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Of Rhythm, Is Very Amiable, Has a Good Memory, and Is 2 Usually Contented Dr. Gordon . New, Rochester, Minn. : I was much inter¬ probably exists everywhere; and no white race is ested in the case Dr. Beck reported, but, as the group I am exempt." were all it was not included. reporting malignant cases, The case described here illustrates that mongolian Regarding the retromaxillary group, I have seen several cases idiocy occurs in the race, and the with secondary involvement of the nasopharynx from retro¬ Mongolian Mongolian features of mongolian idiocy are not masked by the maxillary malignancy. I believe these are entirely different features from those I mention in The I am Mongolian of the Mongolian race. Indeed, my paper. group reporting the are primarily nasopharyngeal tumors and are usually in Rosen- family of the affected boy testified that "his eyes müller's fossa. I did not mention treatment because I felt are more slitlike than those of the other children." that if I did I would get away from the question of diagnosis. CASE We have seen many of these patients who were treated with REPORT OF radium continue well for three or four years, and feel that it From the mother it was learned that the child was born is the only treatment that offers relief. When surgery has at term. No instruments were used, and there was no been attempted, most patients have been made worse. asphyxia. The child was breast fed. The first tooth appeared at 11 months. The child crawled at 13 months, sat up at 14 months, and walked at 2 years. The other children of the family walked at 1 year. The first word was at 14 MONGOLIAN IDIOCY IN A CHINESE spoken months, and distinct phrases were used at 4 years. His BOY speech was never so distinct nor so fluent as that of the other children. It was always guttural in tone. Nocturnal enuresis I. HARRISON TUMPEER, S.M., M.D. stopped at S or 6 years, but a tendency remained. Later, it Professor and of Associate Head the Department of Pediatrics, Post was learned that the child was delivered by Dr. Effie Graduate Hospital and Medical School; Adjunct in Pediatrics, Lo'bdell, who the information that the labor was Michael Reese Hospital gave normal. At 6 months, she recognized the condition and prescribed thyroid CHICAGO extract combined with pituitary extract in 2-grain (0.13 gm.) three times The term is a condi- doses, daily. This was continued at varying "mongolian idiocy" applied to intervals tion certain children of retarded devel- for three years. Dr. Lobdell had noted the short, presented by thick fingers and the constant The mental and drooling. child was opment manifesting peculiar physical taught to walk in a chair and was found to be very imitative. phenomena, the most remarkable of which is the resem- blance to the Mongolian facies. The mongolian idiot resembles the Mongolian in the oblique palpebral fis- sure, epicanthal fold, widely placed eyes, flat-bridged, snub nose and expanded ali nasi. Brachycephaly is present in both; but the flattening of the occiput is characteristic of the idiot. The mongolian idiot differs physically from the Mongolian in the squat hand with tapering fingers, short thumbs and short, incurvated little fingers, the second phalanx of which is shorter than the terminal. His hair is not usually black, although it may be straight and is often wiry. The face is broad, but it is moon shaped and has no promi- nent cheek bones. He is small in stature and has broad, flat feet. There is marked hypermobility of the joints. There are chronic infections of the mucous membrane, with affections of the lids, lips', nose and throat. The tongue is furrowed and fissured from con¬ stant motion. It is described as "scrotal" by the French writers. There is no blue spot. The voice is guttural. Changes in the sella turcica have recently been described by Timme.1 The mongolian idiot differs mentally from the Mongolian in his retarded develop¬ ment, marked restlessness and activity, mimicry and indifference to his surroundings. In addition, the idiot manifests a fondness for music, exhibits a good sense of rhythm, is very amiable, has a good memory, and is 2 usually contented. Sutherland happily states that the Fig. 1.—Appearance of patient. "smiling face of the mongolian imbecile suggests the possession of a secret source of joy." The mother stated that he had never had an acute illness, From the time of Langdon Down,3 who first although the drooling and purulent nasal discharge were described in until the practically constant. mongolian idiocy 1866, present, The died cases have race. paternal grandparents in China at 71 and 74 the reported belonged to the Caucasian of The In 1903, Muir 4 concluded from his that years age. maternal grandparents were living in investigations China. The family was Cantonese. The father was 66 years was a this peculiar form of retardation matter of the of age and the mother 38. They were not related to each Caucasian race and summarized thus : "Mongolism other. All the children were born in the United States. There were nine pregnancies. There are no other retarded From the of the Post and Medi- Pediatric Clinic Graduate Hospital children in the immediate or remote cal School. family, and there are no cases 1. Timme, Walter: The Mongolian Idiot: A Preliminary Note on the of supernumerary digits, such as are sometimes Sella Turcica Finding, Arch. Neurol. & Psychiat. 5:568 (May) 1921. found. 2. Sutherland: Lancet 1:23, 1900. In to a as to 3. Down, Langdon: London Hosp. Rep., 1866, p. 259. response question whether they had noted 4. Muir: Arch. Pediat. 1903. 20:161 (March) anything peculiar about the boy, his mother and a cousin, a Downloaded From: http://jama.jamanetwork.com/ by a Johns Hopkins University User on 06/09/2015 student at the university, volunteered that his eyes were more flattened, and there was a shadow under the anterior clinoid slitlike than those of the other children, his tongue was process (Fig. 3). There was some separation of the sutures larger, and his head was flatter; the other Chinese children in the vertex of the skull, and there were digital impressions, of the neighborhood as well as his own brothers and sisters particularly in the frontal region. knew that he was different. His mother also stated that The mental age of the child was 3 years and 4 months by he was always happy, gave her no trouble, and did not even the Stanford revision of the Binet-Simon scale. care if his playthings were taken from him. Examination revealed a moon-faced boy, who looked as CONCLUSIONS if his face had been pushed in (Fig. 1). His mouth hung 1. This Mongolian child was a mongolian idiot in the generic sense of the word, as proved by the typical findings of the oblique palpebrai fissture, epicanthal fold, flattened bridge, widely placed eyes, "pushed in" face, flattened occiput, scrotal tongue, guttural voice, squat hands, Telford Smith5 finger, stub thumbs, hypotonia, excavation of the sella turcica and other less characteristic findings, such as chronic nasopharyngeal infection, undescended testicle and inward bowing of the phalanges of the great toe. There was further proof in the characteristic mental phenomena of restless¬ ness, amiability, mimicry, good memory and mental retardation. 2. The most recent finding in mongolian idiocy is here confirmed. In twenty-three of his twenty-four cases, Timme found an excavation under the anterior clinoid process and presumably under the olivary process and the optic groove. It communicated with the anterior portion of the fossa itself. He suggests that this change may explain the stature and lack of sexual development. 3. case Fig. 2.—Appearance of hands: incurvated little finger, shortening of This speaks against the theory of exhaustion second phalanx of little finger, and normal centers of ossification. of the generative organs as the etiology of mongolian idiocy, since there were normal children born before open, and a mucopurulent discharge issued from his nostrils. and after the patient. If the idea of exhaustion be The lobules of the ear were adherent, and the bridge of the even remotely entertained in this instance, it must be were and there nose was flattened. The eyes widely spaced, ascribed to the paternal element and not the usually was a marked epicanthal fold. The teeth were irregular, and condemned maternal element, since the father was 57 were decayed. The pupils reacted to light and accom¬ many and the mother 29 at the birth of the modation, and were equal. The tongue was of the typical boy. "scrotal" variety. The tonsils were large and irregular, and the throat was congested. There were signs of blepharitis. The chest and abdomen were not remarkable. There were no signs of the congenital heart lesions which are often described. The prepuce was long, and the left testicle was not in the scrotum. The reflexes were all active. There was marked hypotonia, with hypermobility of the joints, so that the most bizarre attitudes were adopted with ease. He assumed the uterine position on lying down. The hands were squat ; the fingers tapered ; the thumbs were stubby ; the little fingers were incurvated and did not reach the terminal phalangeal joints of the adjacent fingers. There was general adenopathy. The scapulae were moderately scaphoid. There was no blue spot. The weight was 46j4 pounds (21 kg.); height, 122.5 cm.; sitting height, 68.3 cm.; occipitofrontal circumferences, 46.6 cm. ; the anteroposterior diameter, 1S.4 cm. ; biparietal diam¬ eter, 14 cm.; the arm span, 116.5 cm.; the interacromial diam¬ eter, 26 cm.
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