Papers - Seki, Morinobu

Camptocormia in Japanese patients with Parkinson's disease: A multicenter study

Seki M., Takahashi K., Koto A., Mihara B., Morita Y., Isozumi K., Ohta K., Muramatsu K., Gotoh J., Yamaguchi K., Tomita Y., Sato H., Nihei Y., Iwasawa S., Suzuki N.

Movement Disorders (Movement Disorders)  26 ( 14 ) 2567 - 2571 2011.12

ISSN  08853185

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Objective: The aim of this work was to investigate the prevalence of camptocormia and the clinical characteristics of patients with camptocormia in a large population of PD patients. Background: Although camptocormia has been recognized as a prominent phenomenon in PD, the previous epidemiological reports were limited, especially in terms of sample size. Methods:: We evaluated 531 PD patients (disease duration: 7.0 ± 5.5 years, mean ± standard deviation). We examined their clinical features and the prevalence of camptocormia. Results: Camptocormia was detected in 22 patients (4.1%) and found in patients who were older and had more severe motor symptoms and a higher levodopa (L-dopa) dose (P < 0.05), compared to the patients without camptocormia. Patients with camptocormia showed significantly higher frequencies of autonomic symptoms, such as constipation and urinary incontinence (P < 0.05). Conclusions: Camptocormia is uncommon in PD and is associated with disease severity, higher L-dopa dose and higher frequencies of autonomic symptoms. © 2011 Movement Disorder Society.

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Perfusion ¹²³IMP-SPECT shows reversible abnormalities in GABA<inf>B</inf> receptor antibody associated encephalitis with normal MRI

Ohta K., Seki M., Dalmau J., Shinohara Y.

Brain and Behavior (Brain and Behavior)  1 ( 2 ) 70 - 72 2011.11

ISSN  21623279

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A new subtype of autoimmune encephalitis associated with antibodies against GABAB receptor was recently identified. Although immune-mediated functional abnormalities are suggested for the pathogenesis, functional brain imaging such as perfusion SPECT has not been documented. A 62-year-old woman with anti-GABAB receptor associated encephalitis underwent 123I-IMP SPECT in the beginning and after methylprednisolone pulse therapy. Three-dimensional stereotactic surface projection analysis was used to evaluate the cortical distribution of perfusion abnormality. The patient presented with clinical features of subacute limbic encephalitis. Antibodies to GABAB receptor were identified in her serum and cerebrospinal fluid (CSF), but no tumor was detected. Despite normal magnetic resonance imaging (MRI) findings, the first SPECT revealed hypoperfusion of the frontal, parietal and medial temporal lobes, as well as thalamus, and cerebellum. These areas are known to contain high levels of GABAB receptors. In contrast, the SPECT revealed hyperperfusion in the motor strip and left temporal lobe, which are areas related to some of the patient's symptoms, including seizures, orolingual dyskinesia, and Wernicke aphasia. After treatment with pulses of methylprednisolone, the neuropsychiatric symptoms resolved and the SPECT findings showed substantial improvement in most of these regions. In conclusion, the findings suggest that immunotherapy improved the cortical dysfunction mediated by GABAB receptor antibodies. © 2011 The Authors.

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[Limbic encephalitis up to date].

Seki M., Suzuki S., Suzuki N.

Nippon rinsho. Japanese journal of clinical medicine (Nippon rinsho. Japanese journal of clinical medicine)  69 ( 3 ) 442 - 447 2011.03

ISSN  00471852

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Limbic encephalitis (LE) refers to an inflammatory disorder involving the hippocampus, amygdala and insular cortex. LE is now regarded as a more frequent disorder than it was previously thought, and the concept of LE has been expanded because of the development of neuroimaging and the increasing recognition of the associated antibodies. We categorized LE into 5 groups. LE caused by virus infection, autoantibody-mediated LE, LE with autoimmune disease, LE associated with drugs and LE during pregnancy. LE having the antibodies against intracellular antigens frequently related to cancer and are resistant to treatment. While, LE with antibodies against cell-membrane antigens, including NMDAR, AMPAR, GABA(B)R, VGKC, tend to respond better to antitumor therapy and immunotherapy.

Laparoscopic Cystectomy of Ovarian Teratoma in Anti-NMDAR Encephalitis: 2 Case Reports

Asai S., Ishimoto H., Yabuno A., Asada H., Seki M., Iwata S.

Journal of Minimally Invasive Gynecology (Journal of Minimally Invasive Gynecology)  18 ( 1 ) 135 - 137 2011.01

ISSN  15534650

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We present 2 case reports of anti- N-methyl- d-aspartate receptor (NMDAR) encephalitis that was successfully treated via laparoscopic ovarian cystectomy. In both cases, resection of an ovarian teratoma resulted in eventual full recovery. Although adnexectomy has been reported for tumor resection in anti-NMDAR encephalitis, we chose ovarian cystectomy to preserve ovarian function. The efficacy of cystectomy is equivalent to that of adnexectomy. This suggests that ovarian adnexectomy may not be necessary in anti-NMDAR encephalitis with ovarian teratoma. © 2011 AAGL.

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Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS

Ito D., Seki M., Tsunoda Y., Uchiyama H., Suzuki N.

Annals of Neurology (Annals of Neurology)  69 ( 1 ) 152 - 162 2011.01

ISSN  03645134

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Objective: The fused in sarcoma/translated in liposarcoma (FUS/TLS) protein was recently identified as a cause of familial amyotrophic lateral sclerosis (ALS), as well as a major component of the inclusion bodies found in subtypes of frontotemporal lobar degeneration (FTLD). These diseases now are collectively known as the novel clinical spectrum, FUS proteinopathy. ALS-linked mutations of FUS are clustered in the C-terminal region; however, the molecular properties of mutant FUS remain unclear. To gain insight into the pathogenesis of FUS proteinopathy, we examined the biochemical and cellular characteristics of mutant FUS in expressing cells. Methods and Results: Expression of ALS-linked FUS mutations resulted in their assembly into cytoplasmic stress granules (SGs), cellular structures that package mRNA and RNA-binding proteins during cell stress. A deletion mutant series revealed that the C-terminal region in FUS is critical for nuclear retention via Ran guanosine triphosphatase-dependent transport machinery. A parallel study of subcellular distribution revealed that ALS-linked mutants additively disturb the function of the C-terminus for nuclear traffic, resulting in cytoplasmic accumulation and the formation of SGs. Interpretation: This study demonstrates that mutant FUS, which is missing the nuclear traffic activity of the C-terminus, is dislocated to cytoplasm and assembled into SGs, indicating that disruption of translational regulation and metabolism of mRNA via inappropriate/excessive SGs may be crucial for FUS proteinopathies. Our findings provide new biological and pathological insights into the FUS protein that should help our understanding of the pathogenesis of ALS/FTLD. © 2011 American Neurological Association.

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Anti-NMDAR encephalopathy: Treatment

Suzuki S., Seki M., Suzuki N.

Clinical Neurology (Clinical Neurology)  48 ( 11 ) 923 - 925 2008.11

ISSN  0009918X

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Anti-NMDAR encephalopathy is included in paraneoplastic limbic encephalopathy and show the good response to treatment compared to other paraneoplastic syndromes. Treatment of anti-NMDAR encephalopathy includes immunotherapy and/or tumor removal. About 65% of patients with anti-NMDAR encephalopathy had fully or near-full recovery. Immunotherapy is principally necessary and effective in patients with and without tumor. Corticosteroids and intravenous immunoglobulin are most frequently used. It is likely that patients who do not respond to one form of immunotherapy might respond to others regimens including plasmapheresis, cyclophosphamide, and rituximab. A tumor was found in 58% of patients with anti-NMDAR encephalopathy. Early removal of tumor should be considered based on following reasons. First, patients with ovarian teratoma showed higher mortality and higher titer of anti-NMDAR antibody compared with those without. Second, relapsing neurological symptoms occurred in 13% of patients, usually related to a delay in tumor diagnosis. Third, when a tumor was found and removed, recovery was faster and predictable. However, early removal of tumor cannot be conducted because of unstable conditions such as hypoventilation and dyskinesias. In supportive cares, severe central hypoventilation requires mechanical ventilation. The involuntary movements and facial dyskinesias are refractory to anti-epileptic drugs. In conclusion, search for and removal of an ovarian teratoma should be promptly considered after the diagnosis of anti-NMDAR encephalopathy.

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Neurological response to early removal of ovarian teratoma in anti-NMDAR encephalitis

Seki M., Suzuki S., Iizuka T., Shimizu T., Nihei Y., Suzuki N., Dalmau J.

Journal of Neurology, Neurosurgery and Psychiatry (Journal of Neurology, Neurosurgery and Psychiatry)  79 ( 3 ) 324 - 326 2008.03

ISSN  00223050

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We report an 18-year-old woman with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, who developed psychiatric symptoms, progressive unresponsiveness, dyskinesias, hypoventilation, hypersalivation and seizures. Early removal of an ovarian teratoma followed by plasma exchange and corticosteroids resulted in a prompt neurological response and eventual full recovery. Serial analysis of antibodies to NR1/NR2B heteromers of the NMDAR showed an early decrease of serum titres, although the cerebrospinal fluid titres correlated better with clinical outcome. The patients' antibodies reacted with areas of the tumour that contained NMDAR-expressing tissue. Search for and removal of a teratoma should be promptly considered after the diagnosis of anti-NMDAR encephalitis.

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血液透析患者の剖検にて発見された全身性AAアミロイドーシスの1例

大島 直紀, 目黒 真理子, 三上 修治, 北村 直人, 関 守信, 村上 円人

日本透析医学会雑誌 ((一社)日本透析医学会)  39 ( 11 ) 1531 - 1536 2006.11

ISSN  1340-3451

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85歳女。3年の血液透析歴があり、透析中に嘔吐・吐血を認め、上部消化管内視鏡検査で出血性胃潰瘍を認めた。その後、腹部膨満が出現し、腹部CTにてイレウス所見を認めた。以後、水様性下痢便が出現・持続し、4週間後に施行した腹部CTにて大腸の拡張、直腸壁の肥厚を認め、下部消化管内視鏡検査を施行した。肉眼的所見より潰瘍性大腸炎と診断し、プレドニゾロン40mg/日を点滴静注したが症状は改善せず、全身状態悪化し永眠した。剖検での病理組織学的所見で結腸粘膜筋板、粘膜下層および漿膜下層の小血管にアミロイド沈着を認めた。アミロイドはAA蛋白と同定され、全身性AAアミロイドーシスと診断された。

気管挿管確認の際,定量的呼気終末CO2モニターが有用であった溺水による非心原性肺水腫の1例

関 守信, 正岡 建洋, 井口 豪, 山崎 元靖, 並木 淳, 藤島 清太郎, 堀 進悟, 相川 直樹

日本救急医学会関東地方会雑誌 (日本救急医学会-関東地方会)  26   146 - 147 2005.12

ISSN  0287-301X

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67歳男.糖尿病に対するインスリン注射のコンプライアンスが不良で,低血糖発作を頻回に起こしていた.風呂桶に水没しているのを発見され,救急隊到着時の心電図は心静止で,来院5分後に気管挿管を施行した.エピネフリン1mgを3分毎に計12回静注し,血糖値が簡易測定器で感度以下であったため40%ブドウ糖を静脈注射した.CPRを継続したが心静止が持続し,病着から50分後に死亡を確認した.途中で経鼻胃管を挿入し,血性成分を含む排液を認め,腹部レントゲンで胃内に大量のガスを認めた.気管挿管に際しては,まず聴診を行い,胸部および心窩部のいずれでも微弱な音を聴取した.バルブタイプの食道挿管検知器を装着したところ,圧縮した吸引用バルブを気管チューブに接続してもバルブが膨らまず,吸引が維持された.この段階で食道挿管を疑ったが,定量的呼気終末CO2モニターで呼気中にCO2を検出し,気管内への挿管を確認した.胸部X線では気管内に留置された挿管チューブと,溺水によると思われる非心原性肺水腫の所見を認めた