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FUS 突变患者中多发性硬化症与肌萎缩侧索硬化症的共病:一例报告

Co-Occurrence of Multiple Sclerosis and Amyotrophic Lateral Sclerosis in an FUS-Mutated Patient: A Case Report.

作者信息

Fiondella Luigi, Cavallieri Francesco, Canali Elena, Cabboi Maria Paola, Marti Alessandro, Sireci Francesca, Fiocchi Alena, Montanari Gloria, Montepietra Sara, Valzania Franco

机构信息

Neurology Unit, Neuromotor and Rehabilitation Department, AUSL-IRCCS of Reggio Emilia, 42123 Reggio Emilia, Italy.

Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy.

出版信息

Brain Sci. 2022 Apr 21;12(5):531. doi: 10.3390/brainsci12050531.

Abstract

A concomitant presentation of relapsing remitting multiple sclerosis (RRMS) and amyotrophic lateral sclerosis (ALS) is quite rare. However, a review of the literature showed an increased co-occurrence of both diseases, including in genetically determined cases. We report the case of a 49-year-old woman with a history of RRMS who developed a progressive subacute loss of strength in her left arm. The patient’s father died from ALS, and her paternal uncle had Parkinson’s disease. Brain and cervical MRIs were performed, and new demyelinating lesions were excluded. Electromyography (EMG) of the upper limbs showed fibrillations and fasciculations in distal muscles of both arms. In the following months, the patient presented a progressive loss of strength in the proximal and distal muscles of the right arm and hyperreflexia in the lower limbs. EMG and central motor conduction were consistent with ALS. A genetic test was carried out, revealing a mutation in the FUS gene (exon 15; c. 1562 G>A). To our knowledge, the co-occurrence of MS and ALS in patients with FUS mutation is extremely rare. We hypothesize a common pathway for both diseases based on the possibility of a shared oligodendroglial dysfunction due to FUS mutation.

摘要

复发缓解型多发性硬化症(RRMS)与肌萎缩侧索硬化症(ALS)同时出现的情况相当罕见。然而,文献回顾显示这两种疾病的共现情况有所增加,包括在基因决定的病例中。我们报告了一例49岁有RRMS病史的女性,其左臂出现进行性亚急性肌力丧失。患者的父亲死于ALS,她的叔祖父患有帕金森病。进行了脑部和颈部MRI检查,排除了新的脱髓鞘病变。上肢肌电图(EMG)显示双臂远端肌肉有纤颤和肌束震颤。在接下来的几个月里,患者右臂近端和远端肌肉出现进行性肌力丧失,下肢反射亢进。EMG和中枢运动传导与ALS相符。进行了基因检测,发现FUS基因(第15外显子;c. 1562 G>A)存在突变。据我们所知,FUS突变患者中MS和ALS同时出现的情况极为罕见。基于FUS突变导致少突胶质细胞功能障碍共享的可能性,我们推测这两种疾病存在共同的发病途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b56/9139033/2334cdc51c37/brainsci-12-00531-g001.jpg

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