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.
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