title: Pseudo-piano playing motions and nocturnal hypoventilation in anti-NMDA receptor encephalitis: response to prompt tumor removal and immunotherapy.
abstract: 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.
authors.affiliation: Department of Neurology, Kitasato University, School of Medicine, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Uchino
authors.forename: Takahiro
authors.initials: T
authors.lastname: Iizuka
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Urano
authors.forename: Masahide
authors.initials: M
authors.lastname: Arai
authors.forename: Atsuko
authors.initials: A
authors.lastname: Hara
authors.forename: Junichi
authors.initials: J
authors.lastname: Hamada
authors.forename: Ryuichi
authors.initials: R
authors.lastname: Hirose
authors.forename: Josep
authors.initials: J
authors.lastname: Dalmau
authors.forename: Hideki
authors.initials: H
authors.lastname: Mochizuki
mesh_terms: D020820:Dyskinesias / Q000209:etiology / Q000276:immunology / Q000601:surgery*; D004660:Encephalitis / Q000150:complications / Q000276:immunology / Q000601:surgery*; D005260:Female; D006801:Humans; D007040:Hypoventilation / Q000209:etiology / Q000276:immunology / Q000601:surgery*; D007167:Immunotherapy / Q000379:methods; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology; D013997:Time Factors; D055815:Young Adult
chemical_list: D016194:Receptors, N-Methyl-D-Aspartate