Pseudo-Piano Playing Motions and Nocturnal Hypoventilation in Anti-NMDA Receptor Encephalitis: Response to Prompt Tumor Removal and Immunotherapy

• Uchino Akiko

  Department of Neurology, Kitasato University, School of Medicine, Japan

• Iizuka Takahiro

  Department of Neurology, Kitasato University, School of Medicine, Japan

• Urano Yoshiaki

  Department of Neurology, Kitasato University, School of Medicine, Japan

• Arai Masahide

  Department of Obstetrics and Gynecology, Kitasato University, School of Medicine, Japan

• Hara Atsuko

  Department of Pathology, Kitasato University, School of Medicine, Japan

• Hamada Junichi

  Department of Neurology, Kitasato University, School of Medicine, Japan

• Hirose Ryuichi

  Department of Neurology, Kitasato Institute Medical Center Hospital, Japan

• Dalmau Josep

  Department of Neurology, Division of Neuro-oncology, University of Pennsylvania, School of Medicine, USA

• Mochizuki Hideki

  Department of Neurology, Kitasato University, School of Medicine, Japan

抄録

Tumor resection is recommended in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, however it is often difficult during an early stage of the disease. We report here the efficacy of early tumor removal in a patient with anti-NMDAR encephalitis. This 21-year-old woman was admitted to another hospital with rapidly progressive psychiatric symptoms, a decreased level of consciousness, and seizures. Abdominal CT showed a pelvic mass. On day 1 of admission to our center, she developed hypoventilation requiring mechanical support. She had orofacial dyskinesias with well-coordinated, pseudo-piano playing involuntary finger movements. Based on these clinical features, she was immediately scheduled for tumor resection on day 3. While awaiting surgery, she began to receive high-dose intravenous methylprednisolone. After tumor removal, she received plasma exchange, followed by intravenous immunoglobulin and additional high-dose methylprednisolone. Two weeks after tumor removal, she started following simple commands and progressive improvement, although she remained on mechanical ventilation for 10 weeks due to nocturnal central hypoventilation. Anti-NMDAR antibodies in serum/CSF were detected. Pathological examination showed immature teratoma with foci of infiltrates of B- and T-cells. Early tumor resection with immunotherapy facilitates recovery from this disease, but central hypoventilation may require long mechanical support. Non-jerky elaborate finger movements suggest antibody-mediated disinhibition of the cortico-striatal systems.<br>

収録刊行物

• Internal Medicine

  Internal Medicine 50 (6), 627-630, 2011

  一般社団法人 日本内科学会

キーワード

• paraneoplastic
• encephalitis
• ovarian tumor
• NMDA receptor
• early treatment

詳細情報

• CRID: 1390001204872820480
• NII論文ID: 130000649906
• DOI: 10.2169/internalmedicine.50.4764
• ISSN: 13497235
• ISSN: 09182918
• Web Site: http://www.jstage.jst.go.jp/article/internalmedicine/50/6/50_6_627/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2012013326
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可