Neuromyelitis optica with intraspinal expansion of Schwann cell remyelination

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Hayato IkotaYoichi Nakazato

Abstract

We report a case of neuromyelitis optica (NMO) with an unusual pattern of remyelination in the spinal cord. A Japanese woman complained of pain and numbness in the left thumb at the age of 36 years. She mainly presented with optic and spinal symptoms and was initially diagnosed as multiple sclerosis (MS). Her bilateral eyesight decreased, which led to light perception only in the right eye. She became unable to walk without a wheelchair. In spite of steroid pulse therapy, plasma exchange therapy and immunosuppressive therapy, her symptoms gradually worsened. After 33 years of a relapsing-remitting course, she died of septic urinary tract infection at the age of 69 years. Autopsy revealed prominent demyelination in the optic tract and the spinal cord. The optic nerve showed extensive demyelination accompanied by axon depletion. The spinal cord lesions were found in C8 to L2 level (contiguous 15 segments), especially Th5 to Th11 level. The thoracic spinal cord showed extensive remyelination spreading from the entry zone of peripheral nerves to the central portion. Regenerative myelin showed immunopositivity for Schwann/2E, a marker of Schwann cells and myelin of the peripheral nervous system. Expressions of glial fibrillary acidi...Continue Reading

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Citations

Mar 1, 2012·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Aya SuzukiYoichi Nakazato
Feb 16, 2018·Journal of Neuroscience Research·Ivana BjelobabaIrena Lavrnja
May 9, 2019·Physiological Reviews·Christine StadelmannMikael Simons
Dec 19, 2013·Brain Pathology·Claudia F LucchinettiTatsuro Misu
Oct 22, 2016·Acta Neuropathologica·Hans Lassmann, Monika Bradl
Jun 3, 2021·International Journal of Molecular Sciences·Ulises Gómez-PinedoJorge Matías-Guiu

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