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Approximately 15% of myasthenia gravis (MG) patients do not have any detectable antiacetylcholine receptor (AChR) antibodies and are referred to as ‘seronegative.’ Of these, antibodies against muscle-specific tyrosine kinase (MuSK) are positive in 30–50%.1 2 Tongue muscle atrophy is frequent in MuSK-positive MG.3 The pathophysiology of muscle atrophy is unclear, and it has not yet been reported whether tongue muscle atrophy in MuSK-MG is reversible or not. We herein report a 6-year observation of tongue muscle atrophy with its MRI evaluation in a MuSK-MG patient.
In 2002, a 46-year-old woman was admitted with an 8-month history of progressive neck weakness, hoarseness and dysphagia. These symptoms did not fluctuate throughout the day. The patient had lost her body weight by 14 kg over 8 months. Neurological examination showed equivocal blepharoptosis on the right, prominent nasal voice and dysphagia, and mild atrophy of the tongue. There was moderate weakness in the neck muscles and mild weakness in the limbs. No fasciculations were observed. Tendon reflexes were active in all four limbs.
Serum anti-AChR antibodies were negative. The patient's percentage vital capacity (%VC) was decreased to 62%. An edrophonium test was negative. A repetitive nerve stimulation test showed no significant decremental responses in …
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