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Show Journal of Neuro- Ophthatmology 16( 3): 185- 187, 1996. © 1996 Lippincott- Raven Publishers, Philadelphia Neuro- Ophthalmologic Findings in the Asperger Disorder Michael C. Brodsky, M. D., Laurie G. Barber, M. D., Byron L. Lam, M. D., Lawrence M. Merin, F. I. M. I., and Stephen Edelson, Ph. D. Asperger disorder is a complex behavioral disorder that may be related to autism. We examined a 49- year- old man with Asperger disorder who had multiple neuro-ophthalmologic abnormalities, including colobomatous defects involving the optic discs and peripapillary retina, and abnormal ocular motility with an oculocephalic dyskinesia. Asperger disorder may be associated with a variety of neuro- ophthalmologic disturbances. Key Words: Asperger disorder- Coloboma. Asperger disorder, originally described in 1944, comprises a cluster of behavioral characteristics including concrete thinking, inappropriate reciprocal social interactions, pedantic speech, obsession with unusual and sometimes complex topics, and physical awkwardness or clumsiness ( 1,2). There is some controversy as to whether Asperger disorder forms a distinct category within the pervasive developmental disorders or falls within the continuum of high- functioning autism since the prevalence of Asperger disorder is much higher than that of autism ( 3). In contrast to autism, in Asperger disorder there are no delays in language or cognitive development ( 2). Asperger disorder is more common in males and among family members of individuals with the disorder, but no specific inheritance pattern has been established ( 2). We p r o v i d e the first d e s c r i p t i o n of n e u r o - ophthalmologic abnormalities in a patient with the Asperger disorder. CASE REPORT Manuscript received April 3, 1995; accepted July 10, 1995. From the University of Arkansas for Medical Sciences ( M. C. B., L. G. B., B. L. L., L. M. M.), Little Rock, Arkansas, and the Center for the Study of Autism ( S. E.), Beaverton, Oregon, U. S. A. Address correspondence and reprint requests to Dr. Michael C. Brodsky, Arkansas Children's Hospital, 800 Marshall, Little Rock, AR 72202, U. S. A. A 49- year- old man with the Asperger disorder was evaluated for decreased vision, abnormal ocular movements, and frequent head tilting to either side, which had been noted since childhood. The patient and four first- degree relatives fulfilled the diagnosis criteria for Asperger disorder as defined by the Diagnostic and Statistical Manual of Mental Disorders ( 2). Corrected visual acuity was 20/ 40 in the right eye and 20/ 50 in the left eye. Both pupils reacted briskly to light, and there was no afferent pupillary defect. The patient displayed an oculocephalic dyskine- 185 286 M. C. BRODSKY ET AL. sia consisting of periodic alternating torticollis as- DISCUSSION sociated with torsional eye movements. During casual conversation, he alternately tilted Electrophysiologic abnormalities have been re-his head to the right and left at approximately half- cently documented in autistic patients. Creel et al. minute intervals. He stated that he did not perceive ( 4) a n d R i t v o e t a l ( 5) f o u n d n o v i s i b l e r e t i n a l ab_ any visual benefit from the torticollis and often tried normalities in 22 patients with high- functioning to suppress it. He had 50 diopters of alternating es- autism, but they detected reduced b- wave ampli-otropia, mild bilateral abduction limitation, and a tudes following stimulation with scotopic dim blue large A- pattern. These findings were accompanied flashes i n 1 0 p a t i e n t S / suggesting that subnormal by bilateral overaction of the superior oblique and rod function m a y b e a n electrophysiologic marker superior rectus muscles, which produced alternating for hieh- functionine autism hyperdeviations on gaze to either side. The patient To d a t 6 / t h e r e h a v e b e e n n 0 r e c o g n i z e d neuro-displayed slow, large- amplitude counterclockwise ophthalmologic associations in the Asperger disor-torsional movements of the globes during spontane- der. Q u r patient had diffuse neuro- ophthalmo-ous right head tilting and similar clockwise torsional logic dysftmction. The finding of a retinochoroidal movements of the globes during spontaneous left coloboma in our patient's left eye suggests that the head tilting, with no associated vertical movement of inferior segmental optic hypoplasia and the adja-the globes. The torsional movements damped when cent infrapapillary depigmentation in the right eye he consciously maintained his head in its neutral po- probably also constitute a colobomatous defect. s i n o n - The A- pattern with alternating hyperdeviations in Ophthalmoscopy disclosed bilateral incyclodevi- side g a z G / a n d bilateral incyclodeviation are conation with optic disc and retinal malformations sistent w i t h p r i m a r y overaction of the superior ( Fig. 1). In the right eye, a patchy area of infrapap- oblique muscles. The alternating torticollis and as-illary retinochoroidal depigmentation was sepa- sociated torsional eye movements, which our pa-rated from a segmentally hypoplastic optic disc by tient c o u l d a c t i v e i y SUppr ess, represent a cyclical a narrow bridge of normal retina. In the left eye, a oculocephalic dyskinesia. An organized study of a well- circumscribed infrapapillary retinochoroidal large n u m b e r Qf patients with the Asperger disor-coloboma was separated from a horizontally elon- der would be useful to determine whether there gated optic disc by a similar bridge of normal- are a n y consistent ocular or neuro- ophthalmologic appearing retina. findings. FIG. 1. Optic disc photographs demonstrate inferior segmental hypoplasia with inferior juxtapapillary retinochoroidal depigmentation in the right eye ( A) and anomalous horizontal configuration of the optic disc with a juxtapapillary retinochoroidal coloboma in the left eye ( B). Note incyclodeviation of both fundi. / Neuro- OpMhalmol, Vol. 16, No. .3, 29.96 ASPERGER DISORDER 187 Acknowledgment: Supported in part by a grant from Research to Prevent Blindness, Inc. REFERENCES 1. Asperger H. Die ' Autishtischen Psychpathen' im Kindesal-ter. Arch Psychiatr Nervenkrankheiten 1944; 117: 76- 136. 2. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 4th ed. Washington, DC: American Psychiatric Association, 1994: 75- 7. 3. Frith U. Autism and Asperger syndrome. Cambridge: Cambridge University Press, 1991. 4. Creel DJ, Crandall AS, Pingree C, Ritvo ER. Abnormal elec-troretinograms in autism. Clin Vis Sci 1989; 1: 85- 8. 5. Ritvo ER, Creel D, Crandall AS, et al. Retinal pathology in autistic children- a possible biological marker for a subtype? / Am Acad Child Psychiatry 1986; 25: 137. / Neuw- Ophtlmlmol, Vol. 16, No. 3, 2996 |
