Keratoconus and Psychosis
To the Editor: We report on a patient with a provisional diagnosis of schizophreniform disorder and keratoconus, an eye disease associated with abnormal personality characteristics (1–3). Keratoconus is a bilateral degenerative disease of the cornea beginning in adolescence, with a prevalence of 1 in 2,000 (1). Ophthalmologists have long speculated about the existence of a “keratoconic personality,” described as paranoid, anxious, compulsive, and somatically oriented (1).
Several studies have documented differences in personality characteristics when keratoconic patients are compared to healthy subjects, including higher scores on the schizophrenic subscale of the MMPI (3). It is unclear, however, whether this is independent of the psychological stress caused by chronic eye disease (1).
Mr. A was a 23-year-old man admitted to our inpatient psychiatric service after a 5-month history of delusions, including the belief that his corneal transplants were radio transmitters implanted in his head. He had been diagnosed with keratoconus at age 17 and underwent bilateral corneal transplantation at age 21. He had had one previous psychiatric hospitalization in his late teens after an incident in which he lost his temper, doused his car with gasoline, and set it afire. He also had a history of cocaine, amphetamine, and LSD abuse. His last use of LSD was 2 years before admission, and his last use of amphetamines and cocaine was a month before admission.
Mr. A had illogical speech, inappropriate affect, and frequent thought derailment. He felt that his problems were due to the “audiovisual stuff” implanted in his eyes. He also complained of the sensation of being burned by cigarettes on his legs and back. Mr. A was given olanzapine, 20 mg/day. Soon after admission he became less agitated, although his thoughts easily became derailed when he discussed his delusions, which persisted throughout his 3-week hospitalization.
Although the association in our patient may have been coincidental, we believe that the co-occurrence of psychosis and other syndromes is of value and may lead to the further elucidation of genetic correlates and pathophysiological processes, as in velocardiofacial syndrome and schizophrenia (4). Keratoconus is a genetic disease with familial clustering; like schizophrenia, it is thought to be a final common pathway stemming from multiple etiologies (5). Linkage studies have identified possible genetic loci, including loci mapped to chromosome 21. Although there is no evidence of linkage between keratoconus and schizophrenia, observations of the co-occurrence of these two syndromes might prompt further investigation.
1. Mannis MJ, Morrison TL, Zadnik K, Holland EJ, Krachmer JH: Personality trends in keratoconus. Arch Ophthalmol 1987; 105:798-800Crossref, Medline, Google Scholar
2. Swartz NG, Cohen EJ, Scott DG, Genvert GI, Arentsen JJ, Laibson PR: Personality and keratoconus. CLAO J 1990; 16:62-64Medline, Google Scholar
3. Gorskova EN, Sevost"ianov EN, Baturin NA: [Results of psychological testing of patients with keratoconus.] Vestn Oftalmol 1998; 114:44-45 (Russian)Medline, Google Scholar
4. Eliez S, Blasey CM, Schmitt EJ, White CD, Hu D, Reiss AL: Velocardiofacial syndrome: are structural changes in the temporal and mesial temporal regions related to schizophrenia? Am J Psychiatry 2001; 158:447-453Link, Google Scholar
5. Klintworth GK: Advances in the molecular genetics of corneal dystrophies. Am J Ophthalmol 1999; 128:747-754Crossref, Medline, Google Scholar
