Bilateral inferior hemianopsia as initial symptom in Creutzfeldt-Jakob disease (Heidenhain type)

History and signs: We report on a 60-year-old woman who developed within a few weeks bilateral inferior hemianopsia. Morphological findings of the eye s were normal. Neurological examination and cerebral CT/MRI were normal too . P 100 latency of visual evoked potentials (VEP) of both eyes, however, wa s delayed. Outcome: During the course of the disease visual field narrowed progressive ly and 3 months after the first symptoms the patient went blind. Only at th at time myoclonia, hyperkinesia, and psychiatric symptoms appeared. Changes of EEG and cerebrospinal fluid were suggestive of Creutzfeldt-Jakob diseas e (CJD). The patient died after 5 1/2 months and diagnosis was confirmed ne uropathologically with mainly occipital spongiform encephalopathy. Conclusion: This case illustrates the diagnostic difficulties of the Heiden hain type of CJD and demonstrates that in patients with progressive visual field defects without morphological abnormalities of the eyes or radiologic al abnormalities of the brain CJD should be considered. It should be looked out for additional neurological (especially extrapyramidal) and psychiatri c symptoms.