increased and slightly overactive on the left. The Achilles reflex on the right was markedly increased; the Babinski sign was definite on the right, and very slight on the left. Ankle and patellar clonus were not obtained. All forms of sensation and the sphincters were normal.

Roentgen-Ray Findings.-One plate showed no evidence of an enlarged thymus. A second plate showed considerable cloudiness of the upper lobes of the lungs on both sides with a rather unusual type of peribronchial thickening. It is uncertain whether or not this was due to definite pulmonary pathology or to lack of inspiration.

Electrical Reactions.-The flexor muscles of the right upper extremity showed a normal response. The extensor muscles and muscles of the thumb showed a reaction of degeneration.

The serologic findings were negative, and the eye examination revealed nothing abnormal.

A CASE OF UNILATERAL ASCENDING DEGENERATION OF THE PYRAMIDAL TRACT.
DR. FRANCIS X. DERCUM.

A white, unmarried man, aged 39, was admitted to Jefferson Hospital, March 4, 1924, complaining of loss of power and stiffness in the right leg and right arm. The family history was unimportant. He had had gonorrhea about fifteen years before, and about the same time there was a local sore which healed promptly and which was not followed by any secondary manifestations.

The present condition began seventeen years before with slight weakness and stiffness in the right leg which gradually increased; later a sense of stiffness made its appearance in the right arm. This gradually became more pronounced. There was at no time pain or numbness.

Examination revealed a healthy-looking man with a gait suggestive of ordinary hemiplegia except that the spasticity in neither arm nor leg was as marked as that usually seen in typical hemiplegia following apoplexy. However, the arm was held automatically in a more or less semiflexed position, while the leg was held extended, and this was particularly noticeable in the gait. The face was not involved. The tendon reflexes in both the arm and leg were exaggerated. There was no Babinski sign, no wasting of muscles of the thenar or hypothenar eminence nor of the muscles of the shoulder girdle. There were no sensory losses. The right pupil measured 4.5 mm. and the left 5 mm. Both reacted promptly to light and convergence. The eyegrounds were normal. Serologically, blood and spinal fluid were normal.

This is the first case of the kind that I have ever observed. It is interesting to note that the onset of the condition antedated the local venereal lesion.

DISCUSSION

DR. N. W. WINKELMAN: In Dr. Burns' patient, are the bones smaller on that side? It looks as if the hand is smaller, aside from the flesh. Could not the case be a developmental condition such as Little's disease?

DR. CHARLES K. MILLS: I believe that this case (Dr. Dercum's) of unilateral degeneration of the pyramidal tract is a real type of disease, one which I described some years ago. I saw one or two cases and another with Dr. Spiller, which came to necropsy and in which microscopic examination by Dr. Spiller showed degeneration of the pyramidal tract.

This type of disease must be carefully diagnosticated both from unilateral ascending paralysis agitans and amyotrophic lateral sclerosis of the ascending type. In one of my papers, I discussed at length the form of ascending and descending paralysis agitans.

DR. FRANCIS X. DERCUM: I have never seen a case exactly like that of Dr. Burns'. There can be no doubt as to the structures involved, namely, the anterior cornua and the lateral tracts. Perhaps in the age and mode of onset lies the ultimate explanation.

I am satisfied that the case which I have just shown should be classified as one of unilateral ascending degeneration of the pyramidal tract; in other words, it is illustrative of the condition originally described by Dr. Mills.

DR. M. A. BURNS: I believe my case began as acute poliomyelitis in which the lateral tracts were evidently involved very early. some shortening of his bone, but it is not marked. disease do not show atrophy of anterior horn cells, boy has.

CASES OF ENDOCRINE ANOMALIES.

The patient may have Most cases of Little's which apparently this

DR. FRANCIS X. DERCUM.

CASE 1.-A white girl, aged 9, was admitted to the Jefferson Hospital because of mild Sydenham's chorea affecting mainly the left side.

The father had died at the age of 30 of meningitis. The mother was well and said that she had matured early and was adult in development at the time of puberty, which was established at 13. There was no history of mental, cardiorenal, pulmonary or malignant disease in the family.

The patient had had chickenpox and measles but no other childhood infections. She had been slow in learning to walk and talked plainly first at the age of 7. Very large tonsils and adenoids were removed at the age of 6. She was sent to school at that age, and made good progress. The mother said that the thyroid gland had for a long time been large, and that during the past year it had notably increased in size. The child had always been nervous and easily frightened.

At the age of 7, she began to menstruate, the periods recurring every twenty-eight days and lasting three, four and even seven days.

About a year before presentation, she was vaccinated, and two days later developed chorea, which improved greatly on rest in bed but did not entirely disappear.

The patient looked like a girl of 17. The thyroid gland was greatly enlarged and uniform. There was a marked mitral murmur; otherwise the visceral examination was of little importance. Gait, station and reflexes were normal. The mammary glands were well developed. The pubis and genitalia were adult in appearance.

There was no tachycardia or prominence of the eyeballs, and no von Graefe, Stelwag or Moebius sign. Pupils and eyegrounds were normal. The urine was normal. The blood contained 5,000,000 red cells and 7,400 white cells and 44 per cent. small mononuclears.

The child lay in bed playing with her doll and was easily pleased. Her talk and interests were childlike. Clearly there was no mental precocity.

The basal metabolism was plus 9.

A roentgenogram of the skull revealed the posterior clinoid processes slightly larger than usual; otherwise the sella turcica appeared to be normal. The pineal gland was calcified. There was no roentgen-ray evidence of an enlarged thymus. But it must be borne in mind that the roentgen-ray examination of the thymus is very unsatisfactory, and that unless the enlargement is such as to cause marked shadows projecting beyond the sternum it cannot be observed. In this case, the large percentage of small mononuclears must be taken into account. It is to be regarded as evidence of lymphatic persistence, which naturally suggests also some degree of thymic persistence, and in the present instance would be in keeping with the history of tonsillar enlargement and adenoids; in other words, with an undoubted degree of lymphatic hyperplasia. If this interpretation of the case be correct, the precocious sexual development must be regarded as an expression of biologic failure. The enlargement of the thyroid must be regarded as an effort at

compensation. It is not accompanied by the symptoms of hyperthyroidism. Clearly it must be looked on as an under-compensation, and to prove the correctness of this view, I should state that thyroid administration has thus far been followed by a notable diminution in the size of the gland. This result is further in keeping with previous observations of my own that thyroid enlargement in girls at or near puberty is the expression of an effort at compensation. Commonly such enlargements disappear on thyroid administration. If hyperthyroidism, the opposite condition, were present, such a result would assuredly not ensue.

CASE 2.-A Hungarian woman, aged 21, single, a cigar-maker, was admitted to the Jefferson Hospital, March 21, 1924. She presented herself first at the outpatient department suffering from an ordinary Bell's palsy on the right side. It was noted also that she had decided enlargement of the thyroid gland. The family history was negative except that one sister presented thyroid enlargement similar to that of the patient. The personal history was negative as regards childhood infections, with the exception of measles. Menstruation

appeared at 16, was painful and persisted for a year, after which it ceased for seven months. Since then it had been normal. The facial palsy had been present for one week. She said that she had not been well for four years.

She was rather thin and awkward, 5 feet 7 inches in height. The viscera were normal. The marked thyroid enlargement was symmetrical and soft. There was no bruit over it. No adenopathy was detected. Gait, station and reflexes were normal. The Wassermann tests of the blood and spinal fluid were negative. The urine showed a faint trace of albumin; otherwise it was normal. The differential blood count showed 27 per cent. small mononuclears. The eyegrounds, pupils and ocular muscles were normal, except that there was weakness of the right orbicularis incident to the Bell's palsy.

The patient said that the thyroid enlargement was at one time much more marked, that it became decidedly less during her voyage to this country, and that she suffered from enlarged lymphatic glands but that these improved under treatment.

The roentgen-ray examination revealed enlarged thymus extending not only into the costal interspaces, but also into the supraclavicular area and a pituitary fossa somewhat larger than the average (Figs. 1 and 2).

The enlargement of the thyroid gland I think must be regarded as compensatory. It is probable that there was also enlargement, moderate in degree, of the anterior lobe of the pituitary. This was suggested by the patient's height, and, if so, like the enlargement of the thyroid is to be interpreted as an effort at compensation. Both are to be regarded as under-compensations. There was no tachycardia, no exophthalmos, no von Graefe or other ocular