Progressive visuospatial disorder: A behavioristic focal dementia syndrome. Case study
KOUNTI F., KITSIOS G., TSOLAKI S., TSOLAKI M., KAZIS A.

The term dementia is non-spesific and it is reffering to the cognitive and behavioristic disturbances that have an insidious onset and gradual deterioration. Four neuropsychological profiles of dementia have been described and progressive visuospatial dysfunction is one of them. It is characterized by the insidious onset and gradual exacerbation of visuospatial deficits, such as many features of Balint syndrome, hemispatial disorientation with neglect, visual agnosia or prosopoagnosia. There are no primary memory deficits within the first two years, although other mild cognitive impairments may coexist. Alternations in daily living activities are attributed predominantly to the visuospatial deficits. The patients complain of an epinephelus vision, a difficulty in reading and in recognizing familiar persons and of an inability to find objects in common view. The underlying pathology includes many diseases such as Alzheimer’s disease, Creutzfeld-Jakob disease, focal brain atrophy with gliosis, posterior brain atrrophy and glycogen storage diseases. In order to identify a dementia case as progressive visuospatial dysfunction, the following criteria should be met: 1) gradual deterioration of the visuospatial and visuoconstructional function to a point that disturbs the daily living activities, 2) No primary memory deficits within the first two years and 3) other mild cognitive deficits (linguistic, behavioristic) may coexist.

Our case study is a 61-year-old right-handed agriculturist, who was examined at the outpatient memory and dementia clinic of 3rd University Department of Neurology in Papanicolaou hospital and he was found to have a progressive impairment of his visuospatial skills during the last six years. He complained of memory deficits, dressing apraxia and inability to find objects that are in common view. he had a serious difficulty in reading and he paid frequent visits to his opthalmologist, complaining of his vision. He was aware of his disability and he felt ashamed because of it. The patient had a family history of dementia of unknown cause. Opthalmologic examination did not reveal any disorder incompatible with his age. The findings of the neurological clinical examination were limited and non-specific. Magnetic resonance imaging revealed generalized brain atrophy, without any focal lesions. However, the atrophy was more localized in the right brain areas, including the parietal, temporal and occipital lobe, accompanied by a dilatation of the ventricular system. Detailed neuropsychological assessment revealed many disturbances compatible with the progressive visuospatial disorder, such as a disturbed postural orientation and inability to drive or use the digital keypad. Many other functions were found to be intact, such as speaking, time orientation and working memory. The neuropsychological tests that were used including the Geriatric Depression Scale, the Functional Rating Scale, for Symptoms of Dementia, the Dual Task Paradigm, the CAMCOG, the STROOP test, the WCST, the FUCAS, the Rivermead Behavioral Memory Test and Boston Naming Test. The diagnosis of neurodegenerative dementia was suppotred by family history, the neuropsychological, neuroimaging and laboratory tests. This case was very interesting because the progressive visuospatial disorder is a rare clinical case and the differential diagnosis of this disorder is important for the specification of the treatment strategy. Our patient was treated with acetylcholinesterase inhibitors and he followed a specialized cognitive rehabilitation program suitable for his deficits.

Key words: Visuospatial Disorder, Focal Degenerative Dementia.