GH Sergievsky Center, College of Physicians and Surgeons, Columbia University, New York, USA 10032, USA.
J Neurol Neurosurg Psychiatry. 2011 Mar;82(3):261-5. doi: 10.1136/jnnp.2010.215681. Epub 2010 Aug 27.
Rest tremor may occur in as many as 30% of essential tremor (ET) patients. It is not clear whether this tremor is a sentinel marker for brainstem Lewy body pathology. Here we report the clinical and post-mortem findings of nine ET cases with upper-extremity rest tremor in the absence of other parkinsonian features.
All brains had a complete neuropathological assessment. Tissue sections from the brainstem and basal ganglia were immunostained with α-synuclein antibody.
All cases had longstanding ET (median duration=42 years) with moderate to severe arm tremor. Rest tremor involved both arms in seven (77.8%) cases and one arm in two cases. The rest tremor score was correlated with the total action tremor score (r=0.69, p=0.04). The number of torpedoes was elevated, and Purkinje cells, reduced. Post-mortem changes in the substantia nigra pars compacta (SNc), caudate, putamen and globus pallidum were minimal, and neither Lewy bodies nor Lewy neurites were evident.
In nine ET brains with upper-extremity rest tremor, neither Lewy body-containing neurons nor Lewy neurites were found on α-synuclein immunostained sections, and other pathological changes in the basal ganglia were minimal. These data support the notion that isolated rest tremor in longstanding ET is not the expression of underlying Lewy body pathology in the SNc.
多达 30%的特发性震颤（ET）患者可能出现静止性震颤。目前尚不清楚这种震颤是否是脑干路易体病理的一个前驱标志物。在此，我们报告了 9 例无其他帕金森特征的上肢静止性震颤的 ET 病例的临床和尸检结果。
所有大脑均进行了完整的神经病理学评估。使用α-突触核蛋白抗体对脑干和基底节组织切片进行免疫染色。
所有病例均有长期（中位病程=42 年）的 ET，手臂震颤程度为中度至重度。7 例（77.8%）病例的双侧上肢出现静止性震颤，2 例病例的单侧上肢出现静止性震颤。静止性震颤评分与总动作性震颤评分呈正相关（r=0.69，p=0.04）。Torpedo 现象增多，浦肯野细胞减少。黑质致密部（SNc）、尾状核、壳核和苍白球的尸检改变最小，未见路易体或路易神经纤维。
在 9 例上肢静止性震颤的 ET 大脑中，α-突触核蛋白免疫染色切片均未发现含有路易体的神经元或路易神经纤维，基底节的其他病理改变也很小。这些数据支持这样一种观点，即长期 ET 中孤立的静止性震颤不是 SNc 中潜在的路易体病理的表现。
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