Rapid Progressive Form of Posterior Cerebral Dysfunction with Apperceptive Agnosia in a 34-Year-Old Man - Another Case of Heidenhain's Syndrome?

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Abstract
Posterior cortical atrophy (PCA) or progressive posterior cerebral dysfunction is a neurodegenerative disorder initially dominated by disturbances in higher visual functions including object agnosia, prosopagnosia, aiexia, environmental agnosia and Baiint's syndrome. Language, memory, insight and judgement remain relatively preserved until late in the course. The aetioiogy of thls syndrome has been discussed controversially. We report the case of a 34-year-old music teacher who presented with the clinical picture of PCA dominated by a severe apperceptive agnosia. The most striking neuropsychoiogical feature of the syndrome was the complete dissociation between ail forms of visual processing and high level language comprehension. Serial MRI and PET scans demonstrated a moderate bllaterai parieto-occipital atrophy and a severe hypometabolism of the occipito-temporoparietai association cortices (left>right) excluding the primary cortical fields, the frontal lobes, the basal ganglia and the cerebellum. However, the subsequent course showed a rapid deterioration of nearly all cognitive capacities and personailty traits and the development of an aklnetic-rigid syndrome associated with myoclonus. PET now showed a severe hypometabolism of almost ail cortical association areas sparing again the primary motor and visual cortex. At that stage, the clinical diagnosis of Creutzfeldt-Jakob disease (Heidenhain's syndrome) was made. Taking into account the recent reports of neuropathoiogicai findings in patients with PCA, our case gives further evidence that PCA is a clinically and aetiologically heterogeneous syndrome rather than a distinct neuropathoiogicai entity.

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