Afasia primaria progresiva asociada a degeneración corticobasal

Abstract

Progressive primary aphasia generally progresses to global cognitive deterioration of Alzheimer type, although occasionally it remains unchanged. The neuropathological findings are varied: Alzheimer, spongiform etc. The case we describe followed a very unusual course. Initially there was a clinical picture of progressive primary aphasia with which that of corticobasal degeneration was later associated. The patient, who had no relevant previous clinical history apart from chronic obstructive pulmonary disease presented with gradually deteriorating oral expression, with no loss of memory and maintenance of autonomy in everyday activities. This condition was later associated with slowing of voluntary movements, hypertonia of the right limbs, difficulty in manipulation with the right hand and myoclonic jerks. Neuro imaging on cranial CT showed global cerebral atrophy, most marked on the right and cerebral SPECT showed reduced fronto temporal uptake. No other significant alterations were found in the investigations done. On neuropsychological examination there was alteration of language in the form of mutism and reduction in denomination, repetition, fluency and comprehension (except for simple orders). However, copies of drawings were done correctly with absence of apraxia of ideas or ideomotor initiation. Handwriting was also normal. . The unusual aspect of our case was the simultaneous presentation of an aphasic disorder and corticobasal degeneration.