Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Efficacy of high-dose steroid pulse therapy for anti-galactocerebroside antibody-positive combined central and peripheral demyelination

Masashi Hoshino, M.D.1), Yu Suzuki, M.D., Ph.D.1), Hisanao Akiyama, M.D., Ph.D.1), Kouji Yamada, M.D., Ph.D.1), Sayuri Shima, M.D., Ph.D.2), Tatsuro Mutoh, M.D., Ph.D.2) and Yasuhiro Hasegawa, M.D., Ph.D.1)

1)Department of Internal Medicine, Division of Neurology, St Marianna University School of Medicine
2)Department of Neurology, Fujita Health University School of Medicine

A 59-year-old man had been admitted to another hospital because of diplopia and thirst at the beginning of March and was diagnosed with diabetic ketoacidosis. He was referred to our hospital because he had limb weakness, dysarthria, and bilateral sensory impairment of the upper limbs, which worsened rapidly from the middle of March, although plasma glucose had been well controlled after the initiation of insulin therapy in the previous hospital. Contrast spinal MRI in our hospital revealed hyperintense lesions at the level of C4 to C5 and T10. The level of myelin basic protein was high (1,260 pg/ml) in the cerebrospinal fluid and serum anti-neurofascin antibody was negative. Nerve conduction study showed typical findings of demyelination at least 2 regions. Although anti-neurofascin antibody was negative, he was diagnosed with combined central and peripheral demyelination (CCPD) based on these clinical findings. After the repeated methylprednisolone pulse therapy for five times, the hyperintense lesions of the spinal cord disappeared gradually. He was bedridden at the beginning of his hospitalization but could ambulate with a cane on discharge 2 months after the admission. Then we received the result of anti-galactocerebroside antibody test as positive. This case suggested that high-dose steroid pulse therapy is safe and may be effective for anti-galactocerebroside antibody-positive CCPD.
Full Text of this Article in Japanese PDF (855K)

(CLINICA NEUROL, 57: 747|752, 2017)
key words: combined central and peripheral demyelination, the anti-neurofascin antibody, the anti-galactocerebroside antibody, high dose steroid pulse treatment

(Received: 27-Oct-16)