Rinsho Shinkeigaku (Clinical Neurology)

Case Report

An autopsied case of postinfectious neuromyelitis optica in an 84-year-old man

Shun'ichi Satoh, M.D., Ph.D.1), Kennichi Hoshi, M.D., Ph.D.1), Masahide Watanabe, M.D., Ph.D.2), Akiyo Hineno, M.D.3), Kiyomitsu Oyanagi, M.D., Ph.D.4) and Hiroyuki Yahikozawa, M.D., Ph.D.1)

1)Department of Neurology, Nagano Red-cross Hospital
2)Department of Pathology, Nagano Red-cross Hospital
3)Department of Neurology, Shinsyu University School of Medicine
4)Division of Neuropathology, Department of Brain Disease Research, Shinsyu University School of Medicine

An 84-year-old man presented with acute bilateral visual impairment 2 weeks after an upper respiratory tract infection. A few days later, he developed left hemiparesis, followed by paraplegia. The brain magnetic resonance imaging (MRI) showed high-intensity lesions in the right cerebellum, pons, left and right corona radiata, and right putamen. The diffusion weighted image also showed these high-intensity lesions. The spinal MRI showed an edematous, longitudinally extensive, cord lesion at the C5-Th6 level of the spine. Intravenous corticosteroid therapy was initiated, but the patient showed mild improvement. Although methylprednisolone pulse therapy was administered 5 times, he continued to present with clinical relapse and died on day 50. Anti-aquaporin-4 (AQP4) antibodies were detected in the patient's serum. Autopsy findings showed necrotic lesions at the spinal cord, brain, and optic chiasma and nerves. An immunohistopathological study showed the loss of AQP4- and glial fibrillary acidic protein (GFAP)-positive cells, with relatively preserved myelin basic protein (MBP)-positive myelin in the necrotic lesions. We diagnosed the patient as having neuromyelitis optica (NMO) because of the seropositivity for anti-AQP4 antibodies and on the basis of above-mentioned other immunohistochemical findings. It is difficult to distinguish NMO from ADEM clinically, when the patient has a preceding infection. NMO should be considered in patients with multifocal lesions in the central nervous system who have prominent myelitis and optic neuritis, irrespective of the postinfectious onset of the lesions and the sex and age of the patient.
Full Text of this Article in Japanese PDF (1201K)

(CLINICA NEUROL, 51: 583|589, 2011)
key words: neuromyelitis optica, aquaporin 4, Devic's disease, postinfectious encephalomyelitis

(Received: 3-Feb-10)