Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A 39 years old woman responding to modafinil with bilateral hypothalamic lesion associated with hyperthermia and hypersomnia: a case report

Naoko Saito, M.D.1), Masato Inoue, M.D.2), Kanehiro Hasuo, M.D., Ph.D.3), Takashi Kanbayashi, M.D., Ph.D.4), Shigeo Murayama, M.D., Ph.D.5) and Sosuke Takeuchi, M.D., Ph.D.1)

1)Department of Neurology, National Center of Grobal Health and Medicine
2)Department of Neurosurgery, National Center of Grobal Health and Medicine
3)Department of Radiology, National Center of Grobal Health and Medicine
4)Department of Psychiatry, Akita University School of Medicine
5)Department of Neuropathology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology

A 39 years old woman was admitted to our hospital with a status epilepticus, with high fever of 41°C. Magnetic resonance Imaging (MRI) revealed high signal intensities of both sides of thalami and hypothalami in T2 weighted and fluid attenuated inversion recovery (FLAIR) images. A needle biopsy of the thalamic lesion was consistent with neuromyelitis optica spectrum disorder although her serum antibody to aquaporin-4 was negative. The level of orexin in celebrospinal fluid (CSF) was reduced. She presented hypersomnia, which didn't improve even after intravenous methylprednisolone 1 g daily for 3 days. Administration of oral modafinil extended her waking time. There is a number of reports about neuromyelitis optica (NMO) with hypothalamic lesions. We report this case as important suggestion of treatment of thse cases.
Full Text of this Article in Japanese PDF (7521K)

(CLINICA NEUROL, 54: 550|555, 2014)
key words: hypothalamic lesions, neuromyelitis optica spectrum disorder, hyperthermia, hypersomnia, modafinil

(Received: 28-Dec-12)