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[A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease].
Rinsho Shinkeigaku 2019; 59(3):139-143RS

Abstract

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.

Authors+Show Affiliations

Department of Neurology, Osaka City General Hospital. Present address: Department of Neurology, National Hospital Organization Kyoto Medical Center.Department of Neurology, Osaka City General Hospital. Present address: Department of Neurology, Kyoto University, Graduate School of Medicine.Department of Neurology, Osaka City General Hospital. Present address: Department of Neurology, Nishinomiya Kyoritsu Neurosurgical Hospital.Department of Neurology, Osaka City General Hospital.Department of Neurology, Osaka City General Hospital.

Pub Type(s)

Case Reports
Journal Article

Language

jpn

PubMed ID

30814445

Citation

Takeda, Kiyoaki, et al. "[A Case of Neuromyelitis Optica Spectrum Disorders, With Slowly Progressive Bulbar Palsy, Mimicking a Motor Neuron Disease]." Rinsho Shinkeigaku = Clinical Neurology, vol. 59, no. 3, 2019, pp. 139-143.
Takeda K, Yoshimura K, Kanki R, et al. [A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease]. Rinsho Shinkeigaku. 2019;59(3):139-143.
Takeda, K., Yoshimura, K., Kanki, R., Morihata, H., & Nakano, S. (2019). [A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease]. Rinsho Shinkeigaku = Clinical Neurology, 59(3), pp. 139-143. doi:10.5692/clinicalneurol.cn-001211.
Takeda K, et al. [A Case of Neuromyelitis Optica Spectrum Disorders, With Slowly Progressive Bulbar Palsy, Mimicking a Motor Neuron Disease]. Rinsho Shinkeigaku. 2019 Mar 28;59(3):139-143. PubMed PMID: 30814445.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [A case of neuromyelitis optica spectrum disorders, with slowly progressive bulbar palsy, mimicking a motor neuron disease]. AU - Takeda,Kiyoaki, AU - Yoshimura,Kenji, AU - Kanki,Rie, AU - Morihata,Hirokazu, AU - Nakano,Satoshi, Y1 - 2019/02/28/ PY - 2019/3/1/pubmed PY - 2019/4/25/medline PY - 2019/3/1/entrez KW - anti-aquaporin 4 antibody KW - intractable hiccup and nausea KW - motor neuron disease KW - neuromyelitis optica spectrum disorders KW - tongue atrophy SP - 139 EP - 143 JF - Rinsho shinkeigaku = Clinical neurology JO - Rinsho Shinkeigaku VL - 59 IS - 3 N2 - 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. SN - 1882-0654 UR - https://www.unboundmedicine.com/medline/citation/30814445/[A_case_of_neuromyelitis_optica_spectrum_disorders_with_slowly_progressive_bulbar_palsy_mimicking_a_motor_neuron_disease]_ L2 - https://dx.doi.org/10.5692/clinicalneurol.cn-001211 DB - PRIME DP - Unbound Medicine ER -