NOTES, CASES, INSTRUMENTS "MOON EYE" Gyrate atrophy of the choroid and retina DONALD J. LYLE CINCINNATI
A boy, twelve years of age, was brought for eye examination by a county nurse from an old country com munity of Kentucky with the complaint of "moon eye". It appears that the boy, who is able to see well enough to play and does not complain of inability to see at his school work during the day, is totally blind at night. This condition was first noticed when he began to play out of doors in the evenings at about the fourth year of age. A bright spot of light aggravates the poor vision. If the light is "not too bright" he can approach it with ease. He can not walk with a lan tern at night without stumbling and "feeling his way". On going from a light to a dark room in the day time he must "follow the walls with his hands". Family history reveals an invalid father, cause unknown: a healthy mother who is "moon eyed" and color blind: and five other children. The youngest sister, the only other child ex amined, has an eye condition similar to,
though not as marked as the boy here reported. Four of the six children, in cluding the two seen, have webbed toes. Upon inquiry it was found that the pa ternal grandparents were first cousins. The intermarriage of relatives is com mon in this isolated community. Past history, which was obtained with difficulty, is probably inaccurate. The main facts are: At age two, whoop ing cough; at age four, night blindness noted; at age five, measles; at age six, chickenpox; at age seven, struck on head, unconscious and in convulsions fifteen hours; at age eight, paralytic stroke in right arm "after eating green grapes". General examination gave negative findings for the most part. The chief pathologic items were: Second and third toes of both feet were "webbed" together; slight mental retardation. Neurological examination showed underactive deep reflexes with no other positive findings. The blood Wassermann was negative. Eye examination showed a slight drooping of the lids, or possibly a nar rowing of the palpebral fissure to afford better vision. The right eye turned slightly upward toward the right, the left eye downward toward the left.
Fig. 1 (Lyle). Fundi showing retinal and choroidal atrophy. 1165
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NOTES, CASES AND INSTRUMENTS
There seemed to be some extrinsic muscle incoordination in all directions. The conjunctivae were neither injected nor congested. Media were clear. The pupils were equal and reacted normally to all reflexes. A refractive error (my opia) was noted. The fundi showed a peculiar condi tion about equally marked in both eyes. The optic nerves were asymetrically oval with distinct margins, possibly slightly pale. There was no exudate over
Fig. 2 (Lyle). Fields taken with one de gree white test object. One-half degree white test object could not be seen. the papilla nor atrophic cupping. The retinal vessels were of normal appear ance. The entire retina appeared to be atrophic permitting a clear view of the choroidal vessels throughout. The choroid also seemed to be atrophic with general evidence of the scleral background. In patches nearly com pletely encircling the peripheral field, closer to the disc on the nasal sides, were seen separate or clustered oval shaped areas of various sizes of total ab sence of both retina and choroid. The margins of these areas were regular and quite distinct as though punched out. Several of these patches contained clumps of pigment. The vessels, both retinal and choroid, passed over these areas in their normal course. This is the picture described by Ernst Fuchs in 1895 as gyrate atrophy of the choroid and retina. The bibliography of this rare condition has been reviewed and presented, with the first case re ported in this country by Dr. Hunter H. McGuire at the meeting of the
American Medical Association at New Orleans in May, 1932. There was present a compound myop ic astigmatism in both eyes: uncorrect ed in the right eye 5/200, corrected to 20/100: uncorrected in the left eye 10/200, corrected to 20/50. Visual fields show only presence of central and paracentral fields with one degree white spotter and entire loss of vision to one half degree white spotter. Intraocular tension was normal. The mother although complaining of night-blindness and color-blindness showed normal fundi and fields, with normal vision when a slight hyperopic error was corrected. This condition of the eyes, namely gyrate atrophy of the choroid and retina, appears to be influenced by or associ ated with consanguinity. The main points in this case are: 1. History of blood relationship. 2. Webbed toes a common occurrence in relational intermarriage. 3. Atrophy of retina and choroid with night blindness. 909 Carezv tower. MENINGITIS WITH OCULAR COMPLICATIONS HEDWIG S. K U I I N , M.D. HAMMOND, INDIANA
The case reported here, outside of its interest as a pathological process, is a vivid illustration of the importance of medical ophthalmology. Having been ill for many months under close observa tion but without any attention paid to the fundus picture, the child now brought belatedly for the simple obser vations that might have resulted more happily if made early. Albert, aged eight years, came to our office July 17, 1931, complaining of fail ing vision. Nine months previously he had what was called "intestinal flu." He had been very ill with fever, headaches, and vomiting. He improved and re turned to school for a while, but could not continue. His headaches persisted, associated with occasional spontaneous vomiting. Examination showed a very alert, intelligent little chap with a