[Concurrence of myasthenia gravis, polymyositis, thyroiditis and eosinophilia in a patient with type B1 thymoma].
We presented a 43-year-old Japanese woman who acutely developed weakness of all extremities and difficulty in swallowing and drooping of eyelids, characterized by easy fatigability at the end of December, 2005. On general physical examination, she had moderate goiter. No cervical lymphadenopathy, cardiac murmur, or skin rash was noted. Neurologically, she had blepharoptosis, more on the right, only in the upright position with easy fatigability and marked weakness in the neck flexor, trunk, and all limb muscles much more proximally than distally. She had neither muscular atrophy nor upper motor neuron sign. Laboratory data showed slight leukocytosis with eosinophilia (up to 31%), and serum creatine kinase was markedly increased to over 2,000 IU/l. TSH receptor antibody (11.9%) and anti-acetylcholine receptor antibody (46.6 nmol/L) were also increased. Edrophonium test was positive. Electrophysiologically, muscle evoked potentials by repetitive motor nerve stimulation showed 13% and 50% waning in abductor pollicis brevis and deltoid muscle, respectively, at low frequency and no waxing at high frequency. Needle EMG showed fibrillation potentials and positive sharp waves in proximal muscles. Polymyositis was diagnosed by muscle biopsy which showed infiltration of lymphocytes in the endomysium and around non-necrotic muscle fibers. Upper arm muscle MRI showed multifocal high signal intensity lesions on T2-weighted images which were likely related to myositis. This finding is atypical for polymyositis. X-ray and CT of chest showed a mass lesion in the left pulmonary hilum, which was histologically diagnosed as type B1 thymoma. Thus, the present case had myasthenia gravis, polymyositis, thyroidititis and eosinophilia associated with type B1 thymoma. After the thymectomy, corticosteroid administration and immunoadsorption therapy, clinical symptoms and all laboratory abnormalities markedly improved.
Inoue
Manabu
M
Department of Neurology, Ijinkai Takeda General Hospital.
Kojima
Yasuhiro
Y
Shinde
Akiyo
A
Satoi
Hitoshi
H
Makino
Fumi
F
Kanda
Masutarou
M
Shibasaki
Hiroshi
H
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D004802:Eosinophilia / Q000209:etiology*; D005260:Female; D006801:Humans; D008775:Methylprednisolone / Q000008:administration & dosage; D009157:Myasthenia Gravis / Q000209:etiology*; D017285:Polymyositis / Q000209:etiology*; D011239:Prednisolone / Q000008:administration & dosage; D013934:Thymectomy; D013945:Thymoma / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000601:surgery; D013953:Thymus Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000601:surgery; D013966:Thyroiditis / Q000209:etiology*
D000305:Adrenal Cortex Hormones; D011239:Prednisolone; D008775:Methylprednisolone