Farkli Bir Demans Sendromu: Posterior Kortikal Atrofi: Klinik, Kognitif Degerlendirme ve Beyin Spect Bulgulari/A Distinct Dementia Syndrome: Posterior Cortical Atrophy: Clinical and Cognitive Assesment, and Brain Spect Findings

ÖZET. Amaç: Posterior kortikal atrofi, kortikal vizüel disfonksiyon bulgulari ile baslayan ve nadir görülen bir demans sendromudur. Hastaligin ana klinik görünümü Balint sendromu, Gerstmann sendromu, vizüel agnozi, aleksi, agrafi ve transkortikal sensoriyel afaziyi içerir. Biz bu çalismada PKA ön tanili hastalarimizin klinik özelliklerini ve beyin SPECT bulgularini belirlemeyi amaçladik. Yöntemler: Çalismaya DEÜTF Nöroloji Anabilim Dali Demans polikliniginde izlenen, klinik olarak PKA düsünülen, ayrintili kognitif testleri yapilan ve ayni zamanda beyin SPECT incelemesi yapilmis olan olgular alindi. Toplam 1105 olgunun dosyasi tarandi ve bunlarin arasindan klinik olarak PKA düsünülen 9 olguya ulasildi. Bulgular: Presenil yaslarda baslangiç, görsel uzamsal yeteneklerdeki kayiplarin amnestik tablodan daha belirgin olmasi, vizüel agnozinin erken dönemde saptanmasi, depresyonun, erken dönemde apraksinin eslik etmesi, konusma akiciliginin iyi olmasi PKA'yi düsündürecek özellikler arasindadir. Beyin SPECT incelemesi de PKA tanisi düsünülen hastalarda oldukça yararli bir inceleme yöntemidir. Posterior beyin bölgelerinde hipoperfüzyon saptanan hastalar, PKA açisindan degerlendirilmelidir. Sonuç: Olgularin yas ortalamasi 59.11±4.1 yil, hastaligin baslangiç yasi 55.83±4.97 yildi ve tümü kadindi. 8 olguda tüm bilesenleriyle Gerstmann sendromu varken, 7 olguda simultanagnozi saptandi. Olgularin tamaminda sözel belleklerindeki bozulmadan daha belirgin olan agir görsel bellek bozuklugu vardi. Tüm olgularda görsel mekansal islevler ileri derecede bozulmustu. Sekil kopyalama olgularin tümünde ileri derecede bozulmustu. Saat çizimi ya tamamlanamadi ya da ileri düzeyde bozuktu. Sekiz hastanin ideomotor apraksisi vardi. Konusma tüm olgularda akiciydi, 8 olguda yakinmalarin baslangici ile es zamanli olarak depresyon saptandi. Beyin SPECT incelemesinde posterior temporal, paryetal ya da oksipital loblarda hipoperfüzyon izlendi. Perfüzyon bozuklugu 6 olguda asimetrikti. (Nöropsikiyatri Arsivi 2010; 47: 201-6). Anahtar kelimeler: Demans, Posterior Kortikal Atrofi, beyin SPECT, simultanagnozi, Gerstmann. ABSTRACT. Objective: Posterior cortical atrophy (PCA) is a rare dementia syndrome that presents primarily with signs of cortical visual dysfunction. The main clinical features of the disease include Balint's syndrome, Gerstmann's syndrome, visual agnosia, alexia, agraphia, and transcortical sensory aphasia. In this study, we aimed to determine the clinical characteristics and the brain single proton emission computed tomography (SPECT) findings of patients clinically diagnosed as having PCA. Methods: The subjects followed in the dementia outpatient clinic of neurology department, Dokuz Eylül University Hospital, suspected to have PCA and who have undergone cognitive testing and brain SPECT inspection were included in the study. Records of a total of 1105 subjects were screened and among them, nine patients who were clinically thought to have PCA were reached. Results: The presenile age onset, more prominent loss of visuospatial abilities than amnestic feature, early detection of visual agnosia, comorbidity of depression and apraxia in early term, fluent speech are among the symptoms that preoccupy PCA. Brain SPECT is a useful examination method in patients suspected to have PCA. Patients with hypoperfusion in the posterior brain regions also must be taken into account in terms of recognizing clinical features of PCA. Conclusion: The mean age of the subjects was 59.11±4.1 years, onset of the disease was 55.83±4.97 years and all patients were female. Eight cases had Gerstmann's syndrome with all components, whereas in seven of them simultanagnosia was established. They had severe visual memory defect, which was more prominent than verbal memory defect. Visual-spatial functions were extremely deteriorated in all patients. Figure copying was severely worsened in all patients. Clock drawing was either not accomplished or was severely bad. Eight patients had ideomotor apraxia. Speech was fluent in all patients. Eight patients had depression that simultaneously developed with the onset of the complaints. Brain SPECT revealed either unilateral or bilateral hypoperfusion in the posterior temporal, parietal or occipital lobes. (Archives of Neuropsychiatry 2010; 47: 201-6). Key words: Dementia, posterior cortical atrophy, brain SPECT, simultanagnosia, Gerstmann.

© Galenos Yayinevi 2010. Provided by ProQuest LLC. All Rights Reserved.

The use of this website is subject to the following Terms of Use