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• Vol.59 No.3
• CLINICA NEUROL, 59: 139−143, 2019
• Vol.59 No.3 contents

Case Report

A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease

Kiyoaki Takeda, M.D.¹⁾²⁾, Kenji Yoshimura, M.D.¹⁾³⁾, Rie Kanki, M.D., Ph.D.¹⁾⁴⁾, Hirokazu Morihata, M.D., Ph.D.¹⁾ and Satoshi Nakano, M.D., Ph.D.¹⁾

¹⁾Department of Neurology, Osaka City General Hospital
²⁾Present address: Department of Neurology, National Hospital Organization Kyoto Medical Center
³⁾Present address: Department of Neurology, Kyoto University, Graduate School of Medicine
⁴⁾Present address: Department of Neurology, Nishinomiya Kyoritsu Neurosurgical Hospital

A 52-year old woman first noted dysphagia four months before admission followed by dysarthria two months later. She then developed weakness of all limbs and became unable to walk. All these symptoms, associated with tongue atrophy, slowly progressed, leading to the initial clinical impression of a motor neuron disease, although her nerve conduction study and electromyography showed no abnormalities. Her brain MRI with T₂ weighted/diffusion weighted image (DWI)/fluid attenuated inversion recovery (FLAIR) revealed a high signal lesion located at dorsal medulla oblongata. She proved positive for anti-aquaporin 4 antibody, which confirmed the diagnosis of neuromyelitis optica spectrum disorders (NMOSD). We conclude that NMOSD may initially present with progressive bulbar palsy and pyramidal tract disorder over a few months, mimicking a motor neuron disease. Awareness of this atypical presentation helps establish an early diagnosis of this treatable entity.
Full Text of this Article in Japanese PDF (696K)

(CLINICA NEUROL, 59: 139−143, 2019)
key words: neuromyelitis optica spectrum disorders, anti-aquaporin 4 antibody, motor neuron disease, intractable hiccup and nausea, tongue atrophy

(Received: 10-Aug-18)