LOSS OF VISUAL-ACUITY - INITIAL SYMPTOM O F CREUTZFELD-JACOB-DISEASE

Background: Creutzfeldt-Jacob-Disease (CJD) is considered to be transm issible by PRION. Their most frequent symptoms are changes in intellec tual function, together with abnormalities of cerebellar function, but in some instances actual impairment of visual acuity precedes mental changes. The latter variant of CJD is named Heidenhain disease or occi pitoparietal variant and the key symptom of it is cortical blindness. Diseases transmissible by PRION gained public interest because of the discussion about possible transmission of bovine spongiforme encephalo pathy to man; this risk was evoked because of the occurence of unusual histories of CJD in young English people. Transmission of CJD to othe r patients is reported in the literature, thus early diagnosis is impo rtant. Patient: A 70 year old butcher presented bilateral loss of visu al acuity and metamorphopsia. Ophthalmologic examination revealed bila teral cornea guttata, cataracta incipiens and normal fundus. We perfor med a systemic steroids therapy because of the simultaneously occuring symptoms. headache, loss of body weight and elevated blood sedimentat ion rate, suspective for M. Horton. During hospitalisation the patient suffered from progressing dementia, ataxia and myoclonic jerks. Neuro n-specific enolase was elevated in liquor, the clinical symptoms were progressing and the EEG showed typical triphasic complexes: thus clini cal diagnosis of CJD was evident. The diagnosis was confirmed by autop sy. Conclusion: Patients suffering from CJD may initially consult thei r ophthalmologist because of their symptoms. The ophthalmologist shoul d therefore be aware of symptoms and history of this disease.