平山病:报告1例
Q4 Medicine
引用次数: 0
摘要
The patient, a 28 year old male, was admitted to our outpatient clinic on February 19, 2016 due to progressive weakness of the distal right upper limb with muscle atrophy for 5 years. 5 years ago, the patient had no obvious cause of weakness in the right hand fingers, which worsened during cold weather. Gradually, the right hand fingers became clumsy and unable to move together, and fine movements such as threading needles and wires were significantly restricted. The symptoms persisted without significant relief, accompanied by atrophy of the right hand muscles, no obvious jumping sensation, normal movement of other limbs, no pain, numbness or other sensory abnormalities, no speech disorders, swallowing difficulties, etc. Since the onset of the disease, the patient's mental state, sleep, and diet have been moderate, with no significant abnormalities in bowel and bowel movements, and no significant changes in weight. There is no special history in the past, personal history, or family history. The individual reported significant height growth between the ages of 15 and 18 (specific details are not available). Physical examination after admission: The patient's body temperature is 36.7 ° C, pulse rate is 71 times/min, respiration is 16 times/min, blood pressure is 115/65 mm Hg (1 mm Hg=0.133 kPa); Clear consciousness, fluent language, with equal and round pupils on both sides, with a diameter of approximately 3 mm. No obvious abnormalities were found in the nervous system during physical examination; Atrophy of the thenar and interosseous muscles in the right hand, no obvious muscle atrophy in the left hand, and tremors in the hand muscles can be observed when extending the hands. The distal muscle strength of the right upper limb is 3+to 4 levels, and the muscle tension is reduced. The proximal and left limb muscle strength is 5 levels, and the muscle tension is normal. The radial periosteal reflex, biceps reflex, and triceps reflex in the right upper limb are strongly positive, while the left upper limb is positive. Both Hoffmann and Babinski signs are negative, No obvious abnormalities were observed in the ataxia and deep and shallow sensations, and the meningeal stimulation sign was negative. All indicators tested in the laboratory are within the normal range. Imaging examination: Cervical MRI (February 24, 2016) showed mild thinning of the C6-7 level spinal cord with irregular abnormal signals, forward displacement of the posterior wall of the dural sac, and widening of the dorsal epidural space (Figure 1). No obvious abnormalities were found on the MRI of the head. Electrophysiological examination: Electromyography (February 25, 2016) showed neurogenic damage in both upper limbs, mainly chronic damage, involving bilateral C7-T1 innervated muscles, especially on the right side. The first consideration was damage to the anterior horn cells of the spinal cord (Figure 2). The clinical diagnosis is Hirayama disease. Vitamin B12 25 administered μ G/d and vitamin B1 5 mg/d oral nutrition for nerves, as well as neck support immobilization to reduce head bending and neck flexion movements. Outpatient follow-up, follow-up 6 months after treatment, no significant improvement in symptoms, and no progress in muscle atrophy.本文章由计算机程序翻译，如有差异，请以英文原文为准。
Hirayama's disease: one case report
患者 男性,28 岁,因渐进性右上肢远端无力 伴肌萎缩 5 年,于 2016 年 2 月 19 日至我院门诊就 诊。患者 5 年前无明显诱因出现右手手指无力,寒 冷时加重,逐渐出现右手手指活动笨拙、不能并拢, 穿针引线等精细活动明显受限,症状持续无明显缓 解,伴右手肌萎缩,无明显肉跳感,其他肢体活动正 常,无疼痛、麻木等感觉异常,无言语障碍、吞咽困 难等。患者自发病以来,精神、睡眠、饮食尚可,大 小便无明显异常,体重无明显改变。既往史、个人 史及家族史均无特殊,自诉 15 ~ 18 岁身高生长显著 (具体不详)。入院后体格检查:患者体温 36.7 °C, 脉搏 71 次/min,呼吸 16 次/min,血压 115/65 mm Hg (1 mm Hg = 0.133 kPa);神志清楚,语言流利,双侧 瞳孔等大、等圆,直径约 3 mm,神经系统查体未见明 显异常;右手大小鱼际肌和骨间肌萎缩,左手未见 明显肌萎缩,伸展双手可见手部肌肉震颤,右上肢 远端肌力 3+ ~ 4 级、肌张力降低,右上肢近端和左侧 肢体肌力 5 级、肌张力均正常,右上肢桡骨骨膜反 射、肱二头肌反射和肱三头肌反射强阳性,左上肢 阳性,双侧 Hoffmann 征和 Babinski 征阴性,共济运动 和深浅感觉未见明显异常,脑膜刺激征阴性。实验 室检查各项指标均于正常值范围。影像学检查:颈 椎 MRI(2016 年 2 月 24 日)显示,C6 ~ 7 水平脊髓轻度 变细伴不规则异常信号,硬脊膜囊后壁向前移位, 背侧硬脊膜外间隙增宽(图 1)。头部 MRI 未见明显 异常。电生理学检查:肌电图(2016 年 2 月 25 日)显 示,双上肢呈神经源性损害,以慢性损害为主,累及 双侧 C7 ~ T1支配肌,尤以右侧显著,首先考虑脊髓前 角细胞损害(图 2)。临床诊断为平山病。予以维生 素 B12 25 μg/d 和维生素 B1 5 mg/d 口服营养神经,以 及颈托制动,减少低头屈颈动作。门诊随访,治疗 后 6 个月复诊,症状未见明显改善,肌萎缩无进展。
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