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进行性核上性麻痹患者的表型变异

Phenotypic Variants of Patients with Progressive Supranuclear Palsy.

作者信息

Gültekın Murat

机构信息

Department of Neurology, Erciyes University School of Medicine, Kayseri, Turkey.

出版信息

Noro Psikiyatr Ars. 2017 Nov 8;57(1):61-64. doi: 10.5152/npa.2017.22771. eCollection 2020 Mar.

Abstract

INTRODUCTION

Progressive supranuclear palsy (PSP) is a commonly observed disease among Parkinson plus syndromes, and its early and accurate diagnosis is usually not possible. PSP is currently reported to have eight different subtypes. This study aims to determine the phenotypic subtypes of PSP and reveal their accompanying characteristics.

METHODS

Eighteen patients who were diagnosed as having PSP according to the National Institute for Neurological Disorders and Society for PSP (NINDS-SPSP) criteria were included in the study. Initial symptoms of the patients, their initial diagnosis, course of the disease with time, cranial images, and predominant clinical features were assessed with regard to the subtypes of PSP.

RESULTS

The mean age of the patients was 65.83 years. According to the NINDS-SPSP criteria, all patients were diagnosed as having probable PSP. An evaluation of the patients with respect to the subtypes showed that five different subtypes had predominant characteristics. Richardson's syndrome was found to be the most common subtype.

CONCLUSION

Classifying PSP with regard to its phenotypes may be useful for informing clinicians about the diverse clinical presentations of PSP, thereby possibly improving the diagnostic sensitivity of the disease.

摘要

引言

进行性核上性麻痹(PSP)是帕金森叠加综合征中常见的疾病,通常无法早期准确诊断。目前报道PSP有八种不同亚型。本研究旨在确定PSP的表型亚型并揭示其伴随特征。

方法

本研究纳入了18例根据美国国立神经疾病与中风研究所和进行性核上性麻痹协会(NINDS - SPSP)标准诊断为PSP的患者。针对PSP的亚型,评估了患者的初始症状、初始诊断、疾病随时间的进程、头颅影像以及主要临床特征。

结果

患者的平均年龄为65.83岁。根据NINDS - SPSP标准,所有患者均被诊断为可能的PSP。对患者亚型的评估显示,有五种不同亚型具有主要特征。发现理查森综合征是最常见的亚型。

结论

根据PSP的表型进行分类可能有助于临床医生了解PSP的多种临床表现,从而可能提高该疾病的诊断敏感性。

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