Journal of Parkinson's Disease and Movement Disorders

Ali AKYOL, Mehmet ESKİCİ, Ayça ÖZKUL, Ahmet ŞAİR, Utku Ogan AKYILDIZ, Nefati KIYLIOĞLU

Adnan Menderes Üniversitesi Tıp Fakültesi Nöroloji Anabilim Dalı, Aydın, Türkiye

Keywords: Autonomic failure; Parkinson-plus syndromes; progressive supranuclear palsy.

Abstract

Progressive supranuclear palsy (PSP) is an unusual neurodegenerative disease, which affects the brainstem and basal ganglia. Its true etiology is still unknown. The patients are admitted with complaints of supranuclear gaze palsy, pseudobulber palsy, progressive axial rigidity, postural instability and mild dementia. In this article, we analyzed five cases of PSP, diagnosed at a later stage, whose initial diagnosis was Parkinson’s disease. There were characteristic clinical findings of PSP in all five of the cases. Four of them had typical cranial imaging findings for the PSP (superior cerebellar peduncle and midbrain atrophy on magnetic resonance imaging, “hummingbird” sign on the midsagittal plane, dilatation of third ventricle). One patient had mild cerebral atrophy in her cranial computed tomography imaging. All patients had failure in autonomous tests. Two patients had mild cognitive impairment and two had mild dementia. These patients were under donepezil treatment. One of the patients had history of diabetes mellitus for more than 20 years, which perplexed the clinical diagnosis. On the other hand, renal and pulmonary failure related to Wegener’s granulomatosis syndrome were added to the clinical findings in the last period. All five of them were taking L-dopa and/or dopamine agonists. Although L-dopa treatment may provide slight improvement in bradykinesia, it has no use for speech and balance disorders.