396 ROBERT C. LAUGHLIN

REFERENCES

I Groenouw, A. Beziehungen der Allgemeinieiden und Organerkrankungen zu Veriinderungenand Krankheiten des Sehorganes. Handbuch der Augenheilkunde. Ed. 3, Berlin, JuliusSpringer, 1920, chapter 22, pt. 1, p. 1095.

2 Weiss, L. Uber die Tuberkulose des Auges. Arch. f. Ophth., 1877, v. 23, pt. 4, p. 141, Case III.3 Brailey, W. A. A case of tubercle of the eye clinically resembling somewhat a retinal glioma.

Med. Times and Gazette, 1882, v. 2, p. 512.• Spalding, ]. A. Tuberculosis of the eye. Two cases: one at the head of the optic nerve, the

other at the equator. Trans. Amer. Ophth. Soc., 1903, v. 39, p. 141.s Knapp, A. A case of localized tuberculosis at the head of the optic nerve. Arch. of Ophth.,

1903, v, 32, p. 22.• Coats, G. A case of tubercle of the nerve head. Royal London Ophth. Hosp. Rep., 1906,

v, 16, p. 381.'Verderame, Ph. Anatomischer Beitrag zur Solitiirtuberkulose der Papilla nervi optici, Klin.

M. f. Augenh., 1908, v. 46, N.F. 5, p. 401.s Komoto,]. Ein Beitrag zur Solitiirtuberkulose des Sehnervenkopfes. Klin. M. f. Augenh.,

1912, v. 50, N.F. 13, p. 82.• Jacobs, M. W. Klinischer and mikroskopischer Beitrag zur Solit.irtubcrkulose der Papilla

nervi optici. Klin. M. f. Augenh., 1912, v. 50, N.F. 14, p. 37.

HEREDITARY PARALYSIS OF THE ABDUCENS NERVE*

REPORT OF A CASE

ROBERT C. LAUGHLIN, M.D.Baltimore, Maryland

The following case is reported for thereason that it represents the compara­tively rare picture of hereditary paralysisof the sixth nerve.

N. F., a white female, aged 4 years and3 months, was brought to the dispensaryof the Wilmer Ophthalmological Instituteby her mother froin whom the followingdata were obtained:

The child's left eye will not turn out.The patient's mother, maternal grand­

mother, and two maternal uncles havebeen unable to abduct the left eye sincebirth. The patient's only brother has nor­mal eyes. There is no history of other eyedisease, and no tuberculosis, cancer, norfamilial nervous disease of any sort.

The child's general health is good; noserious illness has intervened nor any sys­temic disease. Delivery at birth wasnormal.

*From the Wilmer Ophthalmological Clinicof the Johns Hopkins Hospital and University.

Since birth the child has been unable toturn the left eye outward. There is nohistory of pain nor inflammation aboutthe eyes; and no apparent visual dis­turbance.

Eye Examination: The child was alertand quite intelligent. Visual acuity couldnot be recorded accurately, but roughtests showed both eyes to be normal. Thelids, lacrimal apparatus, tension, and pu­pillary reactions were normal. The lefteye could not be rotated outward beyondthe midline (fig. lA) ; there was a slightoutward movement up and to the left.With the gaze directed straight aheadthere was no internal deviation of the lefteye (fig. 1 B). Adduction was normal(fig. 1 C) with no narrowing of the pal­pebral fissure nor retraction of the globe.Convergence could not be tested. Extra­ocular movements of the right eye werenormal throughout. The position of theeyes in the sockets was normal.

The grandmother and two maternal

HEREDITARY ABDUCENS-NERVE PARALYSIS 397

uncles were not available for examination,but the mother submitted herself to ex­amination and showed a typical Duane'ssyndrome as described by that writer in1905.' There was no abduction of the lefteye in the horizontal plane (fig. 2 A), butslight outward movement when the gazewas up and out. Adduction was poor andwas accompanied by a vertical deviationupward and marked narrowing of thepalpebral fissure (fig. 2 C). The left globewas retracted 3 mm. when adducted.There was a manifest left static enoph-

eleven families and thirty patients. Thesecases of hereditary abduction insuffi­ciency may be summarized as follows:Gifford," mother left, son bilateral; Har­Ian," mother left, daughter bilateral;Kraus," mother left, son right; Endel­man," mother and daughter both left; Va­rese," one case reported, mother said to beaffected same way; Gunsberg,' motherand son both left, of the son's daughters,one left, one bilateral; Best," case of leftside, maternal grandmother reported tohave same condition; Wardenburg," fa-

Fig. 1 (Laughlin). Patient, N. F. A, looking to the left; B, eyes in primary position; C, look­ing to the right.

Fig. 2 (Laughlin). Mother of patient shown in figure 1. A, looking to the left; B, eyes inprimary position; C, looking to the right.

thalmos which measured 3 mm. with theexophthalmometer. There was no internaldeviation of the left eye when the eyeswere in the primary position (fig. 2 B).The mother habitually kept the head ro­tated to the left about 15 degrees. Therewas occasional slight diplopia when thegaze was turned to the right, but neverwhen directed to the left.

Although congenital defects of ab­duction are of relatively frequent occur­rence there have been but few cases re­ported in which heredity was an evidentfactor. H. Gifford" (1926), in discussingcongenital abduction deficiency reportedone case of his own and seven othersfrom the literature in which there wasa familial tendency. Since then twocases have been recorded which with theone described here bring the total to

ther and three children in family of eleven,all left; Lodberg," sixteen-year-old girl bi­lateral, maternal aunt left; Zentmeyer,'"three instances of Duane's syndrome-inchild, mother, and grandmother; presentcase of five persons in three generations,two of whom were examined-patient,mother, maternal grandmother, and twomaternal uncles, all with left involve­ment. Wolff'" has also reported threesiblings all with left abduction insuffi­ciency and retraction movements. Theseare not included in this list, since they alloccurred in the same generation.

An analysis of these cases shows thehereditary factor is not sex linked, al­though there is definite preponderance offemales. Of the patients whose sex wasreported 75 percent were females and 25percent males. In those concerning whomthe eye was reported all but one of the

398 ROBERT C. LAUGHLIN

unilateral cases occurred on the left side,or 95.3 percent. These figures agree withother reported series of straight con­genital abduction deficiency in whichheredity was not. especially considered.All of these showed a majority of femalesand a majority of left eyes. In Gifford'sseries 60 percent of cases were in females,and 77 percent of the unilateral caseswere in left eyes. Including the presentcase there are only three instances inwhich the abnormality appeared in morethan two generations.

Gifford suggested birth injury as theetiological factor, but it does not seemlikely that this would explain the occur­rence in successive generations nor thepreponderance of afflicted females. The

subject of congenital abduction deficiencyhas already been discussed adequately byDuane, Gifford, Green'" and others.

An excellent bibliography of all heredi­tary diseases of the eye has been com­posed by Clausen," and the entire subjectis dealt with thoroughly by Waarden­burg."

SUMMARY

A case of hereditary paralysis of theabducens nerve is presented, in which fivemembers in three generations of the samefamily were afflicted; two were ex­amined. Ten other cases are reportedfrom the literature. A statistical analysisof the data shows that in this condition amajority of females and of left eyes areaffected.

BIBLIOGRA PIIY

1 Duane, A. Arch. of Ophth., 1905, v. 34, p. 133.2 Gifford, H. Amer, Jour. Ophth., 1926, v. 9, p. 3.• Harlan. Trans. Amer. Ophth. Soc., 1885, p. 48.• Kraus. Munch. Med. Woch., 1905, p, 1957.• Endelman. Abstr, in Zeit. f. Augenh., 1911, July, p. 75.• Varese. Arch. di. Ottal., v, 9, p. 143., Gunsberg. Klin. M. f. Augenh., 1889, v. 37, p. 263.8 Best. Deutsch. Med. Woch., 1903.• Waardenburg. Arner. Jour. Ophth., 1923, v. 6, p. 44.10 Lodberg, C. V. Verh. Ophth, Ges., 1933, p. 23.11 Zentrnayer, W. Trans. ColI. of Physicians of Philadelphia, 1917, v . 38, p. 355.12 Wolff, J. Arch. of Ophth., 1900. v. 29, p. 297."Green, J. Trans. Amer, Acad. Ophth, and Oto-Laryng., 1913, p. 358."Clausen, W. Zentr. f. d. ges, Ophth., 1925, v. 13, p. 173.15 Waardenburg, P. J. Das menschliche Auge und seine Erbanlagen. Haag, Martinus Nijhoff,

1932.