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首页> 外文期刊>Journal of Neurology, Neurosurgery and Psychiatry >Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria.
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Progressive supranuclear palsy: neuropathologically based diagnostic clinical criteria.

机译：进行性核上性麻痹：基于神经病理学的诊断临床标准。

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All cases examined postmortem at the Mayo Clinic that met the classic neuropathological criteria for progressive supranuclear palsy (PSP) were identified for retrospective clinical analyses. The necropsy material was re-examined by a second neuropathologist to confirm the pathological diagnosis of PSP, yielding 12 cases. A range of clinical signs were documented in these patients, with numerous findings beyond those noted in the original descriptions of this disorder. Atypical clinical findings included absence of supranuclear gaze palsy (two cases), prominent asymmetry (two), arm dystonia (two), upper limb apraxia (two), myoclonus (two), chorea (one), eyelid opening apraxia (one), and respiratory disturbance (one). A definite clinical diagnosis of PSP had been made during life in only eight of the 12 patients. From the retrospective analysis of these 12 cases, a set of clinical criteria were developed for the premortem diagnosis of PSP emphasising differences from other akinetic-rigid disorders.
机译：在梅奥诊所验尸后检查的所有病例均符合进行性核上性麻痹（PSP）的经典神经病理学标准，并进行回顾性临床分析。尸检材料由第二位神经病理学家重新检查，以确认PSP的病理诊断，产生12例。在这些患者中记录了一系列临床体征，除了该疾病的原始描述中记载的发现以外，还有许多发现。非典型的临床表现包括：无核上凝视麻痹（2例），明显的不对称（2例），手臂肌张力障碍（2例），上肢失用症（2例），肌阵挛（2例），舞蹈症（1例），眼睑开裂失用（1例），和呼吸障碍（一）。 12例患者中只有8例在生命中对PSP进行了明确的临床诊断。通过对这12例病例的回顾性分析，制定了一套PSP死前诊断的临床标准，强调了与其他非运动性刚性疾病的差异。

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