A previously healthy woman began to present recurrent episodes of reduplicative paramnesia within her home and later structured visual hallucinations. The case was initially oriented as an incipient vascular dementia. Detailed anamnesis and neuropsychological examination suggested a rapidly progressive pattern of neuropsychological deficits mostly attributable to parieto-occipital disturbances with some component of fronto-temporal involvement. Subsequently, cerebellar symptoms were added. Although the initial imaging studies were inconclusive, the MRI performed during follow-up showed a series of findings compatible with a prion disease. Based on the neuropsychological and clinical features and the imaging pattern, the diagnosis of Heidenhain Variant of Creutzfeldt-Jakob disease was established. This is the first report of a Heidenhain Variant of Creutzfeldt-Jakob disease presenting as a reduplicative paramnesia as the first manifestation of this disease.
Keywords: CJD; Creutzfeldt-Jakob; Dementia; Heidenhain; Reduplicative paramnesia.