Neuropathologic findings in a patient with hemiparkinsonism and hemiatrophy syndrome.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Neuropathology Pub Date : 2024-07-07 DOI:10.1111/neup.12994
Masataka Nakamura, Ayako Tsuge, Kosuke Miyake, Takenobu Kunieda, Hirofumi Kusaka, Yusuke Yakushiji
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Abstract

The first postmortem neuropathological findings of a hemiparkinsonism and hemiatrophy (HPHA) patient are presented. A 50-year-old man developed resting tremors affecting the right hand and leg, followed by mild clumsiness of the right hand. On examination, he exhibited muscle atrophy of the right leg extremity, accompanied by right-sided parkinsonism. Brain magnetic resonance imaging was normal. Based on the clinical and radiological findings, HPHA syndrome was diagnosed, showing a good response to L-DOPA. He gradually developed muscular atrophy of the right distal upper extremity. Thirteen years after the onset of the disease, left-sided parkinsonism appeared. The patient died of Trousseau's syndrome associated with a rapidly emerging pancreatic tumor. The total duration of the disease was 14 years. Neuropathologically, the substantia nigra showed markedly left-predominant neuronal loss, along with almost symmetrical Lewy body (LB) pathology. These findings indicated that the patient originally had fewer neurons in the left substantia nigra than in the right, probably caused by congenital or childhood cerebral injury, followed by the development of unilateral parkinsonism due to the progression of LB pathology. Despite our extensive neuropathological analysis, we could not specify the etiology or anatomical substrate responsible for the development of right upper and lower extremity atrophy. Further clinicopathological studies are needed to elucidate the pathoanatomical areas causing hemiparkinsonism and hemiatrophy.

一名患有半帕金森症和半萎缩综合征的患者的神经病理学发现。
本文首次介绍了一名半帕金森病和半萎缩症(HPHA)患者的尸检神经病理学发现。一名 50 岁的男子出现了影响右手和右腿的静止性震颤,随后右手出现轻度笨拙。经检查,他的右腿肢体肌肉萎缩,并伴有右侧帕金森氏症。脑磁共振成像正常。根据临床和放射学检查结果,他被诊断为 HPHA 综合征,对左旋多巴反应良好。他的右上肢远端逐渐出现肌肉萎缩。发病 13 年后,出现了左侧帕金森病。患者死于特鲁索综合征,并伴有迅速出现的胰腺肿瘤。总病程为 14 年。从神经病理学角度看,黑质显示出明显的左侧神经元缺失,以及几乎对称的路易体(LB)病变。这些结果表明,患者左侧黑质的神经元数量原本少于右侧,这可能是先天性或儿童期脑损伤所致,随后由于路易体病变的发展而发展成单侧帕金森病。尽管我们进行了大量的神经病理学分析,但仍无法确定导致右侧上下肢萎缩的病因或解剖基质。我们需要进一步的临床病理学研究来阐明导致半帕金森病和半营养不良的病理解剖学区域。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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