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A 21-year-old man presents with weakness and atrophy of the muscles of his right hand and forearm, which he first noticed at age 18 and has progressedvery gradually since then. He has no sensory symptoms and no symptoms inany other limb. Neurologic examination confirms the right forearm and intrinsic hand muscle weakness, sparing the brachioradialis, with no motor abnormality anywhere else in the body. Sensation and deep tendon reflexes are normal. Nerve conduction studies show normal sensory responses and moderate reduction of the right median and ulnar compound muscle action potential amplitudes with normal latencies. Needle EMG shows evidence of chronic denervation in the muscles that are clinically affected. A flexion cervical MRI is most likely to show which of the following abnormalities?
Explanation
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This patient?s history and examination are typical of monomelic amyotrophy (Hirayama disease), a gradually progressive syndrome that results in unilateral or asymmetric weakness and atrophy of forearm and hand muscles, which derive their innervation from the C7, C8, and T1 nerve roots. The proposed mechanism is a tightened dural sac that cannot accommodate the natural lengthening of the spinal cord with flexion, resulting in repetitive compression of the arterial and venous microcirculation, leading to progressive anterior horn cell damage. Flexion cervical MRI shows loss of dural attachment with forward displacement, posterior epidural flow void, and asymmetric spinal cord atrophy
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Reference
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Page 62,Tavee JO, Levin KH. Myelopathy due to degenerative and structural spine diseases. Continuum (Minneap Minn). 2015 Feb;21(1 Spinal Cord Disorders):52-66. doi: 10.1212/01.CON.0000461084.71618.35. PMID: 25651217.
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Option 1
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Is Correct (Option 1):
Forward displacement of the posterior dura
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Option 2
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Is Correct (Option 2):
Increased signal in the right corticospinal tract
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Option 3
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Is Correct (Option 3):
Intervertebral disk herniation at C3-C4
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Option 4
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Is Correct (Option 4):
Intervertebral disk herniation at C7-T1
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