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ABSTRACTKeratoconus is a disorder affecting the cornea,

characterized by its variably progressive centralthinning, which results in conically shaped protrusion.Patients with keratoconus are sometimes described ashaving peculiar personality characteristics. We presentthe case of a patient with keratoconus, complaining ofimpairment of concentration and memory disturbances.He reported slow progression of the complaints but wasfully capable of performing his professional and socialactivities. Neuropsychological assessment confirmedfluctuations of active attention and diminishedconcentration. Long term memory was within normallimits, but closer to the lower level). MMSE score was27. No significant changes were observed one yearafter baseline assessment. No major psychiatricdisorder was found. In this clinical case we aretempted to discuss the possible role of keratoconus,which, as it has been described in the literature, couldlead to some slight changes of behavior, forming a“keratoconic personality”.

Keywords: cognitive impairment, keratoconus,keratoconic personality

INTRODUCTIONKeratoconus is a disorder affecting the cornea,

characterized by its variably progressive centralthinning, which results in conically shaped protrusion.Severe cases can lead to disability and strongpsychological effects for the patient [1]. Patients withkeratoconus are sometimes described as havingpeculiar personality characteristics. Nevertheless, theliterature lacks a sufficient number of studies on thissubject.

SUBJECTIVE COGNITIVE COMPLAINTS IN APATIENT WITH KERATOCONUS: COGNITIVEIMPAIRMENT OR “KERATOCONICPERSONALITY”?

Ara G. Kaprelyan, Alexandra J. Tzoukeva, Ivan N. Dimitrov, Borislav Ivanov,Nadejda S. Deleva,Department of Neurology,Medical University, Varna, Bulgaria

Journal of IMAB - Annual Proceeding (Scientific Papers) 2012, vol. 18, book 3

Moreira et al. (2007) evaluated 68 patients withkeratoconus and 52 subjects without the disease. Theresults showed psychosocial impairment in thekeratoconus group. Patients were more pain avoiding,with more imaginative intuition, asocial withdrawal andanxious hesitation than the controls [2]. According toGorskova et al. (1998), who examined male and femalesubjects with keratoconus, the population of men wascharacterized by increased levels of psychasthenia andschizophrenia, whereas women developed depression,psychasthenia, and schizophrenia [3]. On the otherhand, the study of Cooke et al. (2003), comparing 118keratoconic patients and 75 myopic controls, found littleevidence to suggest that keratoconics might differsignificantly in personality from a group of moderateto high myopes who also depend on contact lenscorrection for distance vision. The authors could notsubstantiate the clinical notion of the so-calledkeratoconic personality [4]. Having conducted a studyincluding 109 patients with keratoconus, Mannis et al.(1987) revealed no specific complex of personalitycharacteristics attributable to the disorder either.Patients with keratoconus differed from normal controlsin much the same way as did patients with otherchronic eye diseases [5]. A different view of theproblem has been expressed by Giedd et al. (2005),who found keratoconus patients to be less respectfulof practitioners, uncooperative, and noncompliant withtreatment plans. This could cause physicians to lookunfavorably on such patients and would also explainwhy the clinical perception of keratoconus patientspersists, without a distinct keratoconus psychologicalprofile [6].

With this case report, describing the clinical andneuropsychological characteristics of a patient with

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bilateral keratoconus and subjective cognitivecomplaints, we would like to share our experience ofa possible “keratoconic personality”.

CASE REPORTA 58-year-old manager of an information

technology company was admitted to the clinic ofneurology for assessment due to cognitive complaints.Since the age of 7 years, the patient has complainedof gradually diminishing vision of both eyes. At the ageof 18 bilateral keratoconus was found and keratoplastywas performed. Vision was satisfactory afterappropriate correction. A few years later the patientbegan to notice impairment of concentration, memorydisturbances, such as difficulty in recalling names,numbers, and stories. He reported slow progression ofthe complaints. Though the patient was worried by his“mental problems”, he was fully capable of performinghis professional and social activities.

Physical and neurological exam revealed no

pathological findings. Hematology, blood chemistry andelectrolytes were normal. Ophthalmologicalexamination: visual acuity of 0.4 (20/50) for the righteye and 0.5 (20/40) for the left eye; anterior eyesegment: bilateral central transplants, with clouding onthe right; fundoscopy: partial illumination of the fundi,details not visible; keratoconus, postoperative state afterkeratoplasty. Brain MRI was conducted, showingnormal results (Fig. 1). Neuropsychological assessmentrevealed fluctuations of active attention and diminishedconcentration; max. memory fixation = 90% (mediumnormal level); long term memory = 60% (lower normallevel). MMSE score was 27. No impairment ofactivities of daily living was found (4-IADL score =0). The patient was referred to a psychiatrist who foundno major psychiatric disorder. One year laterneuropsychological assessment was repeated. MMSEscore was still 27 and no significant changes wereobserved on the other above-mentioned test scores.

DISCUSSIONThe patient whose case we describe was

diagnosed with keratoconus at the age of 18. He wastreated accordingly and promptly, and attained asatisfactory level of vision. The patient was referred

to our clinic because of his cognitive complaints. Wehad to express our opinion of whether he was, or mightbecome, demented. Of course, according to the DSM-IV criteria that we currently use, dementia was easilyruled out. This was also the case for mild cognitive

Fig. 1. Brain MRI. T2 FLAIR, axial (A) and coronal (B) planes: normal cerebral imaging.

A B

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1. Epstein AB. Keratoconus &Related Disorders. North ShoreContact Lens and Vision Consultants.2000; 87 p.

2. Moreira LB, Alchieri JC, Belfort RJr, Moreira H. Psychological and socialaspects of patients with keratoconus.[Article in Portuguese] Arq BrasOftalmol. 2007 Mar-Apr;70(2):317-322.[PubMed] [CrossRef]

Address for correspondenceAssoc. Prof. Ara Kaprelyan, MD, PhDDepartment of Neurology, Medical University, Varna55, Marin Drinov Str., 9002 Varna, Bulgaria,Å-mail: arakapri07@yahoo.co.uk;

3. Gorskova EN, Sevostianov EN,Baturin NA. Results of psychologicaltesting of patients with keratoconus.[Article in Russian] Vestn Oftalmol.1998 Nov-Dec;114(6):44-45. [PubMed]

4. Cooke CA, Cooper C, Dowds E,Frazer DG, Jackson AJ. Keratoconus,myopia, and personality. Cornea. 2003Apr;22(3):239-242. [PubMed]

5. Mannis MJ, Morrison TL, Zadnik

K, Holland EJ, Krachmer JH.Personality trends in keratoconus. Ananalysis. Arch Ophthalmol. 1987Jun;105(6):798-800. [PubMed][CrossRef]

6. Giedd KK, Mannis MJ, MitchellGL, Zadnik K. Personality in kerato-conus in a sample of patients derivedfrom the internet. Cornea. 2005 Apr;24(3):301-307. [PubMed]

REFERENCES:

impairment, according to the modified criteria ofPetersen, as the subjective cognitive complaints werenot supported by abnormal neuropsychological testperformance. The patient did not conform to thecriteria one year later either. Moreover, one year afterthe baseline assessment, there was no deterioration ofhis neuropsychological performance. The complaintswere not due to a psychiatric disorder. We are

therefore tempted to discuss the possible role ofkeratoconus, which, as it has been described in theliterature, could lead to some slight changes ofbehavior, forming a “keratoconic personality” [2, 3, 5]..Unfortunately, single cases are insufficient for acomplete understanding of the problem of whether suchpersonality type really exists, and more studies areneeded for its clarification.