Hauw J J, Daniel S E, Dickson D, Horoupian D S, Jellinger K, Lantos P L, McKee A, Tabaton M, Litvan I
Raymond Escourolle Neuropathology Laboratory, INSERM U 360, Hôpital de la Salpêtrière, Paris, France.
Neurology. 1994 Nov;44(11):2015-9. doi: 10.1212/wnl.44.11.2015.
We present the preliminary neuropathologic criteria for progressive supranuclear palsy (PSP) as proposed at a workshop held at the National Institutes of Health, Bethesda, MD, April 24 and 25, 1993. The criteria distinguish typical, atypical, and combined PSP. A semiquantitative distribution of neurofibrillary tangles is the basis for the diagnosis of PSP. A high density of neurofibrillary tangles and neuropil threads in the basal ganglia and brain-stem is crucial for the diagnosis of typical PSP. Tau-positive astrocytes or their processes in areas of involvement help to confirm the diagnosis. Atypical cases of PSP are variants in which the severity or distribution of abnormalities deviates from the typical pattern. Criteria excluding the diagnosis of typical and atypical PSP are large or numerous infarcts, marked diffuse or focal atrophy, Lewy bodies, changes diagnostic of Alzheimer's disease, oligodendroglial argyrophilic inclusions, Pick bodies, diffuse spongiosis, and prion protein-positive amyloid plaques. The diagnosis of combined PSP is proposed when other neurologic disorders exist concomitantly with PSP.
我们展示了1993年4月24日和25日在马里兰州贝塞斯达国立卫生研究院举办的研讨会上提出的进行性核上性麻痹（PSP）的初步神经病理学标准。这些标准区分了典型、非典型和混合型PSP。神经原纤维缠结的半定量分布是PSP诊断的基础。基底神经节和脑干中神经原纤维缠结和神经毡丝的高密度对于典型PSP的诊断至关重要。受累区域的tau阳性星形胶质细胞或其突起有助于确诊。PSP的非典型病例是异常严重程度或分布偏离典型模式的变体。排除典型和非典型PSP诊断的标准包括大的或多发性梗死、明显的弥漫性或局灶性萎缩、路易小体、阿尔茨海默病的诊断性改变、少突胶质细胞嗜银包涵体、皮克小体、弥漫性海绵状变以及朊蛋白阳性淀粉样斑块。当其他神经系统疾病与PSP同时存在时，建议诊断为混合型PSP。
