- 期刊
Distal Acquired Demyelinating Symmetric (DADS) Neuropathy without Gammopathy: A Case Report and Comprehensive Review of the Literature
未合併丙種球蛋白病變之遠端後天脫髓鞘對稱性神經病變：一個案報告及文獻回顧
摘要
遠端後天脫髓鞘對稱性神經病變是慢性發炎性脫髓鞘多發性神經病變的一種亞型，此疾病有特殊的臨床與電生理特徵。其臨床病程通常比典型的慢性發炎性脫髓鞘多發性神經病變進展慢且常合併丙種球蛋白病變。在此，我們報告一個罕見的未合併丙種球蛋白病變之遠端後天脫髓鞘對稱性神經病變個案。患者是來自南非的26歲白人女性，因為連續數週的四肢末梢麻木與輕微無力來我們神經科門診求診。神經傳導速度檢查呈現脫髓鞘型感覺運動多發性神經病變併末端潛時指數明顯降低。腦脊髓液檢查呈現蛋白質上升但白血球無顯著上升。住院中，包括血液學、感染、自體免疫、生化、癌症、球蛋白、內分泌等檢查均正常。用類固醇治療，無明顯反應。靜脈注射免疫球蛋白，每日每公斤體重0.4克，連續5日後，症狀顯著改善。免疫球蛋白的療效約維持6個月。之後類似的症狀復發且逐漸進展。患者再次入院。注射大劑量類固醇但無明顯療效，沒多久後她返回家鄉南非。根據此患者特徵性的臨床症狀與神經傳導速度檢查結果，我們診斷此個案為遠端後天脫髓鞘對稱性神經病變。神經傳導速度檢查呈現明顯的脫髓鞘變化與末端潛時指數明顯降低以及對稱的感覺症狀和輕微的、僅限於末端的無力，是此種可治療的罕見神經病變的主要特徵。
並列摘要
Distal acquired demyelinating symmetric (DADS) neuropathy is a subtype of chronic inflammatory demyelinating polyneuropathy (CIDP) with characteristic clinical and electrophysiological features. It usually progresses more slowly than typical CIDP and is frequently associated with an IgM paraprotein. We report a rare case of DADS neuropathy without a paraprotein. A 26-year-old Caucasian woman from South Africa visited our neurological clinic because of subacute symmetric numbness and mild weakness of distal limbs. Nerve conductive study showed sensorimotor polyneuropathy, demyelinating type with a markedly reduced terminal latency index. CSF examination showed elevated protein (148 mg/dL) without significant pleocytosis. Detailed examinations following hospitalization, including hematology, infection, autoimmune, biochemistry, malignancy, paraprotein and endocrine studies, were unremarkable. The patient responded poorly to corticosteroid treatment, but intravenous immunoglobulin (IVIG) 0.4 g/kg/day for five days improved her symptoms. The effect of IVIG lasted for about six months, followed by slow progression of numbness and mild weakness in distal limbs. The patient received steroid pulse therapy but in vain; she then returned to South Africa. She was diagnosed with DADS neuropathy from the characteristic clinical and electrophysiological features. Striking demyelination with a markedly reduced terminal latency index identified by nerve conduction study and symmetric sensory symptoms with minimal distal weakness are the main features of this rare treatable neuropathy.
延伸閱讀
- 吳欣宜、周正亮、鄭舜平、劉作仁、詹瑞棋（2004）。髓鞘糖蛋白抗體神經病變：病例報告。台灣復健醫學雜誌，32(1)，47-53。https://doi.org/10.6315/2004.32(1)07
- Yang, Y. W., Liu, C. H., Tsai, C. H., Lee, C. C., & Jou, S. B. (2004). 多發性後天去髓鞘感覺及運動神經病變：病例報告及文獻探討. Acta Neurologica Taiwanica, 13(1), 24-28. https://doi.org/10.29819/ANT.200403.0007
- Yu, Yu-Ta、Liu, Che-Wei、Lin, Hsi-Ting（2022）。或能以質子密度脂肪抑制核磁共振影像早期發現與地諾單抗相關連非典型股骨骨折之前驅骨髓水腫：一則案例報告。輔仁醫學期刊，20(4)，33-39。https://doi.org/10.53106/181020932022122004004
- Chang, H. Y., Huang, Y. C., Huang, K. M., & Cheng, W. (1998). Lhermitte-Duclos氏疾病（異常發育的神經節細胞瘤）在磁振造影上的影像特徵：一病例報告及文獻回溯. 中華放射線醫學雜誌, 23(5), 193-196. https://www.airitilibrary.com/Article/Detail?DocID=10188940-199810-23-5-193-196-a
- Lin, F. C., Lin, L. C., Su, C. C., Lin, K. L., & Que, J. (2003). 篩骨腔小細胞神經內分泌癌：病例報告及文獻回顧. 放射治療與腫瘤學, 10(3), 175-179. https://doi.org/10.6316/TRO/200310(3)175