A Case of Localized Amyloidosis of the Brachial Plexus
Localized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the...
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Editorial Office of Journal of Rare Diseases
2022-04-01
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Series: | 罕见病研究 |
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Online Access: | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.02.011 |
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author | SUN Chong LIN Jie ZHENG Yongsheng LIU Bingyou CHEN Lin BAO Yifang ZHAO Chongbo |
author_facet | SUN Chong LIN Jie ZHENG Yongsheng LIU Bingyou CHEN Lin BAO Yifang ZHAO Chongbo |
author_sort | SUN Chong |
collection | DOAJ |
description | Localized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the type of amyloid protein. This paper retrospectively analyzes the clinical and imaging data, auxiliary examinations, histological, and immunohistochemical markers. The patient, a 34-year-old woman, presented with a right neck mass and weakness of the right arm. Brachial plexus magnetic resonance imaging (MRI) showed a tumor-like lesion in the nerve root at C5 and C6 and in upper trunk. Electrophysiological studies revealed damage in the upper trunk of the brachial plexus. Positive staining with Congo red was found in brachial plexus biopsy. Mass spectrometry showed that the type of amyloid protein was AHL(G-λ). The patient underwent nerve graft for treatment. Meanwhile, literature review revealed that the average onset age of localized spinal nerve amyloidosis was 62.4 years old.The radial nerve was the most susceptible, followed by the lumbosacral plexus. Fifty percent of the type of amyloid protein is AL.Until now, no consolidated treatment is available. Here, we summarize the clinical characteristics of localized peripheral neuropathy amyloidosis in order to raise the awareness of the disease. |
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format | Article |
id | doaj.art-b10fa29e102f4634bed44598cc7d3ae3 |
institution | Directory Open Access Journal |
issn | 2097-0501 |
language | zho |
last_indexed | 2024-03-08T17:52:25Z |
publishDate | 2022-04-01 |
publisher | Editorial Office of Journal of Rare Diseases |
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series | 罕见病研究 |
spelling | doaj.art-b10fa29e102f4634bed44598cc7d3ae32024-01-02T07:13:52ZzhoEditorial Office of Journal of Rare Diseases罕见病研究2097-05012022-04-011217818210.12376/j.issn.2097-0501.2022.02.011A Case of Localized Amyloidosis of the Brachial PlexusSUN Chong0LIN Jie1ZHENG Yongsheng2LIU Bingyou3CHEN Lin4BAO Yifang5ZHAO Chongbo6Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of the Hand Surgery, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of Radiology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaDepartment of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, ChinaLocalized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the type of amyloid protein. This paper retrospectively analyzes the clinical and imaging data, auxiliary examinations, histological, and immunohistochemical markers. The patient, a 34-year-old woman, presented with a right neck mass and weakness of the right arm. Brachial plexus magnetic resonance imaging (MRI) showed a tumor-like lesion in the nerve root at C5 and C6 and in upper trunk. Electrophysiological studies revealed damage in the upper trunk of the brachial plexus. Positive staining with Congo red was found in brachial plexus biopsy. Mass spectrometry showed that the type of amyloid protein was AHL(G-λ). The patient underwent nerve graft for treatment. Meanwhile, literature review revealed that the average onset age of localized spinal nerve amyloidosis was 62.4 years old.The radial nerve was the most susceptible, followed by the lumbosacral plexus. Fifty percent of the type of amyloid protein is AL.Until now, no consolidated treatment is available. Here, we summarize the clinical characteristics of localized peripheral neuropathy amyloidosis in order to raise the awareness of the disease.https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.02.011localized amyloidosisbrachial plexusmass spectrometry |
spellingShingle | SUN Chong LIN Jie ZHENG Yongsheng LIU Bingyou CHEN Lin BAO Yifang ZHAO Chongbo A Case of Localized Amyloidosis of the Brachial Plexus 罕见病研究 localized amyloidosis brachial plexus mass spectrometry |
title | A Case of Localized Amyloidosis of the Brachial Plexus |
title_full | A Case of Localized Amyloidosis of the Brachial Plexus |
title_fullStr | A Case of Localized Amyloidosis of the Brachial Plexus |
title_full_unstemmed | A Case of Localized Amyloidosis of the Brachial Plexus |
title_short | A Case of Localized Amyloidosis of the Brachial Plexus |
title_sort | case of localized amyloidosis of the brachial plexus |
topic | localized amyloidosis brachial plexus mass spectrometry |
url | https://jrd.chard.org.cn/article/doi/10.12376/j.issn.2097-0501.2022.02.011 |
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