M. Rauber et al., LOSS OF VISUAL-ACUITY - INITIAL SYMPTOM O F CREUTZFELD-JACOB-DISEASE, Klinische Monatsblatter fur Augenheilkunde, 211(5), 1997, pp. 10-13
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.