多发性运动神经病

N.C. Taghiyeva
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During the study, tendon reflexes in the upper and lower extremities were reduced. To clarify the diagnosis, an ENMG examination of the peripheral nerves of the upper and lower extremities was prescribed. A patient on ENMG of the tibial nerve has signs of demyelination (decrease in the speed of propagation of excitation (ERV) to 33.2 m/s). In this regard, to differentiate from other polyneuropathies, the patient underwent ultrasound of the tibial nerve throughout its entire length. At the same time, segmental expansion of the tibial nerve and a decrease in echogenicity with obliteration of the cable structure due to the predominance of the type 3 interstitial component are detected on the right – asymmetric local structural changes. On the left – an increase in echogenicity due to the predominance of thickening of the interstitial component with an erased cable structure, thinning of the fascicular component - an asymmetric local structural change. Based on the nature of the lesion, the patient was diagnosed with multifocal motor neuropathy. A comprehensive examination of the patient, including, along with traditional ENMG and ultrasound, nerves, made it possible to reduce the time for making a diagnosis and start pathogenetic treatment of the patient in a timely manner. Timely prescribed immunoglobulin therapy made it possible to avoid further deterioration of the patient's condition and the risk of his disability. Discussion. The combination of clinical signs that meet the criteria for the disease,developed by the recommendations of the European Federation of Neurological Societies /Society of Peripheral Nerves (EFNS / PNS, 2010) for the study of MMN, as well as the results of instrumental diagnostic studies (ENMG and ultrasound), makes the diagnosis of multifocal motor neuropathy indisputable. Conclusion. 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引用次数: 0

摘要

简介多灶性运动神经病(MMN)是一种慢性自身免疫性多发性神经病,患者的运动纤维会发生孤立性病变,出现不对称的远端瘫痪,主要累及手部。该病非常罕见:全世界 MMN 的发病率为每 10 万人中 0.6-2.0 例。男性发病率是女性的 3-4 倍。有儿童发病的孤立病例。阿塞拜疆共和国尚未对成年人口中 MMN 的发病率进行流行病学研究。本研究的目的强调疑似多灶性运动神经病鉴别诊断系列的特征。病例介绍。患者 A.,29 岁,因主诉缓慢进行性肌无力、麻木、麻痹而入院。在检查过程中,上肢和下肢的腱反射减弱。为了明确诊断,医生要求对患者的上下肢周围神经进行 ENMG 检查。接受胫神经 ENMG 检查的患者有脱髓鞘迹象(兴奋传播速度(ERV)降至 33.2 米/秒)。为此,为了与其他多发性神经病相鉴别,患者接受了胫神经全长的超声检查。与此同时,右侧发现胫神经节段性扩张,回声减弱,3 型间质成分占主导地位导致索状结构消失--局部结构发生不对称变化。左侧--由于间质成分增厚和索状结构消失,回声增强,筋膜成分变薄--不对称的局部结构变化。根据病变的性质,患者被诊断为多灶性运动神经病变。对患者进行全面检查,包括传统的 ENMG 和超声波检查神经,可以缩短诊断时间,并及时开始对患者进行病理治疗。及时的免疫球蛋白治疗避免了患者病情的进一步恶化和致残的风险。讨论根据欧洲神经学会联合会/周围神经学会(EFNS/PNS,2010 年)对多灶性运动神经病研究的建议,临床症状符合该病的标准,结合仪器诊断研究(ENMG 和超声波)的结果,多灶性运动神经病的诊断无可争议。结论多灶性运动神经病是一种获得性自身免疫性脱髓鞘多发性神经病,伴有神经元髓鞘受损,进而导致从大脑到肌肉的运动神经神经冲动传导障碍。对于 ENMG 结果与电生理学结果不完全符合 MMN 标准(EFNS/PNS,2010 年)的疑似 MMN 患者,建议在不注射造影剂的情况下进行臂丛磁共振成像(磁感应强度大于 1 T,以标准 T1 和 T2 模式以及 STIR 和 DTI 模式成像),以提高诊断的可靠性。在本病例中,患者的主诉是神经症状。尽管这种疾病非常罕见,神经系统受损的病例更是罕见(男性发病率是女性的 3-4 倍),但神经科医生仍应警惕 MMN,这有助于及时诊断和指定病因治疗。
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MULTIFOCAL MOTOR NEUROPATHY
Introduction. Multifocal motor neuropathy (MMN) is a chronic autoimmune, multiple neuropathy with an isolated lesion of motor fibers, the development of asymmetric distal paresis with a predominant involvement of the hands. The disease is rare: the prevalence of MMN in the world is from 0.6-2.0 per 100 000 people. The disease is diagnosed 3-4 times more often in men. Isolated cases of the development of the disease in children are described. Epidemiological studies on the prevalence of MMN among the adult population in the Republic of Azerbaijan have not been conducted. The aim of the study. To highlight the features of the differential diagnostic series for suspected multifocal motor neuropathy. Case presentation. Patient A., aged 29, was admitted to our clinic with complaints of slowly progressive muscle weakness, numbness, parasthesia. During the study, tendon reflexes in the upper and lower extremities were reduced. To clarify the diagnosis, an ENMG examination of the peripheral nerves of the upper and lower extremities was prescribed. A patient on ENMG of the tibial nerve has signs of demyelination (decrease in the speed of propagation of excitation (ERV) to 33.2 m/s). In this regard, to differentiate from other polyneuropathies, the patient underwent ultrasound of the tibial nerve throughout its entire length. At the same time, segmental expansion of the tibial nerve and a decrease in echogenicity with obliteration of the cable structure due to the predominance of the type 3 interstitial component are detected on the right – asymmetric local structural changes. On the left – an increase in echogenicity due to the predominance of thickening of the interstitial component with an erased cable structure, thinning of the fascicular component - an asymmetric local structural change. Based on the nature of the lesion, the patient was diagnosed with multifocal motor neuropathy. A comprehensive examination of the patient, including, along with traditional ENMG and ultrasound, nerves, made it possible to reduce the time for making a diagnosis and start pathogenetic treatment of the patient in a timely manner. Timely prescribed immunoglobulin therapy made it possible to avoid further deterioration of the patient's condition and the risk of his disability. Discussion. The combination of clinical signs that meet the criteria for the disease,developed by the recommendations of the European Federation of Neurological Societies /Society of Peripheral Nerves (EFNS / PNS, 2010) for the study of MMN, as well as the results of instrumental diagnostic studies (ENMG and ultrasound), makes the diagnosis of multifocal motor neuropathy indisputable. Conclusion. Multifocal motor neuropathy is classified as an acquired autoimmune demyelinating multiple neuropathy, accompanied by damage to the myelin sheath of neurons, which in turn causes a violation of the conduction of nerve impulses along the motor nerves from the brain to the muscles. MRI of the brachial plexus (magnetic induction value > 1 T, imaging in standard T1 and T2 modes, as well as in STIR and DTI modes) without contrast agent injection is recommended for those patients with suspected MMN in whom ENMG results do not fully correspond to electrophysiological results MMN criteria (EFNS/PNS, 2010), in order to improve the reliability of the diagnosis. In the presented case, the patient's complaints were due to neurological symptoms. Despite the rarity of the disease and even rarer cases of damage to the nervous system (3-4 times more often in men), a neurologist should be wary of MMN, which contributes to timely diagnosis and the appointment of pathogenetic therapy.
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