Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of neuromyelitis optica spectrum disorder associated with central pontine and extrapontine myelinolysis preceded by syndrome of inappropriate antidiuretic hormone secretion

Waka Sakai, M.D.1)2), Naoko Matsui, M.D., Ph.D.1), Koji Fujita, M.D., Ph.D.1), Yuishin Izumi, M.D., Ph.D.1), Yoshihiko Nishida, M.D., Ph.D.3), Toshiyuki Takahashi, M.D., Ph.D.4), Takashi Kanbayashi, M.D., Ph.D.5) and Ryuji Kaji, M.D., Ph.D.1)

1)Department of Clinical Neuroscience, The University of Tokushima Graduate School
2)Department of Neurology, Nagasaki-Kawatana Medical Center
3)Department of Neurology, Itsuki Hospital
4)Department of Neurology, Tohoku University School of Medicine
5)Department of Neuropsychiatry, Akita University School of Medicine

A 36-year-old woman complained of general malaise. She presented with hyponatremia and plasma osmotic pressure was lower than urinary osmotic pressure. In addition, serum antidiuretic hormone level was higher than the measurement sensitivity. She was diagnosed with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). She fell into a coma despite correction of serum sodium level. Brain magnetic resonance imaging (MRI) revealed high signal intensities in the cerebral cortex, striatum, thalamus, hypothalamus, midbrain, and pons in fluid-attenuated inversion recovery images. Spinal MRI revealed a longitudinally extending lesion in the cervical cord. Serum sample was positive for anti-aquaporin-4 antibody, supporting the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) combined with central pontine and extrapontine myelinolysis. In patients with NMOSD, the immune reaction can gradually cause destructive changes of the hypothalamus and lead to unstable ADH secretion in the absence of immunomodulatory treatment.
Full Text of this Article in Japanese PDF (3227K)

(CLINICA NEUROL, 54: 556|560, 2014)
key words: neuromyelitis optica spectrum disorder (NMOSD), syndrome of inappropriate antidiuretic hormone secretion (SIADH), central pontine myelinolysis, extrapontine myelinolysis, anti-aquaporin-4 antibody

(Received: 26-Apr-13)