Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of demyelinating polyneuropathy associated with anti-myelin-associated glycoprotein antibodies with progressive quadriparesis and respiratory failure

Natsuko Yuki, M.D.1), Akira Yoshioka, M.D., Ph.D.2), Reiko Horio, M.D.1), Takuma Ohmichi, M.D.1), Rei Yasuda, M.D.1) and Nobuyuki Oka, M.D., Ph.D.3)

1)Department of Neurology, NHO Maizuru Medical Center
2)Clinical Research, NHO Maizuru Medical Center
3)Department of Neurology, NHO Minami Kyoto Hospital

A 79-year-old man was admitted due to progressive weakness of both hands for two and a half years. Neurologically, he presented with weakness of the upper limbs, predominantly in the left distal portion, and hypoactive deep tendon reflexes. Nerve conduction studies were consistent with a motor and sensory demyelinating neuropathy. Sural nerve biopsy revealed a decrease of myelinated fibers with a predominance of larger diameter fibers. Widening of myelin lamellae and uncompacted myelin were detected on electron microscopy. Laboratory examinations showed IgM-kappatype M-protein and anti-myelin-associated glycoprotein (MAG) antibody. He was diagnosed as having anti-MAG-associated demyelinating neuropathy based on the laboratory, electrophysiological, and pathological findings. While no bulbar symptoms or upper motor neuron signs were shown, the patient developed quadriparesis and respiratory failure after three years. Although anti-MAG-associated demyelinating neuropathy is usually characterized by sensory symptoms, particularly sensory ataxia, the present case indicates that motor symptoms such as quadriparesis and respiratory failure can be among the clinical manifestations of anti-MAG-associated demyelinating neuropathy.
Full Text of this Article in Japanese PDF (2244K)

(CLINICA NEUROL, 54: 671|674, 2014)
key words: anti-myelin-associated glycoprotein antibody, neuropathy, quadriparesis, respiratory failure

(Received: 2-Oct-13)