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针对淀粉样变性的尺神经靶向活检及其诊断性和非诊断性活检的潜在影响:病例说明

Targeted biopsy of the ulnar nerve for amyloidosis with potential implications of a diagnostic and nondiagnostic biopsy: illustrative case.

作者信息

O'Driscoll Christopher J L, Dyck P James B, Spinner Robert J

机构信息

Departments of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota.

Departments of Neurology, Mayo Clinic, Rochester, Minnesota.

出版信息

J Neurosurg Case Lessons. 2025 Sep 1;10(9). doi: 10.3171/CASE25484.

DOI:10.3171/CASE25484
PMID:40889391
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12400844/
Abstract

BACKGROUND

MRI-guided targeted fascicular biopsy has been a valuable technique for diagnosing idiopathic neuropathies. It has an 80% yield and a minor permanent morbidity rate of 5%.

OBSERVATIONS

A 48-year-old woman with a history of localized amyloidosis to the orbital muscle 2 years earlier presented with new neurological symptoms. Extensive evaluations including skin, muscle, and distal cutaneous nerve biopsies were negative, and empirical treatment trials did not improve her condition. An MRI-guided targeted biopsy of the right ulnar nerve in the distal arm was planned. During surgery, a medial triceps branch arising from the ulnar nerve was identified and was biopsied along with a neighboring fascicle of the ulnar nerve to the flexor carpi ulnaris. The pathology of the ulnar nerve fascicle but not the contiguous triceps branch showed amyloid (AL [kappa] subtype on mass spectroscopy). The patient was started on treatment.

LESSONS

The positive biopsy of the ulnar nerve fascicle allowed targeted treatment of the neural amyloidosis. While the negative biopsy of the triceps branch could be attributed to sampling error, it could also be due to the fact that this branch found within the ulnar nerve is thought to originate from the radial nerve. https://thejns.org/doi/10.3171/CASE25484.

摘要

背景

磁共振成像(MRI)引导下的靶向束状活检一直是诊断特发性神经病的一项重要技术。其活检阳性率为80%,永久性轻微发病率为5%。

观察结果

一名48岁女性,2年前有眼眶肌肉局限性淀粉样变性病史,现出现新的神经症状。包括皮肤、肌肉和远端皮神经活检在内的广泛评估均为阴性,经验性治疗试验也未能改善她的病情。计划对其右臂远端的尺神经进行MRI引导下的靶向活检。手术过程中,识别出一条由尺神经发出的肱三头肌内侧支,并与尺神经至尺侧腕屈肌的相邻束状组织一起进行活检。尺神经束状组织的病理检查显示有淀粉样物质(质谱分析为AL[κ]亚型),而相邻的肱三头肌支未见异常。随后患者开始接受治疗。

经验教训

尺神经束状组织活检阳性使得对神经淀粉样变性能够进行靶向治疗。肱三头肌支活检阴性可能是由于取样误差,也可能是因为这条在尺神经内发现的分支被认为起源于桡神经。https://thejns.org/doi/10.3171/CASE25484 。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/8886fa6769d9/CASE25484_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/0105d622df8b/CASE25484_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/641687e0d494/CASE25484_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/8886fa6769d9/CASE25484_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/0105d622df8b/CASE25484_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/641687e0d494/CASE25484_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc9f/12400844/8886fa6769d9/CASE25484_figure_3.jpg

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本文引用的文献

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Ulnar Nerve Innervation to Triceps: A Cadaveric Study and a Technical Note on Partial Triceps to Biceps Transfer.尺神经对肱三头肌的支配:一项尸体研究及肱三头肌部分转位至肱二头肌的技术说明
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Surgical Implications of Innervation Pattern of the Triceps Muscle: A Cadaveric Study.肱三头肌神经支配模式的手术意义:一项尸体研究
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Relationship between the ulnar nerve and the branches of the radial nerve to the medial head of the triceps brachii muscle.
尺神经与桡神经至肱三头肌内侧头分支之间的关系。
Clin Anat. 2019 Jan;32(1):137-142. doi: 10.1002/ca.23207. Epub 2018 Dec 3.
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Targeted nerve biopsy: A technique in evolution.靶向神经活检:一项不断发展的技术。
Clin Anat. 2018 Nov;31(8):1200-1204. doi: 10.1002/ca.23195. Epub 2018 Sep 11.
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Co-Innervation of Triceps Brachii Muscle with Variant Branch of Ulnar Nerve.肱三头肌与尺神经变异分支的共同支配
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6
Targeted fascicular biopsy in a patient with prostate cancer.对前列腺癌患者进行靶向束状活检。
Neurosurg Focus. 2018 Jan;44(VideoSuppl1):V7. doi: 10.3171/2018.1.FocusVid.17580.
7
From targeted fascicular biopsy of major nerve to targeted cutaneous nerve biopsy: Implementing clinical anatomy can catalyze a paradigm shift.从主要神经的靶向束状活检到靶向皮神经活检:应用临床解剖学可推动范式转变。
Clin Anat. 2018 Jul;31(5):616-621. doi: 10.1002/ca.23032. Epub 2018 Jan 30.
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Targeted fascicular biopsy of the brachial plexus: rationale and operative technique.臂丛神经靶向束状活检:原理与手术技术
Neurosurg Focus. 2017 Mar;42(3):E9. doi: 10.3171/2017.1.FOCUS16404.
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Targeted fascicular biopsy of the sciatic nerve and its major branches: rationale and operative technique.坐骨神经及其主要分支的靶向束状活检:理论基础与手术技术
Neurosurg Focus. 2015 Sep;39(3):E12. doi: 10.3171/2015.6.FOCUS15213.
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Clin Anat. 2013 Nov;26(8):1028-30. doi: 10.1002/ca.22270. Epub 2013 May 29.