Department of Neurology, University of Kansas St. Francis Health System, Topeka, KS, USA.
Kansas City University, Kansas City, MO, USA.
BMC Neurol. 2023 Aug 29;23(1):312. doi: 10.1186/s12883-023-03360-x.
The standard of diagnosing primary lateral sclerosis, the Pringle criteria, requires three years of purely upper motor neuron symptom presentation before confirming diagnosis. This classic standard has been questioned on occasion due to its restrictive range of both time period and symptomatic exhibition.
This case report will review a 57-year-old Caucasian female who presented with pyramidal and extrapyramidal features suggestive of the exceedingly rare disease primary lateral sclerosis plus parkinsonism. We will describe the mixture of upper motor neuron signs and striking parkinsonian symptoms experienced by the patient, as well as the full diagnostic workup leading to her preliminary diagnosis. The details of this case will then be utilized to explore the diagnostic criteria of primary lateral sclerosis, as well as to work through the differential of conditions resembling Parkinson's disease.
The current criteria to diagnose primary lateral sclerosis may be excluding patients with the disease and is an ongoing area of investigation. A thorough differential including other neurodegenerative conditions is necessary to consider and requires long-term follow-up.
诊断原发性侧索硬化症的标准（Pringle 标准）要求在确诊前出现纯上运动神经元症状三年。由于其时间范围和症状表现都受到限制，因此该经典标准不时受到质疑。
本病例报告将回顾一位 57 岁的白人女性，她表现出锥体束和锥体外系特征，提示极为罕见的疾病原发性侧索硬化症伴帕金森病。我们将描述患者经历的上运动神经元体征和明显的帕金森症状的混合，以及导致她初步诊断的全面诊断性检查。然后，将利用该病例的详细信息探讨原发性侧索硬化症的诊断标准，并研究类似于帕金森病的疾病的鉴别诊断。
目前诊断原发性侧索硬化症的标准可能排除了患有该病的患者，这是一个正在研究的领域。全面的鉴别诊断包括其他神经退行性疾病是必要的，需要长期随访。
