臨床薬理の進歩 No.45

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男1男1女3女1女女1女1女1表2　ISA67例における免疫組織染色の結果と臨床的特徴小脳外症候　錐体路徴候　末梢神経障害　認知機能障害初発症状　体幹失調　四肢失調　構音障害　眼球運動障害modified Rankin Scaleサンプル採取時の罹病期間 (年) †脳脊髄液検査所見　細胞数増多 (/μL) †　タンパク値上昇 (mg/dL) †頭部MRI所見　小脳萎縮脳血流シンチグラフィ　小脳の血流低下　左右非対称の小脳血流低下治療内容　タルチレリン　免疫療法　　ステロイドパルス療法　　免疫グロブリン大量静注療法†表内の数値は中央値＊＊ p < 0.001、＊p < 0.05 (Fisher’s exact test)表3　免疫療法が施行されたISA患者8例の臨床的特徴と治療反応性年齢性別発症から治療までの期間（年）82歳初発症状歩行障害小脳外症候末梢神経障害MRI所見脳血流SPECT両側小脳半球の血流低下小脳萎縮免疫療法IVIgmRS　治療前/後3/2Total (n = 67)37 (55.2%)27 (40.3%)9 (13.4%)8 (11.9%)51 (76.1%)11 (16.4%)24 (35.8%)2 (3.0%)2 (1–5)3 (0.33–29)47 (70.1%)1 (0–5)40 (22–89)64 (95.5%)48 (71.6%)39/48 (81.3%)15/39 (38.5%)36 (53.7%)24 (35.8%)13 (19.4%)12 (17.9%)5 (7.5%)68歳61歳歩行障害構音障害書字障害歩行障害なしなし小脳萎縮小脳萎縮小脳萎縮正常正常IVIg、IVMPIVMP4/42/1抗小脳抗体陽性 （n=30）neuropil antibodies intracellular antibodies (n = 12)2 (16.7%)1 (8.3%)1 (8.3%)0 (0.0%)(n = 18)12 (66.7%)8 (44.4%)3 (16.7%)3 (16.7%)7 (58.3%)3 (25.0%)5 (41.7%)0 (0.0%)2 (1–5)2 (1–11)8 (66.7%)1 (0–2)41 (23–74)14 (77.8%)2 (11.1%)9 (50.0%)1 (5.6%)2 (1–4)5 (0.33–29)15 (83.3%)1 (0–5)40 (28–89)11 (91.7%)8 (66.7%)17 (94.4%)15 (83.3%)13/15 (86.7%)6/13 (46.2%)8 (44.4%)5 (27.8%)4 (22.2%)4 (22.2%)1 (5.6%)4/8 (50%)3/4 (75%)6 (50.0%)2 (16.7%)4 (33.3%)3 (25.0%)2 (16.7%)80歳82歳13歩行障害なし認知障害小脳萎縮未施行両側小脳半球の血流低下IVIg、IVMPIVMP2/24/3陰性 （n=37）p 値23 (62.2%)18 (48.6%)5 (13.5%)5 (13.5%)0.013*0.041**0.8970.45530 (81.1%)6 (16.2%)10 (27.0%)1 (2.7%)2 (1–5)3 (0.75–13)24 (64.9%)1 (0–5)37 (22–72)0.2900.6080.2251.0000.3890.562 0.2070.83936 (97.3%)25 (67.6%)22/25 (88%)6/22 (27.2%)22 (59.5%)17 (45.9%)5 (13.5%)5 (13.5%)2 (5.4%)0.691 0.0820.1480.5580.1630.3030.5110.37147歳77歳歩行障害構音障害末梢神経歩行障害構音障害歩行障害末梢神経認知障害障害障害小脳萎縮小脳萎縮小脳萎縮未施行未施行IVMPIVMP3/34/3IVIg； intravenous immunoglobulin therapy（免疫グロブリン大量静注療法）、IVMP； intravenous methyl prednisolone therapy（ステロイドパルス療法）、mRS； modified Rankin Scale47歳正常IVMP4/4157157