脊髄小脳変性症としての多 ... - kamagaya-hp.jp

1 = = KC-MIND) 23 10 18 14:00 15:30 SCD ( ) ( ) ( ) (SCD) MSA 6 SCD 34,000 , 1000 MSA 21 MSA (MSA) OPCA ,SDS,SND 1969 1) MSA) Graham and Oppenheimer OPCA, Shy-Drager (SDS), (SND)2 (GCI) 3 MSA-P: MSA-C: - MSA : 230 Watanabe H et al. Brain 2002. 125(5), 1070-83. 131 99 (38-75 MSA-C 155 (67%) MSA-P 75 (33%) 64 44 20 MSA-C 106 49 MSA-P ; Parkinsonism 60 15 MSA * MSA *) OH 3 3 30mmHg, 15mmHg MSA MSA (MRI)Multiplex Families With Multiple System Atrophy Kenju Hara; Arch Neurol.

2 2007;64(4):545-551. The spectrum of pathological findings of SND and OPCA in :127:2657 SND OPCA100 GCIs MSA- OPCA MAS-SND basal ganglia cerebellum GCI NCIs SND OPCA GCI MSA MSA Possible MSA (1) (2 ,(3 Table 2b 1 Probable MSA: 1 +(2 Definitive MSA SND OPCA GCI Red flags (supporting the diagnosis of MSA) , , Pisa , , , Table 2b:MSA MSA- MSA-C Babinski MSA-P levodopa 3 5 MRI SN OPCA FDG-PET , MSA-C MRI SN OPCA SPRCT/PET SND MSA 1 (3 2 5 ) 3 (8 4 (9 Watanabe BRAIN 1 OH 2 3 4 5 QOL 5 6 REM Early Development of Autonomic Dysfunction May Predict Poor Prognosis in Patients With MSA Mari Tada Arch Neurol.)))))

3 2007;64(2) MSA 2004 MSA17 8 9 40 73 11 6 5 51 85 6 4 3 2 1 1 1 (1) 17 8 (2) Peg (3) 7 45 10 7 6 2 NPPV 2 Niigata MSA study: (1)probable MSA floppy epiglottis (2)floppy epiglottis (3)floppy epiglottis CPAP CPAP J Neurol 2008 study Niigata MSA study (A) (B) (C) NPPV NPPV ShimohataT, Ozawa T, Nakayama H, Tomita M, ShinodaH, NishizawaM.

4 Frequency of nocturnal sudden death in patients with multiple system atrophy. J Neurol2008 :255:1483. 2008 (in press). (a) mobility ( ) PaCO2 45mmHg(b) PaO2 ALS : :GBS :MG Pickwick MSA Laryngomalacia, MSA ( 2004 21 51 51 27 66 24 64 1 14 OPCA28;SND15;SDS8 45 88 :I(12 II(12 III(4 IV(6 V(4 VI(7 BMI(119).)))))))

5 15 33 29 ( 12 8 9 30 20 45 4 1 2 VF 14 9 9 6 10 6 19 14 1 MSA PEG . 3 141. MSA28 14 14 38 71 66 2 20 MSA-c17 MSA-p11 10 13 BMI) AMC) BMI Peg MSA + PEG MSA 1 ( ) 2 ( ) 3 MSA MSA ( ) RyujiSakakibara, :19,20-31 RyujiSakakibara1etal:Moxibustion,anAlter nativeTherapy, (2007)1015-1018 MSA Flabeau.

6 Ther Adv Neurol Disord. 2010; 3 : 249-263. 2 >100ml, ; <100ml, , -adrenergic Botulinumtoxin A catheterization 2 to 30mg / Riluzole Minocycline Growth hormone Oestrogen Lithium Rasagiline Intravenous immunoglobulins :granulocyte colony stimulating factor (GCSF) MSA Pezzoli et = Biomarker JAMSAC MSA SHC2 19 Shcproteins Hidenao Sasaki et al.

7 :Copy number loss of (src homology 2 domain containing)-transforming protein 2 (SHC2 ) gene: discordant loss in monozygotic twins and frequent loss in patients with multiple system atrophy. Molecular Brain :24